{"text_format":"1","workbook":"xlsx","n_sheets":1,"sheets":["hsa.matrix138.annot"],"not_rendered":{"charts":0,"images":0,"macros":0,"external_links":0,"defined_names":1,"comments":0,"pivot_tables":0},"unopened":[],"bounds":{"document_bytes":16777216,"sheet_cells":100000,"rows_first":2000,"rows_last":500,"rows_between":2000,"halvings":0,"cell_chars":16384,"cell_head_chars":4096},"cells_cut":0,"cells_raw":0,"read":"whole","date_system":"1900"}

{"sheet":"hsa.matrix138.annot","index":0,"n_rows":1147,"n_cols":181,"rows_kept":562,"cut":true,"hidden":false,"merged":[],"bounds":{"rows_first":250,"rows_last":62,"rows_between":250,"halvings":3}}
[null,null,null,null,null,null,null,null,null,null,null,null,null,null,null,null,null,null,null,null,null,null,"Order",0,1,2,3,4,5,6,7,8,9,10,11,12,13,14,15,16,17,18,19,20,21,22,23,24,25,26,27,28,29,30,31,32,33,34,35,36,37,38,39,40,41,42,43,44,45,46,47,48,49,50,51,52,53,54,55,56,57,58,59,60,61,62,63,64,65,66,67,68,69,70,71,72,73,74,75,76,77,78,79,80,81,82,83,84,85,86,87,88,89,90,91,92,93,94,95,96,97,98,99,100,101,102,103,104,105,106,107,108,109,110,111,112,113,114,115,116,117,118,119,120,121,122,123,124,125,126,127,128,129,130,131,132,133,134,135,136,137,138,null,28]
[null,null,null,null,null,null,null,null,null,null,null,null,null,null,null,null,null,null,null,null,null,null,"Group",null,"Other protozoa","Other protozoa","Other protozoa","Other protozoa","Other protozoa","Kinetoplast","Kinetoplast","Kinetoplast","Kinetoplast","Kinetoplast","Other protozoa","Other protozoa","Other protozoa","Other protozoa","Other protozoa","Other protozoa","Apicomplexa","Apicomplexa","Apicomplexa","Apicomplexa","Apicomplexa","Apicomplexa","Other protozoa","Other protozoa","Other protozoa","Other protozoa","Other protozoa","Other protozoa","Other protozoa","Algae","Algae","Algae","Algae","Metaphyta","Metaphyta","Metaphyta","Metaphyta","Metaphyta","Metaphyta","Metaphyta","Metaphyta","Metaphyta","Metaphyta","Metaphyta","Metaphyta","Amoebozoa","Amoebozoa","Amoebozoa","Deep fungi","Deep fungi","Basiodiomycota","Basiodiomycota","Basiodiomycota","Basiodiomycota","Basiodiomycota","Basiodiomycota","Basiodiomycota","Basiodiomycota","Filamentous ascomycota","Filamentous ascomycota","Filamentous ascomycota","Filamentous ascomycota","Filamentous ascomycota","Filamentous ascomycota","Filamentous ascomycota","Filamentous ascomycota","Filamentous ascomycota","Filamentous ascomycota","Filamentous ascomycota","Filamentous ascomycota","Filamentous ascomycota","Filamentous ascomycota","Filamentous ascomycota","Filamentous ascomycota","Filamentous ascomycota","Filamentous ascomycota","Filamentous ascomycota","Non-filamentous ascomycota","Non-filamentous ascomycota","Non-filamentous ascomycota","Non-filamentous ascomycota","Non-filamentous ascomycota","Non-filamentous ascomycota","Non-filamentous ascomycota","Non-filamentous ascomycota","Non-filamentous ascomycota","Non-filamentous ascomycota","Non-filamentous ascomycota","Non-filamentous ascomycota","Non-filamentous ascomycota","Non-filamentous ascomycota","Non-filamentous ascomycota","Non-filamentous ascomycota","Non-filamentous ascomycota","Non-filamentous ascomycota","Non-filamentous ascomycota","Non-filamentous ascomycota","Non-filamentous ascomycota","Invertebrates","Invertebrates","Invertebrates","Invertebrates","Invertebrates","Invertebrates","Invertebrates","Invertebrates","Invertebrates","Invertebrates","Invertebrates","Invertebrates","Invertebrates","Invertebrates","Invertebrates","Invertebrates","Invertebrates","Invertebrates","Invertebrates","Invertebrates","Invertebrates","Invertebrates","Invertebrates","Invertebrates","Invertebrates","Invertebrates","Vertebrates","Vertebrates","Vertebrates","Vertebrates","Vertebrates","Vertebrates","Vertebrates","Vertebrates","Vertebrates","Vertebrates","Vertebrates","Vertebrates","Vertebrates","Vertebrates",null,"Other protozoa"]
[null,null,null,null,null,null,null,null,null,null,null,null,null,null,null,null,null,null,null,null,null,null,"species_abbrev",null,"ecu","ehi","edi","gla","tva","tbr","tcr","lif","lma","lbz","tgo","cho","cpv","bbo","tan","tpv","pkn","pvx","pfa","pcb","pbe","pyo","ptm","tet","pif","tps","pti","cme","ngr","olu","ota","cre","vcn","ppp","smo","sbi","zma","dosa","dbdi","daly","ath","dlja","dmtr","vvi","pop","rcu","e_ttr","ddi","e_ama","e_spu","mgl","uma","cne","dpch","scm","cci","lbc","spo","bfu","ssl","fgr","mgr","ncr","pan","pcs","act","afm","nfi","afv","aor","ang","ani","ure","cim","cpw","pno","tml","yli","ppa","clu","dha","pgu","pic","lel","ctp","cal","cdu","kla","ago","dkwa","lth","zro","vpo","cgr","dsba","dsmi","sce","dsrd","e_sar","e_cow","mbr","e_sro","smm","bmy","cbr","cel","ddpu","dapi","phu","ame","nvi","dbmo","tca","dme","dpo","aga","aag","cqu","bfo","tad","spu","hmg","nve","cin","dre","dola","dfru","dtni","xtr","gga","mgp","oaa","mdo","ssc","mmu","cfa","bta","hsa",null,"cme"]
[null,null,null,null,null,null,null,null,null,null,null,null,null,null,null,null,null,null,null,null,null,null,"taxid",null,284813,294381,370354,184922,412133,185431,353153,435258,347515,420245,508771,353151,353152,484906,353154,333668,5851,126793,36329,31271,5823,352914,412030,312017,403677,296543,556484,280699,744533,436017,70448,3055,3068,145481,88036,4558,4577,39947,15368,81972,3702,34305,3880,29760,3694,3988,461836,352472,578462,645134,425265,237631,214684,273507,578458,240176,486041,284812,332648,665079,229533,242507,367110,515849,500485,344612,330879,331117,332952,510516,425011,227321,336963,246410,222929,321614,656061,284591,644223,306902,284592,294746,322104,379508,294747,237561,573826,284590,284811,262981,559295,559307,436907,284593,226127,226126,559292,226125,667725,595528,431895,946362,6183,6279,6238,6239,6669,7029,121224,7460,7425,7091,7070,7227,46245,180454,7159,7176,7739,10228,7668,6085,45351,7719,7955,8090,31033,99883,8364,9031,9103,9258,13616,9823,10090,9615,9913,9606,null,280699]
[null,null,null,null,null,null,null,null,null,null,null,null,null,null,null,null,null,null,null,null,null,null,"species name",null,"Encephalitozoon cuniculi","Entamoeba histolytica","Entamoeba dispar","Giardia lamblia","Trichomonas vaginalis","Trypanosoma brucei","Trypanosoma cruzi","Leishmania infantum","Leishmania major","Leishmania braziliensis","Toxoplasma gondii","Cryptosporidium hominis","Cryptosporidium parvum","Babesia bovis","Theileria annulata","Theileria parva","Plasmodium knowlesi","Plasmodium vivax","Plasmodium falciparum 3D7","Plasmodium chabaudi","Plasmodium berghei","Plasmodium yoelii","Paramecium tetraurelia","Tetrahymena thermophila","Phytophthora infestans","Thalassiosira pseudonana","Phaeodactylum tricornutum","Cyanidioschyzon merolae","Naegleria gruberi","Ostreococcus lucimarinus","Ostreococcus tauri","Chlamydomonas reinhardtii","Volvox carteri f. nagariensis","Physcomitrella patens subsp. patens","Selaginella moellendorffii","Sorghum bicolor (sorghum)","Zea mays (maize)","Oryza sativa japonica (Japanese rice) (RAPDB)","Brachypodium distachyon","Arabidopsis lyrata (lyrate rockcress)","Arabidopsis thaliana (thale cress)","Lotus japonicus (lotus)","Medicago truncatula (barrel medic)","Vitis vinifera (wine grape)","Populus trichocarpa (black cottonwood)","Ricinus communis (castor bean)","Thecamonas trahens ATCC 50062","Dictyostelium discoideum (cellular slime mold)","Allomyces macrogynus ATCC 38327","Spizellomyces punctatus DAOM BR117","Malassezia globosa","Ustilago maydis","Cryptococcus neoformans JEC21","Phanerochaete chrysosporium","Schizophyllum commune","Coprinopsis cinerea","Laccaria bicolor","Schizosaccharomyces pombe (fission yeast)","Botryotinia fuckeliana","Sclerotinia sclerotiorum","Fusarium graminearum","Magnaporthe oryzae","Neurospora crassa","Podospora anserina","Penicillium chrysogenum","Aspergillus clavatus","Aspergillus fumigatus","Neosartorya fischeri","Aspergillus flavus","Aspergillus oryzae","Aspergillus niger","Aspergillus nidulans","Uncinocarpus reesii","Coccidioides immitis","Coccidioides posadasii","Phaeosphaeria nodorum","Tuber melanosporum","Yarrowia lipolytica","Pichia pastoris","Clavispora lusitaniae","Debaryomyces hansenii","Meyerozyma guilliermondii","Scheffersomyces stipitis","Lodderomyces elongisporus","Candida tropicalis","Candida albicans","Candida dubliniensis","Kluyveromyces lactis","Ashbya gossypii (Eremothecium gossypii)","Kluyveromyces waltii","Lachancea thermotolerans","Zygosaccharomyces rouxii","Vanderwaltozyma polyspora","Candida glabrata","Saccharomyces bayanus","Saccharomyces mikatae","Saccharomyces cerevisiae (budding yeast)","Saccharomyces paradoxus","Sphaeroforma arctica JP610","Capsaspora owczarzaki ATCC 30864","Monosiga brevicollis","Salpingoeca rosetta","Schistosoma mansoni","Brugia malayi (filaria)","Caenorhabditis briggsae","Caenorhabditis elegans (nematode)","Daphnia pulex (water flea)","Acyrthosiphon pisum (pea aphid) (AphidBase)","Pediculus humanus corporis (human body louse)","Apis mellifera (honey bee)","Nasonia vitripennis (jewel wasp)","Bombyx mori (silkmoth)","Tribolium castaneum (red flour beetle)","Drosophila melanogaster (fruit fly)","Drosophila pseudoobscura pseudoobscura","Anopheles gambiae (mosquito)","Aedes aegypti (yellow fever mosquito)","Culex quinquefasciatus (southern house mosquito)","Branchiostoma floridae (Florida lancelet)","Trichoplax adhaerens","Strongylocentrotus purpuratus (purple sea urchin)","Hydra magnipapillata","Nematostella vectensis (sea anemone)","Ciona intestinalis (sea squirt)","Danio rerio (zebrafish)","Oryzias latipes (Japanese medaka)","Fugu rubripes (Japanese puffer fish)","Tetraodon nigroviridis (green spotted pufferfish)","Xenopus tropicalis (western clawed frog)","Gallus gallus (chicken)","Meleagris gallopavo (turkey)","Ornithorhynchus anatinus (platypus)","Monodelphis domestica (opossum)","Sus scrofa (pig)","Mus musculus (mouse)","Canis familiaris (dog)","Bos taurus (cow)","Homo sapiens (human)",null,"Cyanidioschyzon merolae"]
[null,null,null,null,null,null,null,null,null,null,null,null,null,null,null,null,null,null,null,null,null,null,"Genus.species","Prokaryotes","Encephalitozoon.cuniculi","Entamoeba.histolytica","Entamoeba.dispar","Giardia.lamblia","Trichomonas.vaginalis","Trypanosoma.brucei","Trypanosoma.cruzi","Leishmania.infantum","Leishmania.major","Leishmania.braziliensis","Toxoplasma.gondii","Cryptosporidium.hominis","Cryptosporidium.parvum","Babesia.bovis","Theileria.annulata","Theileria.parva","Plasmodium.knowlesi","Plasmodium.vivax","Plasmodium.falciparum","Plasmodium.chabaudi","Plasmodium.berghei","Plasmodium.yoelii","Paramecium.tetraurelia","Tetrahymena.thermophila","Phytophthora.infestans","Thalassiosira.pseudonana","Phaeodactylum.tricornutum","Cyanidioschyzon.merolae","Naegleria.gruberi","Ostreococcus.lucimarinus","Ostreococcus.tauri","Chlamydomonas.reinhardtii","Volvox.carteri","Physcomitrella.patens","Selaginella.moellendorffii","Sorghum.bicolor","Zea.mays","Oryza.sativa","Brachypodium.distachyon","Arabidopsis.lyrata","Arabidopsis.thaliana","Lotus.japonicus","Medicago.truncatula","Vitis.vinifera","Populus.trichocarpa","Ricinus.communis","Thecamonas.trahens","Dictyostelium.discoideum","Allomyces.macrogynus","Spizellomyces.punctatus","Malassezia.globosa","Ustilago.maydis","Cryptococcus.neoformans","Phanerochaete.chrysosporium","Schizophyllum.commune","Coprinopsis.cinerea","Laccaria.bicolor","Schizosaccharomyces.pombe","Botryotinia.fuckeliana","Sclerotinia.sclerotiorum","Fusarium.graminearum","Magnaporthe.oryzae","Neurospora.crassa","Podospora.anserina","Penicillium.chrysogenum","Aspergillus.clavatus","Aspergillus.fumigatus","Neosartorya.fischeri","Aspergillus.flavus","Aspergillus.oryzae","Aspergillus.niger","Aspergillus.nidulans","Uncinocarpus.reesii","Coccidioides.immitis","Coccidioides.posadasii","Phaeosphaeria.nodorum","Tuber.melanosporum","Yarrowia.lipolytica","Pichia.pastoris","Clavispora.lusitaniae","Debaryomyces.hansenii","Meyerozyma.guilliermondii","Scheffersomyces.stipitis","Lodderomyces.elongisporus","Candida.tropicalis","Candida.albicans","Candida.dubliniensis","Kluyveromyces.lactis","Ashbya.gossypii","Kluyveromyces.waltii","Lachancea.thermotolerans","Zygosaccharomyces.rouxii","Vanderwaltozyma.polyspora","Candida.glabrata","Saccharomyces.bayanus","Saccharomyces.mikatae","Saccharomyces.cerevisiae","Saccharomyces.paradoxus","Sphaeroforma.arctica","Capsaspora.owczarzaki","Monosiga.brevicollis","Salpingoeca.rosetta","Schistosoma.mansoni","Brugia.malayi","Caenorhabditis.briggsae","Caenorhabditis.elegans","Daphnia.pulex","Acyrthosiphon.pisum","Pediculus.humanus","Apis.mellifera","Nasonia.vitripennis","Bombyx.mori","Tribolium.castaneum","Drosophila.melanogaster","Drosophila.pseudoobscura","Anopheles.gambiae","Aedes.aegypti","Culex.quinquefasciatus","Branchiostoma.floridae","Trichoplax.adhaerens","Strongylocentrotus.purpuratus","Hydra.magnipapillata","Nematostella.vectensis","Ciona.intestinalis","Danio.rerio","Oryzias.latipes","Fugu.rubripes","Tetraodon.nigroviridis","Xenopus.tropicalis","Gallus.gallus","Meleagris.gallopavo","Ornithorhynchus.anatinus","Monodelphis.domestica","Sus.scrofa","Mus.musculus","Canis.familiaris","Bos.taurus","Homo.sapiens",null,"Cyanidioschyzon.merolae"]
["KeggID","Entrez","Symbol","Synonyms","chromosome","description","GO celloc","GO molfunc","GO bioproc","SYM","DESCRIPTION","MitoCarta3.0_LIST","MitoCarta3.0_Pathways","MitoCarta3.0_SubMitoLoc","TMHMM_NUM_HELIX","PFAM_DOMAINS","KEGG_PATHWAYS_FILTERED","EnsemblFamilyName","EnsemblFamilyID","EnsemblFamilySize","FirstInEnsemblFamily","OMIM_morbidmap_dz_name_list","sym","Prokaryote","E.cuniculi","E.histolytica","E.dispar","G.lamblia","T.vaginalis","T.brucei","T.cruzi","L.infantum","L.major","L.braziliensis","T.gondii","C.hominis","C.parvum","B.bovis","T.annulata","T.parva","P.knowlesi","P.vivax","P.falciparum","P.chabaudi","P.berghei","P.yoelii","P.tetraurelia","T.thermophila","P.infestans","T.pseudonana","P.tricornutum","C.merolae","N.gruberi","O.lucimarinus","O.tauri","C.reinhardtii","V.carteri","P.patens","S.moellendorffii","S.bicolor","Z.mays","O.sativa","B.distachyon","A.lyrata","A.thaliana","L.japonicus","M.truncatula","V.vinifera","P.trichocarpa","R.communis","T.trahens","D.discoideum","A.macrogynus","S.punctatus","M.globosa","U.maydis","C.neoformans","P.chrysosporium","S.commune","C.cinerea","L.bicolor","S.pombe","B.fuckeliana","S.sclerotiorum","F.graminearum","M.grisea","N.crassa","P.anserina","P.chrysogenum","A.clavatus","A.fumigatus","N.fischeri","A.flavus","A.oryzae","A.niger","A.nidulans","U.reesii","C.immitis","C.posadasii","P.nodorum","T.melanosporum","Y.lipolytica","P.pastoris","C.lusitaniae","D.hansenii","M.guilliermondii","S.stipitis","L.elongisporus","C.tropicalis","C.albicans","C.dubliniensis","K.lactis","A.gossypii","K.waltii","L.thermotolerans","Z.rouxii","V.polyspora","C.glabrata","S.bayanus","S.mikatae","S.cerevisiae","S.paradoxus","S.arctica","C.owczarzaki","M.brevicollis","S.rosetta","S.mansoni","B.malayi","C.briggsae","C.elegans","D.pulex","A.pisum","P.humanus","A.mellifera","N.vitripennis","B.mori","T.castaneum","D.melanogaster","D.pseudoobscura","A.gambiae","A.aegypti","C.quinquefasciatus","B.floridae","T.adhaerens","S.purpuratus","H.magnipapillata","N.vectensis","C.intestinalis","D.rerio","O.latipes","F.rubripes","T.nigroviridis","X.tropicalis","G.gallus","M.gallopavo","O.anatinus","M.domestica","S.scrofa","M.musculus","C.familiaris","B.taurus","H.sapiens","sum","C.merolae","C.merolae_BBH","MitoCarta3.0_Pathways","MitoCarta3.0_Pathways"]
["hsa:10350",10350,"ABCA9","DKFZp686F2450|EST640918|MGC75415",17,"ATP-binding cassette, sub-family A (ABC1), member 9","integral to membrane | membrane","ATPase activity | nucleotide binding | ATP binding","transport","ABCA9","ATP-binding cassette, sub-family A (ABC1), member 9","MitoCarta3.0","Small molecule transport > ABC transporters","Membrane",14,"ABC_tran",null,"ATP BINDING CASSETTE SUB FAMILY A MEMBER","ENSFM00570000851109",5,"FirstInEnsemblFamily",null,"ABCA9",1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,139,1,0,"Small molecule transport > ABC transporters","Small molecule transport "," ABC transporters"]
["hsa:23456",23456,"ABCB10","EST20237|M-ABC2|MTABC2",1,"ATP-binding cassette, sub-family B (MDR/TAP), member 10","integral to mitochondrial membrane | mitochondrion | integral to membrane | mitochondrial inner membrane | plasma membrane","ATPase activity, coupled to transmembrane movement of substances | ATPase activity | transporter activity | protein homodimerization activity | nucleotide binding | oligopeptide-transporting ATPase activity | ATP binding","transport | transmembrane transport","ABCB10","ATP-binding cassette, sub-family B (MDR/TAP), member 10","MitoCarta3.0","Metabolism > Metals and cofactors > Heme synthesis and processing | Small molecule transport > ABC transporters","MIM",5,"ABC_tran | ABC_membrane",null,"ATP BINDING CASSETTE SUB FAMILY B MEMBER MITOCHONDRIAL PRECURSOR","ENSFM00520000517816",2,null,null,"ABCB10",1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,139,1,1,"Metabolism > Metals and cofactors > Heme synthesis and processing | Small molecule transport > ABC transporters","Metabolism "," Metals and cofactors "," Heme synthesis and processing "," Small molecule transport "," ABC transporters"]
["hsa:10058",10058,"ABCB6","ABC|ABC14|FLJ22414|MTABC3|PRP|umat",2,"ATP-binding cassette, sub-family B (MDR/TAP), member 6","mitochondrion | ATP-binding cassette (ABC) transporter complex | Golgi apparatus | vacuolar membrane | mitochondrial envelope | mitochondrial outer membrane | integral to membrane | plasma membrane | integral to mitochondrial outer membrane","efflux transmembrane transporter activity | heme-transporting ATPase activity | ATPase activity | heme binding | nucleotide binding | ATP binding","cadmium ion transmembrane transport | cellular iron ion homeostasis | transport | porphyrin-containing compound biosynthetic process | detoxification of cadmium ion | heme transport | transmembrane transport","ABCB6","ATP-binding cassette, sub-family B (MDR/TAP), member 6","MitoCarta3.0","Metabolism > Metals and cofactors > Heme synthesis and processing | Small molecule transport > ABC transporters","MOM",9,"ABC_tran | ABC_membrane",null,"IRON SULFUR CLUSTERS TRANSPORTER ATM1 MITOCHONDRIAL PRECURSOR","ENSFM00400000131786",2,"FirstInEnsemblFamily","Microphthalmia, isolated, with coloboma 7","ABCB6",1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,139,1,1,"Metabolism > Metals and cofactors > Heme synthesis and processing | Small molecule transport > ABC transporters","Metabolism "," Metals and cofactors "," Heme synthesis and processing "," Small molecule transport "," ABC transporters"]
["hsa:22",22,"ABCB7","ABC7|ASAT|Atm1p|EST140535","X","ATP-binding cassette, sub-family B (MDR/TAP), member 7","mitochondrion | integral to membrane | mitochondrial inner membrane | membrane | cytoplasm","ATPase activity, coupled to transmembrane movement of substances | heme transporter activity | ATPase activity | nucleotide binding | ATP binding","cellular iron ion homeostasis | transport | heme transport | transmembrane transport",null,null,"MitoCarta3.0","Metabolism > Metals and cofactors > Heme synthesis and processing | Metabolism > Metals and cofactors > Fe-S cluster biosynthesis | Metabolism > Metals and cofactors > Molybdenum cofactor synthesis and proteins | Small molecule transport > ABC transporters","MIM",null,"ABC_tran | ABC_membrane",null,"IRON SULFUR CLUSTERS TRANSPORTER ATM1 MITOCHONDRIAL PRECURSOR","ENSFM00400000131786",2,null,"Anemia, sideroblastic, with ataxia","ABCB7",1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,139,1,1,"Metabolism > Metals and cofactors > Heme synthesis and processing | Metabolism > Metals and cofactors > Fe-S cluster biosynthesis | Metabolism > Metals and cofactors > Molybdenum cofactor synthesis and proteins | Small molecule transport > ABC transporters","Metabolism "," Metals and cofactors "," Heme synthesis and processing "," Metabolism "," Metals and cofactors "," Fe-S cluster biosynthesis "," Metabolism "," Metals and cofactors "," Molybdenum cofactor synthesis and proteins "," Small molecule transport "," ABC transporters"]
["hsa:11194",11194,"ABCB8","EST328128|M-ABC1|MABC1",7,"ATP-binding cassette, sub-family B (MDR/TAP), member 8","mitochondrial envelope | mitochondrion | integral to membrane | ATP-binding cassette (ABC) transporter complex | mitochondrial inner membrane | membrane | membrane fraction","ATPase activity, coupled to transmembrane movement of substances | ATPase activity | transporter activity | nucleotide binding | ATP binding","transport | transmembrane transport","ABCB8","ATP-binding cassette, sub-family B (MDR/TAP), member 8","MitoCarta3.0","Small molecule transport > ABC transporters","MIM",3,"ABC_tran | ABC_membrane",null,"ATP BINDING CASSETTE SUB FAMILY B MEMBER MITOCHONDRIAL PRECURSOR","ENSFM00520000517816",2,"FirstInEnsemblFamily",null,"ABCB8",1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,139,1,0,"Small molecule transport > ABC transporters","Small molecule transport "," ABC transporters"]
["hsa:215",215,"ABCD1","ABC42|ALD|ALDP|AMN","X","ATP-binding cassette, sub-family D (ALD), member 1","cytosol | mitochondrion | integral to membrane | peroxisomal membrane | peroxisome | membrane | integral to peroxisomal membrane","ATPase activity, coupled to transmembrane movement of substances | transporter activity | nucleotide binding | ATP binding | protein binding | ATPase activity | peroxisomal fatty-acyl-CoA transporter activity | identical protein binding","peroxisomal long-chain fatty acid import | fatty acid beta-oxidation using acyl-CoA oxidase | peroxisome organization | cellular lipid metabolic process | peroxisomal membrane transport | transmembrane transport","ABCD1","ATP-binding cassette, sub-family D (ALD), member 1","MitoCarta3.0","Small molecule transport > ABC transporters","Membrane",0,"ABC_tran | ABC_membrane_2","Peroxisome","ATP BINDING CASSETTE SUB FAMILY D MEMBER","ENSFM00250000000815",3,"FirstInEnsemblFamily","Adrenoleukodystrophy","ABCD1",1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,139,1,1,"Small molecule transport > ABC transporters","Small molecule transport "," ABC transporters"]
["hsa:225",225,"ABCD2","ABC39|ALDL1|ALDR|ALDRP|hALDR",12,"ATP-binding cassette, sub-family D (ALD), member 2","mitochondrion | ATP-binding cassette (ABC) transporter complex | peroxisomal membrane | peroxisome | integral to plasma membrane | membrane","protein binding | ATPase activity | nucleotide binding | ATP binding","transport | fatty acid metabolic process","ABCD2","ATP-binding cassette, sub-family D (ALD), member 2","MitoCarta3.0","Small molecule transport > ABC transporters","Membrane",0,"ABC_tran | ABC_membrane_2","Peroxisome","ATP BINDING CASSETTE SUB FAMILY D MEMBER","ENSFM00250000000815",3,null,null,"ABCD2",1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,139,1,0,"Small molecule transport > ABC transporters","Small molecule transport "," ABC transporters"]
["hsa:80221",80221,"ACSF2","ACSMW|AVYV493|FLJ20920",17,"acyl-CoA synthetase family member 2","mitochondrion","nucleotide binding | ligase activity | ATP binding","fatty acid metabolic process | lipid metabolic process","FLJ20920","hypothetical protein FLJ20920","MitoCarta3.0","Metabolism > Lipid metabolism > Fatty acid oxidation","Matrix",0,"AMP-binding",null,null,null,null,"FirstInEnsemblFamily",null,"ACSF2",1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,139,1,0,"Metabolism > Lipid metabolism > Fatty acid oxidation","Metabolism "," Lipid metabolism "," Fatty acid oxidation"]
["hsa:197322",197322,"ACSF3","FLJ39242",16,"acyl-CoA synthetase family member 3","mitochondrion","acid-thiol ligase activity | nucleotide binding | ligase activity | ATP binding","fatty acid metabolic process | lipid metabolic process","LOC197322","hypothetical protein LOC197322","MitoCarta3.0","Metabolism > Lipid metabolism > Type II fatty acid synthesis","Matrix",0,"AMP-binding",null,null,null,null,"FirstInEnsemblFamily","Combined malonic and methylmalonic aciduria","ACSF3",1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,139,1,0,"Metabolism > Lipid metabolism > Type II fatty acid synthesis","Metabolism "," Lipid metabolism "," Type II fatty acid synthesis"]
["hsa:2180",2180,"ACSL1","ACS1|FACL1|FACL2|LACS|LACS1|LACS2",4,"acyl-CoA synthetase long-chain family member 1","mitochondrial outer membrane | mitochondrion | integral to membrane | endoplasmic reticulum | peroxisomal membrane | peroxisome | microsome | endoplasmic reticulum membrane | membrane","long-chain fatty acid-CoA ligase activity | nucleotide binding | ligase activity | ATP binding","long-chain fatty-acyl-CoA biosynthetic process | triglyceride biosynthetic process | digestion | fatty acid metabolic process | regulation of fatty acid oxidation | cellular lipid metabolic process","ACSL1","acyl-CoA synthetase long-chain family member 1","MitoCarta3.0","Metabolism > Lipid metabolism > Fatty acid oxidation","MOM",1,"AMP-binding","Peroxisome | Fatty acid metabolism","LONG CHAIN FATTY ACID COA LIGASE EC_6.2.1.3 LONG CHAIN ACYL COA SYNTHETASE LACS","ENSFM00500000269666",3,null,null,"ACSL1",1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,139,1,1,"Metabolism > Lipid metabolism > Fatty acid oxidation","Metabolism "," Lipid metabolism "," Fatty acid oxidation"]
["hsa:23305",23305,"ACSL6","ACS2|FACL6|FLJ16173|KIAA0837|LACS 6|LACS2|LACS5",5,"acyl-CoA synthetase long-chain family member 6","mitochondrion | endoplasmic reticulum | peroxisome | mitochondrial outer membrane | integral to membrane | peroxisomal membrane | plasma membrane | endoplasmic reticulum membrane | microsome","long-chain fatty acid-CoA ligase activity | nucleotide binding | ligase activity | ATP binding","long-chain fatty-acyl-CoA biosynthetic process | triglyceride biosynthetic process | acyl-CoA metabolic process | fatty acid metabolic process | cellular lipid metabolic process | neuroblast proliferation","ACSL6","acyl-CoA synthetase long-chain family member 6","MitoCarta3.0","Metabolism > Lipid metabolism > Fatty acid oxidation","MOM",2,"AMP-binding","Peroxisome | Fatty acid metabolism","LONG CHAIN FATTY ACID COA LIGASE EC_6.2.1.3 LONG CHAIN ACYL COA SYNTHETASE LACS","ENSFM00500000269666",3,null,null,"ACSL6",1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,139,1,0,"Metabolism > Lipid metabolism > Fatty acid oxidation","Metabolism "," Lipid metabolism "," Fatty acid oxidation"]
["hsa:116285",116285,"ACSM1","BUCS1|MACS1|MGC150532",16,"acyl-CoA synthetase medium-chain family member 1","mitochondrion | mitochondrial matrix","fatty acid ligase activity | acyl-CoA ligase activity | butyrate-CoA ligase activity | catalytic activity | nucleotide binding | ligase activity | ATP binding | GTP binding | metal ion binding","xenobiotic metabolic process | energy derivation by oxidation of organic compounds | benzoate metabolic process | fatty acid biosynthetic process | butyrate metabolic process | fatty acid oxidation | lipid metabolic process","ACSM1","acyl-CoA synthetase medium-chain family member 1","MitoCarta3.0","Metabolism > Lipid metabolism > Fatty acid oxidation","Matrix",0,"AMP-binding","Butanoate metabolism","ACYL COENZYME A SYNTHETASE MITOCHONDRIAL PRECURSOR EC_6.2.1.2 ACYL COA SYNTHETASE MEDIUM CHAIN FAMILY MEMBER BUTYRATE COA LIGASE BUTYRYL COENZYME A SYNTHETASE MIDDLE CHAIN ACYL COA SYNTHETASE","ENSFM00250000001196",7,"FirstInEnsemblFamily",null,"ACSM1",1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,139,1,0,"Metabolism > Lipid metabolism > Fatty acid oxidation","Metabolism "," Lipid metabolism "," Fatty acid oxidation"]
["hsa:123876",123876,"ACSM2A","A-923A4.1|ACSM2|FLJ34659|MGC150530",16,"acyl-CoA synthetase medium-chain family member 2A","mitochondrion | mitochondrial matrix","butyrate-CoA ligase activity | nucleotide binding | metal ion binding | ligase activity | ATP binding","fatty acid metabolic process | lipid metabolic process","LOC123876","hypothetical protein LOC123876","MitoCarta3.0","Metabolism > Lipid metabolism > Fatty acid oxidation | Metabolism > Detoxification > Xenobiotic metabolism","Matrix",0,"AMP-binding","Butanoate metabolism","ACYL COENZYME A SYNTHETASE MITOCHONDRIAL PRECURSOR EC_6.2.1.2 ACYL COA SYNTHETASE MEDIUM CHAIN FAMILY MEMBER BUTYRATE COA LIGASE BUTYRYL COENZYME A SYNTHETASE MIDDLE CHAIN ACYL COA SYNTHETASE","ENSFM00250000001196",7,null,null,"ACSM2A",1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,139,1,0,"Metabolism > Lipid metabolism > Fatty acid oxidation | Metabolism > Detoxification > Xenobiotic metabolism","Metabolism "," Lipid metabolism "," Fatty acid oxidation "," Metabolism "," Detoxification "," Xenobiotic metabolism"]
["hsa:348158",348158,"ACSM2B","ACSM2|HXMA",16,"acyl-CoA synthetase medium-chain family member 2B","mitochondrion | mitochondrial matrix","butyrate-CoA ligase activity | nucleotide binding | metal ion binding | ligase activity | ATP binding","xenobiotic metabolic process | fatty acid metabolic process | lipid metabolic process",null,null,"MitoCarta3.0","Metabolism > Lipid metabolism > Fatty acid oxidation","Matrix",null,"AMP-binding","Butanoate metabolism","ACYL COENZYME A SYNTHETASE MITOCHONDRIAL PRECURSOR EC_6.2.1.2 ACYL COA SYNTHETASE MEDIUM CHAIN FAMILY MEMBER BUTYRATE COA LIGASE BUTYRYL COENZYME A SYNTHETASE MIDDLE CHAIN ACYL COA SYNTHETASE","ENSFM00250000001196",7,null,null,"ACSM2B",1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,139,1,0,"Metabolism > Lipid metabolism > Fatty acid oxidation","Metabolism "," Lipid metabolism "," Fatty acid oxidation"]
["hsa:6296",6296,"ACSM3","SA|SAH",16,"acyl-CoA synthetase medium-chain family member 3","mitochondrion | mitochondrial matrix | cellular_component","fatty acid ligase activity | molecular_function | butyrate-CoA ligase activity | nucleotide binding | metal ion binding | ligase activity | ATP binding","regulation of blood pressure | fatty acid biosynthetic process | lipid metabolic process","ACSM3","acyl-CoA synthetase medium-chain family member 3","MitoCarta3.0","Metabolism > Lipid metabolism > Fatty acid oxidation","Matrix",0,"AMP-binding","Butanoate metabolism","ACYL COENZYME A SYNTHETASE MITOCHONDRIAL PRECURSOR EC_6.2.1.2 ACYL COA SYNTHETASE MEDIUM CHAIN FAMILY MEMBER BUTYRATE COA LIGASE BUTYRYL COENZYME A SYNTHETASE MIDDLE CHAIN ACYL COA SYNTHETASE","ENSFM00250000001196",7,null,null,"ACSM3",1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,139,1,0,"Metabolism > Lipid metabolism > Fatty acid oxidation","Metabolism "," Lipid metabolism "," Fatty acid oxidation"]
["hsa:341392",341392,"ACSM4","-",12,"acyl-CoA synthetase medium-chain family member 4","mitochondrion | mitochondrial matrix","butyrate-CoA ligase activity | fatty-acyl-CoA synthase activity | nucleotide binding | metal ion binding | ligase activity | ATP binding","acyl-CoA metabolic process | fatty acid metabolic process | lipid metabolic process","LOC341392","similar to olfactory specific medium-chain acyl CoA synthetase","MitoCarta3.0","Metabolism > Lipid metabolism > Fatty acid oxidation","Matrix",0,"AMP-binding","Butanoate metabolism","ACYL COENZYME A SYNTHETASE MITOCHONDRIAL PRECURSOR EC_6.2.1.2 ACYL COA SYNTHETASE MEDIUM CHAIN FAMILY MEMBER BUTYRATE COA LIGASE BUTYRYL COENZYME A SYNTHETASE MIDDLE CHAIN ACYL COA SYNTHETASE","ENSFM00250000001196",7,null,null,"ACSM4",1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,139,1,0,"Metabolism > Lipid metabolism > Fatty acid oxidation","Metabolism "," Lipid metabolism "," Fatty acid oxidation"]
["hsa:54988",54988,"ACSM5","FLJ20581",16,"acyl-CoA synthetase medium-chain family member 5","mitochondrion | mitochondrial matrix","GTP binding | butyrate-CoA ligase activity | nucleotide binding | metal ion binding | ligase activity | ATP binding","fatty acid metabolic process | lipid metabolic process","FLJ20581","hypothetical protein FLJ20581","MitoCarta3.0","Metabolism > Lipid metabolism > Fatty acid oxidation | Metabolism > Detoxification > Xenobiotic metabolism","Matrix",0,"AMP-binding","Butanoate metabolism","ACYL COENZYME A SYNTHETASE MITOCHONDRIAL PRECURSOR EC_6.2.1.2 ACYL COA SYNTHETASE MEDIUM CHAIN FAMILY MEMBER BUTYRATE COA LIGASE BUTYRYL COENZYME A SYNTHETASE MIDDLE CHAIN ACYL COA SYNTHETASE","ENSFM00250000001196",7,null,null,"ACSM5",1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,139,1,0,"Metabolism > Lipid metabolism > Fatty acid oxidation | Metabolism > Detoxification > Xenobiotic metabolism","Metabolism "," Lipid metabolism "," Fatty acid oxidation "," Metabolism "," Detoxification "," Xenobiotic metabolism"]
["hsa:84532",84532,"ACSS1","ACAS2L|AceCS2L|FLJ45659|MGC33843",20,"acyl-CoA synthetase short-chain family member 1","mitochondrion | mitochondrial matrix","acetate-CoA ligase activity | protein binding | catalytic activity | nucleotide binding | ligase activity | AMP binding | ATP binding","xenobiotic metabolic process | acetate biosynthetic process | acetyl-CoA biosynthetic process | ethanol oxidation | propionate biosynthetic process","ACSS1","acyl-CoA synthetase short-chain family member 1","MitoCarta3.0","Metabolism > Lipid metabolism > Fatty acid oxidation","MIM",0,"AMP-binding","Propanoate metabolism | Pyruvate metabolism | Glycolysis / Gluconeogenesis","ACETYL COENZYME A SYNTHETASE EC_6.2.1.1 ACETATE COA LIGASE ACYL ACTIVATING ENZYME","ENSFM00250000000648",2,"FirstInEnsemblFamily",null,"ACSS1",1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,139,1,0,"Metabolism > Lipid metabolism > Fatty acid oxidation","Metabolism "," Lipid metabolism "," Fatty acid oxidation"]
["hsa:79611",79611,"ACSS3","FLJ21963",12,"acyl-CoA synthetase short-chain family member 3","mitochondrion","acetate-CoA ligase activity | nucleotide binding | ligase activity | ATP binding",null,null,null,"MitoCarta3.0","Metabolism > Lipid metabolism","Matrix",null,"AMP-binding","Propanoate metabolism",null,null,null,"FirstInEnsemblFamily",null,"ACSS3",1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,139,1,0,"Metabolism > Lipid metabolism","Metabolism "," Lipid metabolism"]
["hsa:10939",10939,"AFG3L2","FLJ25993|SCA28",18,"AFG3 ATPase family gene 3-like 2 (S. cerevisiae)","mitochondrion | integral to membrane | mitochondrial inner membrane | membrane","unfolded protein binding | peptidase activity | nucleoside-triphosphatase activity | metalloendopeptidase activity | zinc ion binding | nucleotide binding | metal ion binding | ATP binding","neuromuscular junction development | righting reflex | protein catabolic process | muscle fiber development | nerve development | proteolysis | axonogenesis | myelination | cell death | regulation of multicellular organism growth","AFG3L2","AFG3 ATPase family gene 3-like 2 (yeast)","MitoCarta3.0","Protein import, sorting and homeostasis > Protein homeostasis > Proteases","MIM",2,"FtsH_ext | Peptidase_M41 | AAA",null,"EC_3.4.24.-","ENSFM00620000999395",2,null,"Ataxia, spastic, 5, autosomal recessive|Spinocerebellar ataxia 28","AFG3L2",1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,139,1,1,"Protein import, sorting and homeostasis > Protein homeostasis > Proteases","Protein import, sorting and homeostasis "," Protein homeostasis "," Proteases"]
["hsa:204",204,"AK2","ADK2|AK 2",1,"adenylate kinase 2","mitochondrion | mitochondrial inner membrane | mitochondrial intermembrane space","transferase activity | nucleotide binding | ATP binding | adenylate kinase activity","nucleobase-containing small molecule metabolic process | nucleobase-containing small molecule interconversion","AK2","adenylate kinase 2","MitoCarta3.0","Metabolism > Nucleotide metabolism > Nucleotide synthesis and processing","IMS",0,"ADK_lid | ADK","Purine metabolism",null,null,null,"FirstInEnsemblFamily","Reticular dysgenesis","AK2",1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,139,1,1,"Metabolism > Nucleotide metabolism > Nucleotide synthesis and processing","Metabolism "," Nucleotide metabolism "," Nucleotide synthesis and processing"]
["hsa:50808",50808,"AK3","AK3L1|AK6|AKL3L|AKL3L1|FIX",9,"adenylate kinase 3","mitochondrion | mitochondrial matrix","nucleoside triphosphate adenylate kinase activity | nucleobase-containing compound kinase activity | GTP binding | transferase activity | identical protein binding | nucleotide binding | ATP binding | adenylate kinase activity","blood coagulation | AMP phosphorylation | nucleobase-containing compound metabolic process","AK3","adenylate kinase 3","MitoCarta3.0","Metabolism > Nucleotide metabolism > Nucleotide synthesis and processing","Matrix",0,"ADK_lid | ADK","Pyrimidine metabolism","GTP:AMP PHOSPHOTRANSFERASE MITOCHONDRIAL EC_2.7.4.10 ADENYLATE KINASE 3 AK 3 ADENYLATE KINASE 3 ALPHA  1","ENSFM00500000270040",3,"FirstInEnsemblFamily",null,"AK3",1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,139,1,0,"Metabolism > Nucleotide metabolism > Nucleotide synthesis and processing","Metabolism "," Nucleotide metabolism "," Nucleotide synthesis and processing"]
["hsa:205",205,"AK4","AK3|AK3L1|AK3L2|MGC166959",1,"adenylate kinase 4","mitochondrion | mitochondrial matrix","GTP binding | transferase activity | nucleotide binding | ATP binding | adenylate kinase activity","nucleobase-containing compound metabolic process",null,null,"MitoCarta3.0","Metabolism > Nucleotide metabolism > Nucleotide synthesis and processing","Matrix",null,"ADK_lid | ADK","Purine metabolism","GTP:AMP PHOSPHOTRANSFERASE MITOCHONDRIAL EC_2.7.4.10 ADENYLATE KINASE 3 AK 3 ADENYLATE KINASE 3 ALPHA  1","ENSFM00500000270040",3,null,null,"AK4",1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,139,1,0,"Metabolism > Nucleotide metabolism > Nucleotide synthesis and processing","Metabolism "," Nucleotide metabolism "," Nucleotide synthesis and processing"]
["hsa:381",381,"ARF5","-",7,"ADP-ribosylation factor 5","Golgi apparatus | plasma membrane | intracellular | perinuclear region of cytoplasm | cytoplasm","protein binding | GTP binding | GTPase activity | nucleotide binding","vesicle-mediated transport | protein transport | small GTPase mediated signal transduction | GTP catabolic process","ARF5","ADP-ribosylation factor 5","MitoCarta3.0",0,"unknown",0,"Arf | Miro | Ras",null,"ADP RIBOSYLATION FACTOR","ENSFM00500000269612",6,null,null,"ARF5",1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,139,1,0,0,0]
["hsa:402",402,"ARL2","ARFL2",11,"ADP-ribosylation factor-like 2","mitochondrion | Golgi apparatus | lateral plasma membrane | centrosome | intracellular | mitochondrial intermembrane space | cytoplasm | cytosol | nucleus | cytoskeleton","GDP binding | protein binding | GTP binding | GTPase inhibitor activity | GTPase activity | nucleotide binding","centrosome organization | small GTPase mediated signal transduction | positive regulation of microtubule polymerization | regulation of microtubule polymerization | GTP catabolic process | negative regulation of GTPase activity | maintenance of protein location in nucleus | positive regulation of cell-substrate adhesion | acetylcholine transport | tubulin complex assembly | cell cycle | tight junction assembly","ARL2","ADP-ribosylation factor-like 2","MitoCarta3.0","Mitochondrial dynamics and surveillance > Fusion","IMS",0,"Arf | Miro",null,null,null,null,"FirstInEnsemblFamily",null,"ARL2",1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,139,1,0,"Mitochondrial dynamics and surveillance > Fusion","Mitochondrial dynamics and surveillance "," Fusion"]
["hsa:84896",84896,"ATAD1","AFDC1|FLJ14600",10,"ATPase family, AAA domain containing 1","mitochondrion | peroxisome","nucleoside-triphosphatase activity | nucleotide binding | ATP binding",null,"ATAD1","ATPase family, AAA domain containing 1","MitoCarta3.0","Protein import, sorting and homeostasis > Protein homeostasis > Proteases","MOM",0,"AAA",null,null,null,null,"FirstInEnsemblFamily",null,"ATAD1",1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,139,1,1,"Protein import, sorting and homeostasis > Protein homeostasis > Proteases","Protein import, sorting and homeostasis "," Protein homeostasis "," Proteases"]
["hsa:55210",55210,"ATAD3A","FLJ10709|FLJ35514",1,"ATPase family, AAA domain containing 3A",null,"nucleoside-triphosphatase activity | nucleotide binding | ATP binding",null,"ATAD3A","ATPase family, AAA domain containing 3A","MitoCarta3.0","Mitochondrial central dogma > mtDNA maintenance > mtDNA nucleoid | Mitochondrial dynamics and surveillance > Organelle contact sites | Mitochondrial dynamics and surveillance > Intramitochondrial membrane interactions","MIM",0,"AAA",null,"ATPASE FAMILY AAA DOMAIN CONTAINING 3","ENSFM00250000002488",3,"FirstInEnsemblFamily",null,"ATAD3A",1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,139,1,0,"Mitochondrial central dogma > mtDNA maintenance > mtDNA nucleoid | Mitochondrial dynamics and surveillance > Organelle contact sites | Mitochondrial dynamics and surveillance > Intramitochondrial membrane interactions","Mitochondrial central dogma "," mtDNA maintenance "," mtDNA nucleoid "," Mitochondrial dynamics and surveillance "," Organelle contact sites ",null," Mitochondrial dynamics and surveillance "," Intramitochondrial membrane interactions"]
["hsa:83858",83858,"ATAD3B","AAA-TOB3|KIAA1273|TOB3",1,"ATPase family, AAA domain containing 3B",null,"nucleoside-triphosphatase activity | nucleotide binding | ATP binding",null,"ATAD3B","ATPase family, AAA domain containing 3B","MitoCarta3.0","Mitochondrial central dogma > mtDNA maintenance > mtDNA nucleoid | Mitochondrial dynamics and surveillance > Organelle contact sites | Mitochondrial dynamics and surveillance > Intramitochondrial membrane interactions","MIM",0,"AAA",null,"ATPASE FAMILY AAA DOMAIN CONTAINING 3","ENSFM00250000002488",3,null,null,"ATAD3B",1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,139,1,0,"Mitochondrial central dogma > mtDNA maintenance > mtDNA nucleoid | Mitochondrial dynamics and surveillance > Organelle contact sites | Mitochondrial dynamics and surveillance > Intramitochondrial membrane interactions","Mitochondrial central dogma "," mtDNA maintenance "," mtDNA nucleoid "," Mitochondrial dynamics and surveillance "," Organelle contact sites ",null," Mitochondrial dynamics and surveillance "," Intramitochondrial membrane interactions"]
["hsa:498",498,"ATP5A1","ATP5A|ATP5AL2|ATPM|MOM2|OMR|ORM|hATP1",18,"ATP synthase, H+ transporting, mitochondrial F1 complex, alpha subunit 1, cardiac muscle","signalosome | mitochondrion | mitochondrial inner membrane | plasma membrane | mitochondrial matrix | mitochondrial proton-transporting ATP synthase complex | mitochondrial proton-transporting ATP synthase complex, catalytic core F(1)","ADP binding | hydrolase activity, acting on acid anhydrides, catalyzing transmembrane movement of substances | nucleotide binding | proton-transporting ATPase activity, rotational mechanism | ATP binding | protein binding | hydrogen ion transporting ATP synthase activity, rotational mechanism | transmembrane transporter activity | eukaryotic cell surface binding | ATPase activity | MHC class I protein binding","ADP biosynthetic process | embryo development | ion transport | respiratory electron transport chain | lipid metabolic process | negative regulation of endothelial cell proliferation | proton transport | ATP hydrolysis coupled proton transport | mitochondrial ATP synthesis coupled proton transport | ATP catabolic process | ATP biosynthetic process","ATP5A1","ATP synthase, H+ transporting, mitochondrial F1 complex, alpha subunit 1, cardiac muscle","MitoCarta3.0","OXPHOS > Complex V > CV subunits | OXPHOS > OXPHOS subunits","MIM",0,"ATP-synt_ab | ATP-synt_ab_N | ATP-synt_ab_C","Oxidative phosphorylation",null,null,null,"FirstInEnsemblFamily",null,"ATP5F1A",1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,139,1,1,"OXPHOS > Complex V > CV subunits | OXPHOS > OXPHOS subunits","OXPHOS "," Complex V "," CV subunits "," OXPHOS "," OXPHOS subunits"]
["hsa:506",506,"ATP5B","ATPMB|ATPSB|MGC5231",12,"ATP synthase, H+ transporting, mitochondrial F1 complex, beta polypeptide","mitochondrial nucleoid | mitochondrion | mitochondrial proton-transporting ATP synthase, catalytic core | mitochondrial membrane | plasma membrane | cell surface | mitochondrial matrix | mitochondrial proton-transporting ATP synthase complex","hydrolase activity | lipoprotein particle receptor activity | transporter activity | hydrolase activity, acting on acid anhydrides, catalyzing transmembrane movement of substances | nucleotide binding | proton-transporting ATPase activity, rotational mechanism | ATP binding | protein binding | hydrogen ion transporting ATP synthase activity, rotational mechanism | eukaryotic cell surface binding | transmembrane transporter activity | ATPase activity | MHC class I protein binding | hydrogen-exporting ATPase activity, phosphorylative mechanism | calcium ion binding","transport | ADP biosynthetic process | angiogenesis | ion transport | respiratory electron transport chain | lipid metabolic process | generation of precursor metabolites and energy | proton transport | negative regulation of cell adhesion involved in substrate-bound cell migration | receptor-mediated endocytosis | regulation of intracellular pH | ATP hydrolysis coupled proton transport | mitochondrial ATP synthesis coupled proton transport | ATP catabolic process | ATP biosynthetic process","ATP5B","ATP synthase, H+ transporting, mitochondrial F1 complex, beta polypeptide","MitoCarta3.0","OXPHOS > Complex V > CV subunits | OXPHOS > OXPHOS subunits","MIM",0,"ATP-synt_ab | ATP-synt_ab_N | ATP-synt_ab_C","Oxidative phosphorylation",null,null,null,"FirstInEnsemblFamily",null,"ATP5F1B",1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,139,1,1,"OXPHOS > Complex V > CV subunits | OXPHOS > OXPHOS subunits","OXPHOS "," Complex V "," CV subunits "," OXPHOS "," OXPHOS subunits"]
["hsa:617",617,"BCS1L","BCS|BCS1|BJS|FLNMS|GRACILE|Hs.6719|PTD|h-BCS",2,"BCS1-like (S. cerevisiae)","mitochondrion | integral to membrane | mitochondrial inner membrane | membrane | mitochondrial respiratory chain complex III","protein binding | nucleoside-triphosphatase activity | nucleotide binding | ATP binding","mitochondrial respiratory chain complex IV assembly | mitochondrion organization | mitochondrial respiratory chain complex III assembly | mitochondrial respiratory chain complex I assembly","BCS1L","BCS1-like (yeast)","MitoCarta3.0","OXPHOS > Complex III > CIII assembly factors | OXPHOS > OXPHOS assembly factors","MIM",0,"BCS1_N | AAA",null,null,null,null,"FirstInEnsemblFamily","Bjornstad syndrome|GRACILE syndrome|Mitochondrial complex III deficiency","BCS1L",1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,139,1,0,"OXPHOS > Complex III > CIII assembly factors | OXPHOS > OXPHOS assembly factors","OXPHOS "," Complex III "," CIII assembly factors "," OXPHOS "," OXPHOS assembly factors"]
["hsa:622",622,"BDH1","BDH|MGC2723|MGC4347|MGC9788|SDR9C1",3,"3-hydroxybutyrate dehydrogenase, type 1","mitochondrion | mitochondrial inner membrane | mitochondrial matrix","phospholipid binding | oxidoreductase activity | 3-hydroxybutyrate dehydrogenase activity | nucleotide binding","response to cadmium ion | ketone body biosynthetic process | response to toxin | response to growth hormone stimulus | response to drug | liver development | cellular ketone body metabolic process | response to hormone stimulus | ketone body catabolic process | adipose tissue development | response to ethanol | response to nutrient | metabolic process | cellular lipid metabolic process | response to starvation | response to estradiol stimulus | response to corticosterone stimulus | brain development | response to insulin stimulus","BDH1","3-hydroxybutyrate dehydrogenase, type 1","MitoCarta3.0","Metabolism > Carbohydrate metabolism > Ketone metabolism","MIM",0,"adh_short","Synthesis and degradation of ketone bodies | Butanoate metabolism",null,null,null,"FirstInEnsemblFamily",null,"BDH1",1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,139,1,0,"Metabolism > Carbohydrate metabolism > Ketone metabolism","Metabolism "," Carbohydrate metabolism "," Ketone metabolism"]
["hsa:84869",84869,"CBR4","FLJ14431|SDR45C1",4,"carbonyl reductase 4","mitochondrion | mitochondrial matrix","protein binding | quinone binding | oxidoreductase activity | NADPH dehydrogenase (quinone) activity | NADPH binding | nucleotide binding","protein homotetramerization | fatty acid biosynthetic process","CBR4","carbonyl reductase 4","MitoCarta3.0","Metabolism > Lipid metabolism > Type II fatty acid synthesis","Matrix",0,"KR | adh_short",null,null,null,null,"FirstInEnsemblFamily",null,"CBR4",1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,139,1,1,"Metabolism > Lipid metabolism > Type II fatty acid synthesis","Metabolism "," Lipid metabolism "," Type II fatty acid synthesis"]
["hsa:81570",81570,"CLPB","FLJ13152|HSP78|SKD3",11,"ClpB caseinolytic peptidase B homolog (E. coli)","mitochondrion | cellular_component","nucleoside-triphosphatase activity | molecular_function | nucleotide binding | ATP binding","cellular response to heat","CLPB","ClpB caseinolytic peptidase B homolog (E. coli)","MitoCarta3.0","Protein import, sorting and homeostasis > Protein homeostasis > Proteases","IMS",0,"Ank | AAA_2 | AAA_5",null,null,null,null,"FirstInEnsemblFamily",null,"CLPB",1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,139,1,1,"Protein import, sorting and homeostasis > Protein homeostasis > Proteases","Protein import, sorting and homeostasis "," Protein homeostasis "," Proteases"]
["hsa:10845",10845,"CLPX","-",15,"ClpX caseinolytic peptidase X homolog (E. coli)","mitochondrial nucleoid | mitochondrion | mitochondrial endopeptidase Clp complex | mitochondrial inner membrane","unfolded protein binding | peptidase activator activity | nucleoside-triphosphatase activity | ATP-dependent peptidase activity | nucleotide binding | ATP binding | protein binding | ATPase activity | metal ion binding","protein folding | proteolysis involved in cellular protein catabolic process | positive regulation of peptidase activity | ATP catabolic process","CLPX","ClpX caseinolytic peptidase X homolog (E. coli)","MitoCarta3.0","Protein import, sorting and homeostasis > Protein homeostasis > Proteases","Matrix",0,"AAA | AAA_2",null,null,null,null,"FirstInEnsemblFamily",null,"CLPX",1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,139,1,1,"Protein import, sorting and homeostasis > Protein homeostasis > Proteases","Protein import, sorting and homeostasis "," Protein homeostasis "," Proteases"]
["hsa:55157",55157,"DARS2","ASPRS|FLJ10514|LBSL|MT-ASPRS|RP3-383J4.2",1,"aspartyl-tRNA synthetase 2, mitochondrial","mitochondrion | nucleus | mitochondrial matrix | cytoplasm","aspartate-tRNA(Asn) ligase activity | tRNA binding | protein homodimerization activity | aspartate-tRNA ligase activity | aminoacyl-tRNA ligase activity | nucleotide binding | ligase activity | ATP binding | nucleic acid binding","mitochondrial asparaginyl-tRNA aminoacylation | tRNA aminoacylation | gene expression | tRNA aminoacylation for protein translation","DARS2","aspartyl-tRNA synthetase 2 (mitochondrial)","MitoCarta3.0","Mitochondrial central dogma > Translation > mt-tRNA synthetases","Matrix",0,"tRNA-synt_2 | GAD | tRNA_anti","Aminoacyl-tRNA biosynthesis",null,null,null,"FirstInEnsemblFamily","Leukoencephalopathy with brain stem and spinal cord involvement and lactate elevation","DARS2",1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,139,1,1,"Mitochondrial central dogma > Translation > mt-tRNA synthetases","Mitochondrial central dogma "," Translation "," mt-tRNA synthetases"]
["hsa:55794",55794,"DDX28","FLJ11282|MDDX28",16,"DEAD (Asp-Glu-Ala-Asp) box polypeptide 28","mitochondrial nucleoid | mitochondrion | nucleus","hydrolase activity | RNA binding | helicase activity | nucleotide binding | ATP-dependent helicase activity | ATP binding",null,"DDX28","DEAD (Asp-Glu-Ala-Asp) box polypeptide 28","MitoCarta3.0","Mitochondrial central dogma > mtRNA metabolism > mtRNA granules | Mitochondrial central dogma > Translation > Mitochondrial ribosome assembly","Matrix",0,"Helicase_C | DEAD",null,null,null,null,"FirstInEnsemblFamily",null,"DDX28",1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,139,1,1,"Mitochondrial central dogma > mtRNA metabolism > mtRNA granules | Mitochondrial central dogma > Translation > Mitochondrial ribosome assembly","Mitochondrial central dogma "," mtRNA metabolism "," mtRNA granules "," Mitochondrial central dogma "," Translation "," Mitochondrial ribosome assembly"]
["hsa:25979",25979,"DHRS7B","DKFZp566O084|MGC8916|SDR32C1",17,"dehydrogenase/reductase (SDR family) member 7B","integral to membrane | peroxisomal membrane | peroxisome | membrane","oxidoreductase activity | nucleotide binding",null,"DHRS7B","dehydrogenase/reductase (SDR family) member 7B","MitoCarta3.0",0,"Membrane",1,"KR | adh_short",null,"DEHYDROGENASE/REDUCTASE SDR FAMILY MEMBER EC_1.1.-.-","ENSFM00560000771026",2,"FirstInEnsemblFamily",null,"DHRS7B",1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,139,1,0,0,0]
["hsa:22907",22907,"DHX30","DDX30|FLJ11214|KIAA0890|RETCOR",3,"DEAH (Asp-Glu-Ala-His) box polypeptide 30","mitochondrial nucleoid | mitochondrion | cytoplasm","hydrolase activity | protein binding | chromatin binding | RNA binding | helicase activity | nucleotide binding | ATP-dependent helicase activity | ATP binding",null,"DHX30","DEAH (Asp-Glu-Ala-His) box polypeptide 30","MitoCarta3.0","Mitochondrial central dogma > mtRNA metabolism > mtRNA granules | Mitochondrial central dogma > Translation > Mitochondrial ribosome assembly","Matrix",0,"DUF1605 | dsrm | HA2 | DEAD | Helicase_C",null,"ATP DEPENDENT RNA HELICASE EC_3.6.4.13 DEAH BOX","ENSFM00260000050346",5,null,null,"DHX30",1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,139,1,0,"Mitochondrial central dogma > mtRNA metabolism > mtRNA granules | Mitochondrial central dogma > Translation > Mitochondrial ribosome assembly","Mitochondrial central dogma "," mtRNA metabolism "," mtRNA granules "," Mitochondrial central dogma "," Translation "," Mitochondrial ribosome assembly"]
["hsa:1760",1760,"DMPK","DM|DM1|DM1PK|DMK|MDPK|MT-PK",19,"dystrophia myotonica-protein kinase",null,"protein binding | protein serine/threonine kinase activity | protein kinase activity | nucleotide binding | metal ion binding | ATP binding","protein modification process | regulation of sodium ion transport | regulation of excitatory postsynaptic membrane potential involved in skeletal muscle contraction | regulation of heart contraction | protein phosphorylation","DMPK","dystrophia myotonica-protein kinase","MitoCarta3.0",0,"MOM",0,"DMPK_coil | Pkinase | Pkinase_C",null,"SERINE/THREONINE KINASE MRCK EC_2.7.11.1 CDC42 BINDING KINASE DMPK  MYOTONIC DYSTROPHY KINASE RELATED CDC42 BINDING KINASE MRCK MYOTONIC DYSTROPHY KINASE","ENSFM00540000717914",4,"FirstInEnsemblFamily","Myotonic dystrophy 1","DMPK",1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,139,1,0,0,0]
["hsa:1763",1763,"DNA2","DNA2L|FLJ10063|KIAA0083|MGC133297",10,"DNA replication helicase 2 homolog (yeast)","mitochondrial nucleoid | gamma DNA polymerase complex | nucleoplasm | nucleus","hydrolase activity | site-specific endodeoxyribonuclease activity, specific for altered base | helicase activity | 5'-flap endonuclease activity | DNA binding | nucleotide binding | ATP binding | ATP-dependent DNA helicase activity | ATPase activity","telomere maintenance via semi-conservative replication | S phase of mitotic cell cycle | base-excision repair | mitotic cell cycle | mitochondrial DNA repair | telomere maintenance via recombination | DNA replication, removal of RNA primer | mitochondrial DNA replication | DNA strand elongation involved in DNA replication | telomere maintenance | ATP catabolic process | positive regulation of DNA replication","DNA2L","DNA2 DNA replication helicase 2-like (yeast)","MitoCarta3.0","Mitochondrial central dogma > mtDNA maintenance > mtDNA replication | Metabolism > Metals and cofactors > Fe-S-containing proteins","Matrix",0,"Dna2","DNA replication",null,null,null,"FirstInEnsemblFamily",null,"DNA2",1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,139,1,1,"Mitochondrial central dogma > mtDNA maintenance > mtDNA replication | Metabolism > Metals and cofactors > Fe-S-containing proteins","Mitochondrial central dogma "," mtDNA maintenance "," mtDNA replication "," Metabolism "," Metals and cofactors "," Fe-S-containing proteins"]
["hsa:9093",9093,"DNAJA3","FLJ45758|HCA57|TID1|hTID-1",16,"DnaJ (Hsp40) homolog, subfamily A, member 3","IkappaB kinase complex | cytoplasm | cytosol | mitochondrial nucleoid | actin filament | nucleus | mitochondrial matrix | I-kappaB/NF-kappaB complex","unfolded protein binding | interferon-gamma receptor binding | protein kinase binding | small GTPase regulator activity | NF-kappaB binding | ATP binding | protein binding | heat shock protein binding | transcription factor binding | metal ion binding","neuromuscular junction development | negative regulation of cell proliferation | positive regulation of apoptosis | negative regulation of NF-kappaB transcription factor activity | embryo development | negative regulation of I-kappaB kinase/NF-kappaB cascade | T cell differentiation in thymus | apoptosis | negative regulation of interferon-gamma-mediated signaling pathway | protein folding | positive regulation of protein ubiquitination | mitochondrial DNA replication | activation-induced cell death of T cells | cell death | negative regulation of protein kinase activity | activation of caspase activity | protein stabilization | mitochondrion organization | small GTPase mediated signal transduction | cell aging | negative regulation of caspase activity | negative regulation of apoptosis | response to interferon-gamma | negative regulation of transcription from RNA polymerase II promoter | positive regulation of T cell proliferation | response to heat","DNAJA3","DnaJ (Hsp40) homolog, subfamily A, member 3","MitoCarta3.0","Protein import, sorting and homeostasis > Protein homeostasis > Chaperones","Matrix",0,"DnaJ_C | DnaJ_CXXCXGXG | DnaJ",null,null,null,null,"FirstInEnsemblFamily",null,"DNAJA3",1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,139,1,1,"Protein import, sorting and homeostasis > Protein homeostasis > Chaperones","Protein import, sorting and homeostasis "," Protein homeostasis "," Chaperones"]
["hsa:55735",55735,"DNAJC11","FLJ10737|dJ126A5.1",1,"DnaJ (Hsp40) homolog, subfamily C, member 11","mitochondrion | mitochondrial inner membrane","unfolded protein binding | heat shock protein binding","protein folding","DNAJC11","DnaJ (Hsp40) homolog, subfamily C, member 11","MitoCarta3.0","Protein import, sorting and homeostasis > Protein import and sorting | Mitochondrial dynamics and surveillance > Intramitochondrial membrane interactions","MOM",0,"DnaJ",null,null,null,null,"FirstInEnsemblFamily",null,"DNAJC11",1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,139,1,1,"Protein import, sorting and homeostasis > Protein import and sorting | Mitochondrial dynamics and surveillance > Intramitochondrial membrane interactions","Protein import, sorting and homeostasis "," Protein import and sorting ",null," Mitochondrial dynamics and surveillance "," Intramitochondrial membrane interactions"]
["hsa:84277",84277,"DNAJC30","MGC12943|WBSCR18",7,"DnaJ (Hsp40) homolog, subfamily C, member 30","mitochondrion","unfolded protein binding | heat shock protein binding","protein folding","WBSCR18","Williams Beuren syndrome chromosome region 18","MitoCarta3.0",0,"MIM",1,"DnaJ",null,null,null,null,"FirstInEnsemblFamily",null,"DNAJC30",1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,139,1,0,0,0]
["hsa:3338",3338,"DNAJC4","DANJC4|HSPF2|MCG18|MGC19482|MGC57189|MGC71863",11,"DnaJ (Hsp40) homolog, subfamily C, member 4","integral to membrane | membrane | membrane fraction","unfolded protein binding | heat shock protein binding","protein folding | response to unfolded protein","DNAJC4","DnaJ (Hsp40) homolog, subfamily C, member 4","MitoCarta3.0","Protein import, sorting and homeostasis > Protein homeostasis","Membrane",1,"DnaJ",null,null,null,null,"FirstInEnsemblFamily",null,"DNAJC4",1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,139,1,0,"Protein import, sorting and homeostasis > Protein homeostasis","Protein import, sorting and homeostasis "," Protein homeostasis"]
["hsa:54920",54920,"DUS2L","DUS2|FLJ20399|SMM1|URLC8",16,"dihydrouridine synthase 2-like, SMM1 homolog (S. cerevisiae)","mitochondrion | endoplasmic reticulum | intracellular | cytoplasm","double-stranded RNA binding | oxidoreductase activity | tRNA dihydrouridine synthase activity | flavin adenine dinucleotide binding","tRNA processing","DUS2L","dihydrouridine synthase 2-like, SMM1 homolog (S. cerevisiae)","MitoCarta3.0","Mitochondrial central dogma > mtRNA metabolism > mt-tRNA modifications","Matrix",0,"dsrm | Dus",null,null,null,null,"FirstInEnsemblFamily",null,"DUS2",1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,139,1,0,"Mitochondrial central dogma > mtRNA metabolism > mt-tRNA modifications","Mitochondrial central dogma "," mtRNA metabolism "," mt-tRNA modifications"]
["hsa:124454",124454,"EARS2","KIAA1970|MSE1",16,"glutamyl-tRNA synthetase 2, mitochondrial (putative)","mitochondrion | mitochondrial matrix | cytoplasm","glutamate-tRNA(Gln) ligase activity | RNA binding | glutamate-tRNA ligase activity | nucleotide binding | ligase activity | ATP binding","glutamyl-tRNA aminoacylation | gene expression | tRNA aminoacylation for mitochondrial protein translation | tRNA aminoacylation for protein translation","EARS2","glutamyl-tRNA synthetase 2 (mitochondrial)(putative)","MitoCarta3.0","Mitochondrial central dogma > Translation > mt-tRNA synthetases","Matrix",0,"tRNA-synt_1c","Porphyrin and chlorophyll metabolism | Aminoacyl-tRNA biosynthesis",null,null,null,"FirstInEnsemblFamily",null,"EARS2",1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,139,1,1,"Mitochondrial central dogma > Translation > mt-tRNA synthetases","Mitochondrial central dogma "," Translation "," mt-tRNA synthetases"]
["hsa:23770",23770,"FKBP8","FKBP38|FKBPr38",19,"FK506 binding protein 8, 38kDa","mitochondrial envelope | integral to endoplasmic reticulum membrane | mitochondrion | integral to membrane | mitochondrial membrane | intracellular | membrane","isomerase activity | FK506 binding | protein binding | peptidyl-prolyl cis-trans isomerase activity","protein folding | protein peptidyl-prolyl isomerization | interspecies interaction between organisms | peptidyl-proline modification | apoptosis | intracellular signal transduction","FKBP8","FK506 binding protein 8, 38kDa","MitoCarta3.0","Mitochondrial dynamics and surveillance > Organelle contact sites | Mitochondrial dynamics and surveillance > Mitophagy | Mitochondrial dynamics and surveillance > Autophagy","MOM",1,"TPR_1 | FKBP_C | TPR_2",null,null,null,null,"FirstInEnsemblFamily",null,"FKBP8",1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,139,1,0,"Mitochondrial dynamics and surveillance > Organelle contact sites | Mitochondrial dynamics and surveillance > Mitophagy | Mitochondrial dynamics and surveillance > Autophagy","Mitochondrial dynamics and surveillance "," Organelle contact sites ",null," Mitochondrial dynamics and surveillance "," Mitophagy ",null," Mitochondrial dynamics and surveillance "," Autophagy"]
["hsa:29960",29960,"FTSJ2","DKFZp686J14194|FJH1",7,"FtsJ homolog 2 (E. coli)","mitochondrion | nucleolus | nucleus","methyltransferase activity | rRNA (uridine-2'-O-)-methyltransferase activity | transferase activity | nucleic acid binding","cell proliferation | rRNA methylation | rRNA processing","FTSJ2","FtsJ homolog 2 (E. coli)","MitoCarta3.0","Mitochondrial central dogma > mtRNA metabolism > mtRNA granules | Mitochondrial central dogma > mtRNA metabolism > mt-rRNA modifications | Mitochondrial central dogma > Translation > Mitochondrial ribosome assembly","Matrix",0,"FtsJ",null,null,null,null,"FirstInEnsemblFamily",null,"MRM2",1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,139,1,0,"Mitochondrial central dogma > mtRNA metabolism > mtRNA granules | Mitochondrial central dogma > mtRNA metabolism > mt-rRNA modifications | Mitochondrial central dogma > Translation > Mitochondrial ribosome assembly","Mitochondrial central dogma "," mtRNA metabolism "," mtRNA granules "," Mitochondrial central dogma "," mtRNA metabolism "," mt-rRNA modifications "," Mitochondrial central dogma "," Translation "," Mitochondrial ribosome assembly"]
["hsa:2617",2617,"GARS","CMT2D|DSMAV|GlyRS|HMN5|SMAD1",7,"glycyl-tRNA synthetase","cytosol | mitochondrion | stored secretory granule | soluble fraction | mitochondrial matrix | cytoplasm","protein dimerization activity | glycine-tRNA ligase activity | nucleotide binding | ligase activity | ATP binding","diadenosine tetraphosphate biosynthetic process | cell death | gene expression | tRNA aminoacylation for protein translation | glycyl-tRNA aminoacylation","GARS","glycyl-tRNA synthetase","MitoCarta3.0","Mitochondrial central dogma > Translation > mt-tRNA synthetases","Matrix",0,"tRNA-synt_2b | HGTP_anticodon | WHEP-TRS","Aminoacyl-tRNA biosynthesis",null,null,null,"FirstInEnsemblFamily","Charcot-Marie-Tooth disease, type 2D|\"Neuropathy, distal hereditary motor, type V\"","GARS1",1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,139,1,1,"Mitochondrial central dogma > Translation > mt-tRNA synthetases","Mitochondrial central dogma "," Translation "," mt-tRNA synthetases"]
["hsa:85476",85476,"GFM1","COXPD1|EFG|EFG1|EFGM|EGF1|FLJ12662|FLJ13632|FLJ20773|GFM|hEFG1",3,"G elongation factor, mitochondrial 1","mitochondrion | intracellular","GTP binding | GTPase activity | translation elongation factor activity | nucleotide binding","mitochondrial translational elongation | GTP catabolic process","GFM1","G elongation factor, mitochondrial 1","MitoCarta3.0","Mitochondrial central dogma > Translation > Translation factors","Matrix",0,"EFG_C | GTP_EFTU_D2 | GTP_EFTU | EFG_IV",null,"ELONGATION FACTOR G MITOCHONDRIAL PRECURSOR EF GMT ELONGATION FACTOR G 1 MITOCHONDRIAL MEF G 1 ELONGATION FACTOR G1","ENSFM00250000001606",2,null,"Combined oxidative phosphorylation deficiency 1","GFM1",1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,139,1,1,"Mitochondrial central dogma > Translation > Translation factors","Mitochondrial central dogma "," Translation "," Translation factors"]
["hsa:84340",84340,"GFM2","EF-G2mt|EFG2|MRRF2|MST027|RRF2|RRF2mt|hEFG2|mEF-G 2",5,"G elongation factor, mitochondrial 2","mitochondrion","GTP binding | GTPase activity | translation elongation factor activity | nucleotide binding","ribosome disassembly | mitochondrial translational elongation | GTP catabolic process | mitochondrial translation","GFM2","G elongation factor, mitochondrial 2","MitoCarta3.0","Mitochondrial central dogma > Translation > Translation factors","Matrix",0,"EFG_C | GTP_EFTU_D2 | GTP_EFTU | EFG_IV",null,"ELONGATION FACTOR G MITOCHONDRIAL PRECURSOR EF GMT ELONGATION FACTOR G 1 MITOCHONDRIAL MEF G 1 ELONGATION FACTOR G1","ENSFM00250000001606",2,"FirstInEnsemblFamily",null,"GFM2",1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,139,1,0,"Mitochondrial central dogma > Translation > Translation factors","Mitochondrial central dogma "," Translation "," Translation factors"]
["hsa:2820",2820,"GPD2","GDH2|GPDM|mGPDH",2,"glycerol-3-phosphate dehydrogenase 2 (mitochondrial)","mitochondrion | mitochondrial inner membrane | glycerol-3-phosphate dehydrogenase complex","oxidoreductase activity | glycerol-3-phosphate dehydrogenase activity | glycerol-3-phosphate dehydrogenase [NAD+] activity | sn-glycerol-3-phosphate:ubiquinone-8 oxidoreductase activity | calcium ion binding","gluconeogenesis | glycerol-3-phosphate metabolic process | cellular lipid metabolic process | NADH metabolic process","GPD2","glycerol-3-phosphate dehydrogenase 2 (mitochondrial)","MitoCarta3.0","Metabolism > Carbohydrate metabolism > Glycerol phosphate shuttle | Metabolism > Electron carriers > Q-linked reactions, other","MIM",1,"DAO | efhand","Glycerophospholipid metabolism",null,null,null,"FirstInEnsemblFamily",null,"GPD2",1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,139,1,1,"Metabolism > Carbohydrate metabolism > Glycerol phosphate shuttle | Metabolism > Electron carriers > Q-linked reactions, other","Metabolism "," Carbohydrate metabolism "," Glycerol phosphate shuttle "," Metabolism "," Electron carriers "," Q-linked reactions, other"]
["hsa:2936",2936,"GSR","MGC78522",8,"glutathione reductase","cytosol | mitochondrion | soluble fraction | cytoplasm","electron carrier activity | glutathione-disulfide reductase activity | oxidoreductase activity | NADP binding | glutathione binding | protein homodimerization activity | flavin adenine dinucleotide binding","nucleobase-containing small molecule metabolic process | spermatogenesis | glutathione metabolic process | cell redox homeostasis | nucleobase-containing small molecule interconversion","GSR","glutathione reductase","MitoCarta3.0","Metabolism > Detoxification > ROS and glutathione metabolism","Matrix",0,"Pyr_redox | Pyr_redox_dim | Pyr_redox_2","Glutathione metabolism",null,null,null,"FirstInEnsemblFamily",null,"GSR",1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,139,1,1,"Metabolism > Detoxification > ROS and glutathione metabolism","Metabolism "," Detoxification "," ROS and glutathione metabolism"]
["hsa:85865",85865,"GTPBP10","DKFZp686A10121|FLJ38242|MGC104191|ObgH2",7,"GTP-binding protein 10 (putative)","mitochondrion | nucleolus | chromosome | nucleus | intracellular","GTP binding | magnesium ion binding | GTPase activity | nucleotide binding","ribosome biogenesis",null,null,"MitoCarta3.0","Mitochondrial central dogma > Translation > Mitochondrial ribosome assembly","Matrix",null,"MMR_HSR1 | GTP1_OBG",null,null,null,null,"FirstInEnsemblFamily",null,"GTPBP10",1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,139,1,0,"Mitochondrial central dogma > Translation > Mitochondrial ribosome assembly","Mitochondrial central dogma "," Translation "," Mitochondrial ribosome assembly"]
["hsa:26164",26164,"GTPBP5","FLJ10741|MGC29512|ObgH1|dJ1005F21.2",20,"GTP binding protein 5 (putative)","mitochondrion | intracellular","GTP binding | magnesium ion binding | GTPase activity | nucleotide binding","ribosome biogenesis","GTPBP5","GTP binding protein 5 (putative)","MitoCarta3.0","Mitochondrial central dogma > Translation > Mitochondrial ribosome assembly","Matrix",0,"MMR_HSR1 | GTP1_OBG",null,null,null,null,"FirstInEnsemblFamily",null,"MTG2",1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,139,1,1,"Mitochondrial central dogma > Translation > Mitochondrial ribosome assembly","Mitochondrial central dogma "," Translation "," Mitochondrial ribosome assembly"]
["hsa:60558",60558,"GUF1","EF-4|FLJ13220",4,"GUF1 GTPase homolog (S. cerevisiae)","mitochondrion | mitochondrial inner membrane | membrane","hydrolase activity | GTP binding | GTPase activity | nucleotide binding","translation","GUF1","GUF1 GTPase homolog (S. cerevisiae)","MitoCarta3.0","Mitochondrial central dogma > Translation","Matrix",0,"EFG_C | LepA_C | GTP_EFTU_D2 | GTP_EFTU",null,null,null,null,"FirstInEnsemblFamily",null,"GUF1",1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,139,1,1,"Mitochondrial central dogma > Translation","Mitochondrial central dogma "," Translation"]
["hsa:2987",2987,"GUK1","FLJ42686|FLJ43710|GMK",1,"guanylate kinase 1","cytosol","transferase activity | guanylate kinase activity | kinase activity | nucleotide binding | ATP binding","nucleobase-containing small molecule metabolic process | purine nucleotide metabolic process | nucleobase-containing small molecule interconversion","GUK1","guanylate kinase 1","MitoCarta3.0","Metabolism > Nucleotide metabolism > Nucleotide synthesis and processing","Matrix",0,"Guanylate_kin","Purine metabolism",null,null,null,"FirstInEnsemblFamily",null,"GUK1",1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,139,1,1,"Metabolism > Nucleotide metabolism > Nucleotide synthesis and processing","Metabolism "," Nucleotide metabolism "," Nucleotide synthesis and processing"]
["hsa:23438",23438,"HARS2","HARSL|HARSR|HO3",5,"histidyl-tRNA synthetase 2, mitochondrial (putative)","mitochondrion | mitochondrial matrix | cytoplasm","histidine-tRNA ligase activity | nucleotide binding | ligase activity | ATP binding","histidyl-tRNA aminoacylation | gene expression | translation | tRNA aminoacylation for protein translation","HARSL","histidyl-tRNA synthetase-like","MitoCarta3.0","Mitochondrial central dogma > Translation > mt-tRNA synthetases","Matrix",0,"tRNA-synt_2b | HGTP_anticodon","Aminoacyl-tRNA biosynthesis","HISTIDYL TRNA SYNTHETASE EC_6.1.1.21 HISTIDINE TRNA LIGASE HISRS","ENSFM00250000002869",2,"FirstInEnsemblFamily",null,"HARS2",1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,139,1,0,"Mitochondrial central dogma > Translation > mt-tRNA synthetases","Mitochondrial central dogma "," Translation "," mt-tRNA synthetases"]
["hsa:3329",3329,"HSPD1","CPN60|GROEL|HLD4|HSP-60|HSP60|HSP65|HuCHA60|SPG13",2,"heat shock 60kDa protein 1 (chaperonin)","mitochondrion | stored secretory granule | cyclin-dependent protein kinase activating kinase holoenzyme complex | mitochondrial inner membrane | plasma membrane part | early endosome | cytoplasm | cytosol | coated pit | extracellular space | coated vesicle | lipopolysaccharide receptor complex | cell surface | mitochondrial matrix","unfolded protein binding | DNA replication origin binding | single-stranded DNA binding | cell surface binding | p53 binding | lipopolysaccharide binding | nucleotide binding | ATP binding | protein binding | ATPase activity | chaperone binding","T cell activation | B cell activation | positive regulation of apoptosis | positive regulation of interleukin-12 production | positive regulation of interleukin-10 production | positive regulation of macrophage activation | B cell cytokine production | cell death | response to unfolded protein | interspecies interaction between organisms | MyD88-dependent toll-like receptor signaling pathway | protein maturation | ATP catabolic process | isotype switching to IgG isotypes | activation of caspase activity | positive regulation of interleukin-6 production | chaperone-mediated protein complex assembly | protein stabilization | positive regulation of interferon-alpha production | B cell proliferation | positive regulation of T cell activation | positive regulation of T cell mediated immune response to tumor cell | negative regulation of apoptosis | protein refolding | 'de novo' protein folding | positive regulation of interferon-gamma production","HSPD1","heat shock 60kDa protein 1 (chaperonin)","MitoCarta3.0","Protein import, sorting and homeostasis > Protein homeostasis > Chaperones","Matrix",0,"Cpn60_TCP1","RNA degradation",null,null,null,"FirstInEnsemblFamily","Leukodystrophy, hypomyelinating, 4|\"Spastic paraplegia 13, autosomal dominant\"","HSPD1",1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,139,1,1,"Protein import, sorting and homeostasis > Protein homeostasis > Chaperones","Protein import, sorting and homeostasis "," Protein homeostasis "," Chaperones"]
["hsa:3735",3735,"KARS","CMTRIB|KARS2|KIAA0070|KRS",16,"lysyl-tRNA synthetase","mitochondrion | cytoplasm | cytosol | extracellular region | soluble fraction | nucleus | plasma membrane | mitochondrial matrix","tRNA binding | nucleotide binding | metal ion binding | ligase activity | ATP binding | lysine-tRNA ligase activity | nucleic acid binding","tRNA processing | lysyl-tRNA aminoacylation | interspecies interaction between organisms | gene expression | tRNA aminoacylation for protein translation","KARS","lysyl-tRNA synthetase","MitoCarta3.0","Mitochondrial central dogma > Translation > mt-tRNA synthetases","Matrix",0,"tRNA-synt_2 | tRNA_anti","Aminoacyl-tRNA biosynthesis",null,null,null,"FirstInEnsemblFamily","Charcot-Marie-Tooth disease, recessive intermediate, B","KARS1",1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,139,1,1,"Mitochondrial central dogma > Translation > mt-tRNA synthetases","Mitochondrial central dogma "," Translation "," mt-tRNA synthetases"]
["hsa:9361",9361,"LONP1","LON|LONP|LonHS|MGC1498|PIM1|PRSS15|hLON",19,"lon peptidase 1, mitochondrial","mitochondrial nucleoid | mitochondrion | mitochondrial matrix | cytoplasm","ADP binding | mitochondrial heavy strand promoter anti-sense binding | single-stranded RNA binding | sequence-specific DNA binding | mitochondrial light strand promoter anti-sense binding | ATP binding | peptidase activity | ATPase activity | mitochondrial heavy strand promoter sense binding | single-stranded DNA binding | ATP-dependent peptidase activity | nucleotide binding | mitochondrial light strand promoter sense binding | protein binding | serine-type endopeptidase activity | G-quadruplex DNA binding | DNA polymerase binding","mitochondrion organization | misfolded or incompletely synthesized protein catabolic process | cellular chaperone-mediated protein complex assembly | response to hypoxia | mitochondrial genome maintenance | oxidation-dependent protein catabolic process | cellular response to oxidative stress | proteolysis involved in cellular protein catabolic process | protein homooligomerization | mitochondrial DNA metabolic process","LONP1","lon peptidase 1, mitochondrial","MitoCarta3.0","Protein import, sorting and homeostasis > Protein homeostasis > Proteases","Matrix",0,"Lon_C | LON | AAA | AAA_5",null,"LON PROTEASE HOMOLOG EC_3.4.21.-","ENSFM00250000001712",2,"FirstInEnsemblFamily",null,"LONP1",1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,139,1,1,"Protein import, sorting and homeostasis > Protein homeostasis > Proteases","Protein import, sorting and homeostasis "," Protein homeostasis "," Proteases"]
["hsa:64965",64965,"MRPS9","MRP-S9|RPMS9|S9mt",2,"mitochondrial ribosomal protein S9","mitochondrion | ribosome | intracellular | mitochondrial small ribosomal subunit","structural constituent of ribosome","peptide biosynthetic process | DNA damage response, detection of DNA damage | translation","MRPS9","mitochondrial ribosomal protein S9","MitoCarta3.0","Mitochondrial central dogma > mtRNA metabolism > mtRNA granules | Mitochondrial central dogma > Translation > Mitochondrial ribosome","Matrix",0,"Ribosomal_S9",null,null,null,null,"FirstInEnsemblFamily",null,"MRPS9",1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,139,1,1,"Mitochondrial central dogma > mtRNA metabolism > mtRNA granules | Mitochondrial central dogma > Translation > Mitochondrial ribosome","Mitochondrial central dogma "," mtRNA metabolism "," mtRNA granules "," Mitochondrial central dogma "," Translation "," Mitochondrial ribosome"]
["hsa:92170",92170,"MTG1","GTP|GTPBP7|RP11-108K14.2",10,"mitochondrial GTPase 1 homolog (S. cerevisiae)","mitochondrion | intracellular","GTP binding | nucleotide binding",null,"MTG1","mitochondrial GTPase 1 homolog (S. cerevisiae)","MitoCarta3.0","Mitochondrial central dogma > Translation > Mitochondrial ribosome assembly","Matrix",0,null,null,null,null,null,"FirstInEnsemblFamily",null,"MTG1",1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,139,1,1,"Mitochondrial central dogma > Translation > Mitochondrial ribosome assembly","Mitochondrial central dogma "," Translation "," Mitochondrial ribosome assembly"]
["hsa:4528",4528,"MTIF2","-",2,"mitochondrial translational initiation factor 2","mitochondrion | intracellular","ribosomal small subunit binding | translation factor activity, nucleic acid binding | GTP binding | GTPase activity | nucleotide binding | translation initiation factor activity","regulation of translational initiation | translation","MTIF2","mitochondrial translational initiation factor 2","MitoCarta3.0","Mitochondrial central dogma > Translation > Translation factors","Matrix",0,"Miro | GTP_EFTU_D2 | GTP_EFTU",null,null,null,null,"FirstInEnsemblFamily",null,"MTIF2",1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,139,1,1,"Mitochondrial central dogma > Translation > Translation factors","Mitochondrial central dogma "," Translation "," Translation factors"]
["hsa:79731",79731,"NARS2","FLJ23441|SLM5|asnRS",11,"asparaginyl-tRNA synthetase 2, mitochondrial (putative)","mitochondrion | mitochondrial matrix | cytoplasm","asparagine-tRNA ligase activity | nucleotide binding | ligase activity | ATP binding | nucleic acid binding","asparaginyl-tRNA aminoacylation | gene expression | tRNA aminoacylation for protein translation","NARS2","asparaginyl-tRNA synthetase 2 (mitochondrial)(putative)","MitoCarta3.0","Mitochondrial central dogma > Translation > mt-tRNA synthetases","Matrix",0,"tRNA-synt_2 | tRNA_anti","Aminoacyl-tRNA biosynthesis",null,null,null,"FirstInEnsemblFamily",null,"NARS2",1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,139,1,1,"Mitochondrial central dogma > Translation > mt-tRNA synthetases","Mitochondrial central dogma "," Translation "," mt-tRNA synthetases"]
["hsa:54888",54888,"NSUN2","FLJ20303|MISU|SAKI|TRM4",5,"NOP2/Sun domain family, member 2","nucleolus | nucleus | cytoplasm","tRNA (cytosine-5-)-methyltransferase activity | methyltransferase activity | tRNA binding | transferase activity","tRNA processing | tRNA methylation","NSUN2","NOL1/NOP2/Sun domain family, member 2","MitoCarta3.0","Mitochondrial central dogma > mtRNA metabolism > mt-tRNA modifications","Matrix",0,"Nol1_Nop2_Fmu",null,null,null,null,"FirstInEnsemblFamily","Mental retardation, autosomal recessive 5","NSUN2",1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,139,1,1,"Mitochondrial central dogma > mtRNA metabolism > mt-tRNA modifications","Mitochondrial central dogma "," mtRNA metabolism "," mt-tRNA modifications"]
["hsa:63899",63899,"NSUN3","FLJ22109|FLJ22609|MST077",3,"NOP2/Sun domain family, member 3",null,"methyltransferase activity | transferase activity",null,"NSUN3","NOL1/NOP2/Sun domain family, member 3","MitoCarta3.0","Mitochondrial central dogma > mtRNA metabolism > mt-tRNA modifications","Matrix",0,"Nol1_Nop2_Fmu",null,null,null,null,"FirstInEnsemblFamily",null,"NSUN3",1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,139,1,0,"Mitochondrial central dogma > mtRNA metabolism > mt-tRNA modifications","Mitochondrial central dogma "," mtRNA metabolism "," mt-tRNA modifications"]
["hsa:387338",387338,"NSUN4","MGC22960|RP4-603I14.2",1,"NOP2/Sun domain family, member 4",null,"methyltransferase activity | transferase activity",null,"NSUN4","NOL1/NOP2/Sun domain family, member 4","MitoCarta3.0","Mitochondrial central dogma > mtRNA metabolism > mt-rRNA modifications | Mitochondrial central dogma > Translation > Mitochondrial ribosome assembly","Matrix",0,"Nol1_Nop2_Fmu",null,null,null,null,"FirstInEnsemblFamily",null,"NSUN4",1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,139,1,0,"Mitochondrial central dogma > mtRNA metabolism > mt-rRNA modifications | Mitochondrial central dogma > Translation > Mitochondrial ribosome assembly","Mitochondrial central dogma "," mtRNA metabolism "," mt-rRNA modifications "," Mitochondrial central dogma "," Translation "," Mitochondrial ribosome assembly"]
["hsa:4913",4913,"NTHL1","NTH1|OCTS3",16,"nth endonuclease III-like 1 (E. coli)","nucleoplasm | mitochondrion | nucleus","4 iron, 4 sulfur cluster binding | lyase activity | DNA-(apurinic or apyrimidinic site) lyase activity | DNA binding | double-stranded DNA binding | protein binding | hydrolase activity, acting on glycosyl bonds | DNA N-glycosylase activity | metal ion binding | endonuclease activity","DNA repair | depyrimidination | base-excision repair, AP site formation | base-excision repair | nucleotide-excision repair, DNA incision, 5'-to lesion","NTHL1","nth endonuclease III-like 1 (E. coli)","MitoCarta3.0","Metabolism > Metals and cofactors > Fe-S-containing proteins","unknown",0,"HHH | HhH-GPD","Base excision repair",null,null,null,"FirstInEnsemblFamily",null,"NTHL1",1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,139,1,1,"Metabolism > Metals and cofactors > Fe-S-containing proteins","Metabolism "," Metals and cofactors "," Fe-S-containing proteins"]
["hsa:80224",80224,"NUBPL","C14orf127|FLJ12660|IND1|huInd1",14,"nucleotide binding protein-like","mitochondrion","4 iron, 4 sulfur cluster binding | nucleotide binding | metal ion binding | ATP binding","mitochondrion morphogenesis | mitochondrial respiratory chain complex I assembly","NUBPL","nucleotide binding protein-like","MitoCarta3.0","OXPHOS > Complex I > CI assembly factors | Metabolism > Metals and cofactors > Fe-S cluster biosynthesis | Metabolism > Metals and cofactors > Fe-S-containing proteins | OXPHOS > OXPHOS assembly factors","Matrix",0,null,null,null,null,null,"FirstInEnsemblFamily",null,"NUBPL",1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,139,1,1,"OXPHOS > Complex I > CI assembly factors | Metabolism > Metals and cofactors > Fe-S cluster biosynthesis | Metabolism > Metals and cofactors > Fe-S-containing proteins | OXPHOS > OXPHOS assembly factors","OXPHOS "," Complex I "," CI assembly factors "," Metabolism "," Metals and cofactors "," Fe-S cluster biosynthesis "," Metabolism "," Metals and cofactors "," Fe-S-containing proteins "," OXPHOS "," OXPHOS assembly factors"]
["hsa:64172",64172,"OSGEPL1","Qri7",2,"O-sialoglycoprotein endopeptidase-like 1","mitochondrion","metalloendopeptidase activity","tRNA processing | proteolysis","OSGEPL1","O-sialoglycoprotein endopeptidase-like 1","MitoCarta3.0","Mitochondrial central dogma > mtRNA metabolism > mt-tRNA modifications","Matrix",0,"Peptidase_M22",null,null,null,null,"FirstInEnsemblFamily",null,"OSGEPL1",1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,139,1,1,"Mitochondrial central dogma > mtRNA metabolism > mt-tRNA modifications","Mitochondrial central dogma "," mtRNA metabolism "," mt-tRNA modifications"]
["hsa:140886",140886,"PABPC5","FLJ51677|PABP5","X","poly(A) binding protein, cytoplasmic 5","cytoplasm","RNA binding | nucleotide binding | nucleic acid binding",null,"PABPC5","poly(A) binding protein, cytoplasmic 5","MitoCarta3.0",0,"Matrix",0,"RRM_1","mRNA surveillance pathway | RNA degradation | RNA transport",null,null,null,"FirstInEnsemblFamily",null,"PABPC5",1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,139,1,0,0,0]
["hsa:65018",65018,"PINK1","BRPK|FLJ27236|PARK6",1,"PTEN induced putative kinase 1","cytosol | mitochondrial outer membrane | mitochondrion | integral to membrane | membrane | cytoplasm","C3HC4-type RING finger domain binding | protein serine/threonine kinase activity | calcium-dependent protein kinase activity | magnesium ion binding | kinase activity | nucleotide binding | ubiquitin protein ligase binding | ATP binding","intracellular protein kinase cascade | positive regulation of release of cytochrome c from mitochondria | peptidyl-serine phosphorylation | protein phosphorylation | positive regulation of dopamine secretion | regulation of protein complex assembly | positive regulation of synaptic transmission, dopaminergic | response to stress | positive regulation of I-kappaB kinase/NF-kappaB cascade | mitochondrion degradation | regulation of protein ubiquitination | cell death","PINK1","PTEN induced putative kinase 1","MitoCarta3.0","Mitochondrial dynamics and surveillance > Mitophagy | Mitochondrial dynamics and surveillance > Autophagy | Signaling","MOM",0,"Pkinase",null,null,null,null,"FirstInEnsemblFamily","Parkinson disease 6, early onset","PINK1",1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,139,1,0,"Mitochondrial dynamics and surveillance > Mitophagy | Mitochondrial dynamics and surveillance > Autophagy | Signaling","Mitochondrial dynamics and surveillance "," Mitophagy ",null," Mitochondrial dynamics and surveillance "," Autophagy ",null," Signaling"]
["hsa:10105",10105,"PPIF","CYP3|Cyp-D|FLJ90798|MGC117207",10,"peptidylprolyl isomerase F","mitochondrion | mitochondrial inner membrane | mitochondrial matrix | membrane fraction","isomerase activity | peptidyl-prolyl cis-trans isomerase activity | cyclosporin A binding","protein folding | regulation of mitochondrial membrane permeability | regulation of apoptosis","PPIF","peptidylprolyl isomerase F (cyclophilin F)","MitoCarta3.0","Signaling > Calcium homeostasis > Mitochondrial permeability transition pore","Matrix",0,"Pro_isomerase",null,"PEPTIDYL PROLYL CIS TRANS ISOMERASE A PPIASE A EC_5.2.1.8 CYCLOPHILIN A CYCLOSPORIN A BINDING ROTAMASE A","ENSFM00600000921134",10,"FirstInEnsemblFamily",null,"PPIF",1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,139,1,1,"Signaling > Calcium homeostasis > Mitochondrial permeability transition pore","Signaling "," Calcium homeostasis "," Mitochondrial permeability transition pore"]
["hsa:5566",5566,"PRKACA","MGC102831|MGC48865|PKACA",19,"protein kinase, cAMP-dependent, catalytic, alpha","nucleoplasm | mitochondrion | Golgi apparatus | centrosome | perinuclear region of cytoplasm | cytoplasm | neuromuscular junction | cytosol | cAMP-dependent protein kinase complex | plasma membrane | soluble fraction | nucleus | membrane fraction","protein serine/threonine kinase activity | protein kinase binding | cAMP-dependent protein kinase inhibitor activity | nucleotide binding | ATP binding | cAMP-dependent protein kinase activity | protein binding | ubiquitin protein ligase binding","intracellular protein kinase cascade | protein autophosphorylation | mitotic cell cycle | negative regulation of meiotic cell cycle | peptidyl-serine phosphorylation | energy reserve metabolic process | regulation of synaptic transmission, glutamatergic | transmembrane transport | positive regulation of protein export from nucleus | triglyceride catabolic process | blood coagulation | activation of protein kinase A activity | glucose metabolic process | water transport | cellular response to parathyroid hormone stimulus | activation of phospholipase C activity | protein phosphorylation | carbohydrate metabolic process | lipid metabolic process | regulation of cellular respiration | G2/M transition of mitotic cell cycle | regulation of insulin secretion | gluconeogenesis | nerve growth factor receptor signaling pathway | mesoderm formation | cellular response to glucagon stimulus","PRKACA","protein kinase, cAMP-dependent, catalytic, alpha","MitoCarta3.0","Signaling > cAMP-PKA signaling","MIM",0,"Pkinase","Apoptosis | Oocyte meiosis | Gap junction","CAMP DEPENDENT KINASE CATALYTIC SUBUNIT PKA C EC_2.7.11.11","ENSFM00400000131743",4,"FirstInEnsemblFamily",null,"PRKACA",1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,139,1,0,"Signaling > cAMP-PKA signaling","Signaling "," cAMP-PKA signaling"]
["hsa:80324",80324,"PUS1","MGC11268|MLASA1",12,"pseudouridylate synthase 1","mitochondrion | transcription factor complex | nucleolus | nucleus","isomerase activity | pseudouridine synthase activity | RNA binding | pseudouridylate synthase activity | DNA binding | ligand-dependent nuclear receptor transcription coactivator activity","tRNA processing | positive regulation of transcription from RNA polymerase II promoter | tRNA pseudouridine synthesis","PUS1","pseudouridylate synthase 1","MitoCarta3.0","Mitochondrial central dogma > mtRNA metabolism > mtRNA granules | Mitochondrial central dogma > mtRNA metabolism > mt-tRNA modifications","Matrix",0,"PseudoU_synth_1",null,null,null,null,"FirstInEnsemblFamily","Mitochondrial myopathy and sideroblastic anemia 1","PUS1",1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,139,1,1,"Mitochondrial central dogma > mtRNA metabolism > mtRNA granules | Mitochondrial central dogma > mtRNA metabolism > mt-tRNA modifications","Mitochondrial central dogma "," mtRNA metabolism "," mtRNA granules "," Mitochondrial central dogma "," mtRNA metabolism "," mt-tRNA modifications"]
["hsa:53917",53917,"RAB24","-",5,"RAB24, member RAS oncogene family","cytosol | mitochondrion | membrane | cytoplasm","protein binding | GTP binding | nucleotide binding","autophagy | protein transport | small GTPase mediated signal transduction","RAB24","RAB24, member RAS oncogene family","MitoCarta3.0","Mitochondrial dynamics and surveillance > Fission | Mitochondrial dynamics and surveillance > Autophagy","MOM",0,"Miro | Ras",null,null,null,null,"FirstInEnsemblFamily",null,"RAB24",1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,139,1,0,"Mitochondrial dynamics and surveillance > Fission | Mitochondrial dynamics and surveillance > Autophagy","Mitochondrial dynamics and surveillance "," Fission ",null," Mitochondrial dynamics and surveillance "," Autophagy"]
["hsa:9401",9401,"RECQL4","RECQ4",8,"RecQ protein-like 4","nucleus | cytoplasm","hydrolase activity | DNA strand annealing activity | helicase activity | ATP-dependent 3'-5' DNA helicase activity | nucleotide binding | ATP binding | bubble DNA binding | zinc ion binding | nucleic acid binding | ATP-dependent helicase activity","multicellular organismal development | DNA repair | DNA strand renaturation | DNA duplex unwinding | DNA recombination | DNA replication","RECQL4","RecQ protein-like 4","MitoCarta3.0","Mitochondrial central dogma > mtDNA maintenance > mtDNA repair","Matrix",0,"DEAD | Helicase_C",null,null,null,null,"FirstInEnsemblFamily","Baller-Gerold syndrome|RAPADILINO syndrome|Rothmund-Thomson syndrome","RECQL4",1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,139,1,1,"Mitochondrial central dogma > mtDNA maintenance > mtDNA repair","Mitochondrial central dogma "," mtDNA maintenance "," mtDNA repair"]
["hsa:55288",55288,"RHOT1","ARHT1|FLJ11040|FLJ12633|MIRO-1|MIRO1",17,"ras homolog gene family, member T1","cytosol | mitochondrial outer membrane | mitochondrion | integral to membrane | integral to mitochondrial outer membrane | plasma membrane | intracellular","hydrolase activity | protein binding | GTP binding | GTPase activity | molecular_function | nucleotide binding | calcium ion binding","mitochondrion transport along microtubule | cellular homeostasis | small GTPase mediated signal transduction | regulation of small GTPase mediated signal transduction | apoptosis","RHOT1","ras homolog gene family, member T1","MitoCarta3.0","Signaling > Calcium homeostasis > EF hand proteins | Mitochondrial dynamics and surveillance > Trafficking","MOM",1,"Miro | EF_assoc_1 | EF_assoc_2 | Ras | efhand",null,"MITOCHONDRIAL RHO GTPASE 1 EC_3.6.5.- GTPASE EF HAND OF MITOCHONDRIA 1","ENSFM00250000000940",2,"FirstInEnsemblFamily",null,"RHOT1",1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,139,1,1,"Signaling > Calcium homeostasis > EF hand proteins | Mitochondrial dynamics and surveillance > Trafficking","Signaling "," Calcium homeostasis "," EF hand proteins "," Mitochondrial dynamics and surveillance "," Trafficking"]
["hsa:89941",89941,"RHOT2","ARHT2|C16orf39|MIRO-2|MIRO2|RASL",16,"ras homolog gene family, member T2","mitochondrion | mitochondrial inner membrane | intracellular | mitochondrial outer membrane | cytosol | integral to membrane | plasma membrane | integral to mitochondrial outer membrane","hydrolase activity | GTP binding | GTPase activity | nucleotide binding | calcium ion binding","mitochondrion transport along microtubule | cellular homeostasis | small GTPase mediated signal transduction | regulation of small GTPase mediated signal transduction | apoptosis","RHOT2","ras homolog gene family, member T2","MitoCarta3.0","Signaling > Calcium homeostasis > EF hand proteins | Mitochondrial dynamics and surveillance > Trafficking","MOM",1,"Miro | EF_assoc_1 | EF_assoc_2 | Ras | efhand",null,"MITOCHONDRIAL RHO GTPASE 1 EC_3.6.5.- GTPASE EF HAND OF MITOCHONDRIA 1","ENSFM00250000000940",2,null,null,"RHOT2",1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,139,1,0,"Signaling > Calcium homeostasis > EF hand proteins | Mitochondrial dynamics and surveillance > Trafficking","Signaling "," Calcium homeostasis "," EF hand proteins "," Mitochondrial dynamics and surveillance "," Trafficking"]
["hsa:23409",23409,"SIRT4","MGC130046|MGC130047|MGC57437|SIR2L4",12,"sirtuin 4","mitochondrion | mitochondrial matrix","protein binding | NAD+ ADP-ribosyltransferase activity | NAD+ binding | NAD-dependent protein deacetylase activity | transferase activity | zinc ion binding | metal ion binding","protein ADP-ribosylation | negative regulation of insulin secretion | protein deacetylation | chromatin silencing","SIRT4","sirtuin (silent mating type information regulation 2 homolog) 4 (S. cerevisiae)","MitoCarta3.0","Metabolism > Metals and cofactors > NAD biosynthesis and metabolism","Matrix",0,"SIR2",null,null,null,null,"FirstInEnsemblFamily",null,"SIRT4",1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,139,1,0,"Metabolism > Metals and cofactors > NAD biosynthesis and metabolism","Metabolism "," Metals and cofactors "," NAD biosynthesis and metabolism"]
["hsa:23408",23408,"SIRT5","FLJ36950|SIR2L5",6,"sirtuin 5","mitochondrion | mitochondrial matrix | mitochondrial intermembrane space","hydrolase activity | NAD+ ADP-ribosyltransferase activity | NAD+ binding | zinc ion binding | metal ion binding","protein ADP-ribosylation | protein deacetylation | chromatin silencing","SIRT5","sirtuin (silent mating type information regulation 2 homolog) 5 (S. cerevisiae)","MitoCarta3.0","Metabolism > Metals and cofactors > NAD biosynthesis and metabolism","Matrix",0,"SIR2",null,null,null,null,"FirstInEnsemblFamily",null,"SIRT5",1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,139,1,0,"Metabolism > Metals and cofactors > NAD biosynthesis and metabolism","Metabolism "," Metals and cofactors "," NAD biosynthesis and metabolism"]
["hsa:6687",6687,"SPG7","CAR|CMAR|FLJ37308|MGC126331|MGC126332|PGN|SPG5C",16,"spastic paraplegia 7 (pure and complicated autosomal recessive)","mitochondrion | integral to membrane | mitochondrial membrane | membrane","unfolded protein binding | peptidase activity | nucleoside-triphosphatase activity | metalloendopeptidase activity | zinc ion binding | nucleotide binding | metal ion binding | ATP binding","proteolysis | cell death | mitochondrion organization | protein catabolic process | anterograde axon cargo transport | nervous system development","SPG7","spastic paraplegia 7, paraplegin (pure and complicated autosomal recessive)","MitoCarta3.0","Protein import, sorting and homeostasis > Protein homeostasis > Proteases","MIM",2,"FtsH_ext | Peptidase_M41 | AAA | AAA_5",null,"EC_3.4.24.-","ENSFM00620000999395",2,"FirstInEnsemblFamily","Spastic paraplegia 7, autosomal recessive","SPG7",1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,139,1,0,"Protein import, sorting and homeostasis > Protein homeostasis > Proteases","Protein import, sorting and homeostasis "," Protein homeostasis "," Proteases"]
["hsa:10953",10953,"TOMM34","HTOM34P|TOM34|URCC3",20,"translocase of outer mitochondrial membrane 34","mitochondrial outer membrane | mitochondrion | integral to membrane | membrane | cytoplasm","heat shock protein binding | binding | signal sequence binding","protein targeting to mitochondrion","TOMM34","translocase of outer mitochondrial membrane 34","MitoCarta3.0","Protein import, sorting and homeostasis > Protein import and sorting > TOM","MOM",0,"TPR_1 | TPR_2",null,null,null,null,"FirstInEnsemblFamily",null,"TOMM34",1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,139,1,0,"Protein import, sorting and homeostasis > Protein import and sorting > TOM","Protein import, sorting and homeostasis "," Protein import and sorting "," TOM"]
["hsa:9868",9868,"TOMM70A","FLJ90470",3,"translocase of outer mitochondrial membrane 70 homolog A (S. cerevisiae)","mitochondrion | integral to membrane | mitochondrial outer membrane translocase complex | membrane","protein binding | protein transmembrane transporter activity","protein targeting to mitochondrion","TOMM70A","translocase of outer mitochondrial membrane 70 homolog A (S. cerevisiae)","MitoCarta3.0","Protein import, sorting and homeostasis > Protein import and sorting > TOM","MOM",2,"TPR_1 | TPR_2",null,null,null,null,"FirstInEnsemblFamily",null,"TOMM70",1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,139,1,0,"Protein import, sorting and homeostasis > Protein import and sorting > TOM","Protein import, sorting and homeostasis "," Protein import and sorting "," TOM"]
["hsa:7156",7156,"TOP3A","TOP3",17,"topoisomerase (DNA) III alpha","PML body | chromosome | nucleus","DNA topoisomerase type I activity | DNA binding | nucleotide binding | ATP binding | DNA topoisomerase activity | protein binding | zinc ion binding | metal ion binding","meiosis | DNA topological change","TOP3A","topoisomerase (DNA) III alpha","MitoCarta3.0","Mitochondrial central dogma > mtDNA maintenance > mtDNA replication","Matrix",0,"zf-GRF | Topoisom_bac | Toprim | zf-C4_Topoisom","Homologous recombination","DNA TOPOISOMERASE 3 EC_5.99.1.2 DNA TOPOISOMERASE III","ENSFM00250000001744",2,"FirstInEnsemblFamily",null,"TOP3A",1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,139,1,1,"Mitochondrial central dogma > mtDNA maintenance > mtDNA replication","Mitochondrial central dogma "," mtDNA maintenance "," mtDNA replication"]
["hsa:7284",7284,"TUFM","COXPD4|EF-TuMT|EFTU|P43",16,"Tu translation elongation factor, mitochondrial","mitochondrial nucleoid | mitochondrion | intracellular","GTP binding | GTPase activity | translation elongation factor activity | nucleotide binding","translational elongation","TUFM","Tu translation elongation factor, mitochondrial","MitoCarta3.0","Mitochondrial central dogma > Translation > Translation factors","Matrix",0,"GTP_EFTU_D3 | GTP_EFTU_D2 | GTP_EFTU",null,null,null,null,"FirstInEnsemblFamily","Combined oxidative phosphorylation deficiency 4","TUFM",1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,139,1,1,"Mitochondrial central dogma > Translation > Translation factors","Mitochondrial central dogma "," Translation "," Translation factors"]
["hsa:25828",25828,"TXN2","MT-TRX|MTRX|TRX2",22,"thioredoxin 2","mitochondrion | nucleolus","electron carrier activity | peptide-methionine-(S)-S-oxide reductase activity | protein disulfide oxidoreductase activity | peptide-methionine (R)-S-oxide reductase activity","response to oxidative stress | transport | response to drug | electron transport chain | response to hypoxia | response to axon injury | cell redox homeostasis | response to glucose stimulus | glycerol ether metabolic process | response to hormone stimulus | response to organic cyclic compound | response to nutrient | cellular response to nutrient levels","TXN2","thioredoxin 2","MitoCarta3.0","Metabolism > Vitamin metabolism > Vitamin C metabolism | Metabolism > Detoxification > ROS and glutathione metabolism","Matrix",0,"Thioredoxin",null,null,null,null,"FirstInEnsemblFamily",null,"TXN2",1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,139,1,1,"Metabolism > Vitamin metabolism > Vitamin C metabolism | Metabolism > Detoxification > ROS and glutathione metabolism","Metabolism "," Vitamin metabolism "," Vitamin C metabolism "," Metabolism "," Detoxification "," ROS and glutathione metabolism"]
["hsa:57176",57176,"VARS2","MGC138259|MGC142165|VARS2L|VARSL",6,"valyl-tRNA synthetase 2, mitochondrial (putative)","mitochondrion | cytoplasm","valine-tRNA ligase activity | nucleotide binding | ligase activity | ATP binding","gene expression | valyl-tRNA aminoacylation | tRNA aminoacylation for protein translation","VARSL","valyl-tRNA synthetase like","MitoCarta3.0","Mitochondrial central dogma > Translation > mt-tRNA synthetases","Matrix",0,"Anticodon_1 | tRNA-synt_1","Aminoacyl-tRNA biosynthesis | \"Valine, leucine and isoleucine biosynthesis\"","VALYL TRNA SYNTHETASE EC_6.1.1.9 VALINE TRNA LIGASE VALRS","ENSFM00250000001468",2,"FirstInEnsemblFamily",null,"VARS2",1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,139,1,0,"Mitochondrial central dogma > Translation > mt-tRNA synthetases","Mitochondrial central dogma "," Translation "," mt-tRNA synthetases"]
["hsa:10730",10730,"YME1L1","FTSH|MEG4|PAMP|YME1L",10,"YME1-like 1 (S. cerevisiae)","mitochondrion | membrane","peptidase activity | nucleoside-triphosphatase activity | metalloendopeptidase activity | nucleotide binding | metal ion binding | ATP binding","proteolysis | protein catabolic process","YME1L1","YME1-like 1 (S. cerevisiae)","MitoCarta3.0","Protein import, sorting and homeostasis > Protein homeostasis > Proteases","MIM",0,"Peptidase_M41 | AAA",null,null,null,null,"FirstInEnsemblFamily",null,"YME1L1",1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,139,1,1,"Protein import, sorting and homeostasis > Protein homeostasis > Proteases","Protein import, sorting and homeostasis "," Protein homeostasis "," Proteases"]
["hsa:57505",57505,"AARS2","AARSL|COXPD8|KIAA1270|MT-ALARS|MTALARS|bA444E17.1",6,"alanyl-tRNA synthetase 2, mitochondrial (putative)","mitochondrion | cytoplasm","alanine-tRNA ligase activity | tRNA binding | nucleotide binding | metal ion binding | ligase activity | ATP binding","alanyl-tRNA aminoacylation | gene expression | tRNA aminoacylation for protein translation","AARSL","alanyl-tRNA synthetase like","MitoCarta3.0","Mitochondrial central dogma > Translation > mt-tRNA synthetases","Matrix",0,"tRNA-synt_2c | tRNA_SAD","Aminoacyl-tRNA biosynthesis","ALANYL TRNA SYNTHETASE EC_6.1.1.7 ALANINE TRNA LIGASE ALARS","ENSFM00250000001208",2,"FirstInEnsemblFamily","Combined oxidative phosphorylation deficiency 8","AARS2",1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,138,1,0,"Mitochondrial central dogma > Translation > mt-tRNA synthetases","Mitochondrial central dogma "," Translation "," mt-tRNA synthetases"]
["hsa:79587",79587,"CARS2","DKFZp667A2315|DKFZp686G08243|FLJ12118|FLJ42514",13,"cysteinyl-tRNA synthetase 2, mitochondrial (putative)","cytosol | mitochondrion | mitochondrial matrix | cytoplasm","cysteine-tRNA ligase activity | nucleotide binding | metal ion binding | ligase activity | ATP binding","cysteinyl-tRNA aminoacylation | gene expression | tRNA aminoacylation for protein translation","FLJ12118","hypothetical protein FLJ12118","MitoCarta3.0","Mitochondrial central dogma > Translation > mt-tRNA synthetases","Matrix",0,"tRNA-synt_1g | tRNA-synt_1e","Aminoacyl-tRNA biosynthesis",null,null,null,"FirstInEnsemblFamily",null,"CARS2",1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,138,1,0,"Mitochondrial central dogma > Translation > mt-tRNA synthetases","Mitochondrial central dogma "," Translation "," mt-tRNA synthetases"]
["hsa:10202",10202,"DHRS2","HEP27|SDR25C1",14,"dehydrogenase/reductase (SDR family) member 2","mitochondrion | nuclear envelope | nucleus | cytoplasm","oxidoreductase activity | nucleotide binding | carbonyl reductase (NADPH) activity","oxidation-reduction process | negative regulation of apoptosis | cellular response to oxidative stress | negative regulation of cell proliferation | response to toxin | myeloid dendritic cell differentiation | C21-steroid hormone metabolic process",null,null,"MitoCarta3.0","Metabolism > Detoxification > Xenobiotic metabolism","Matrix",null,"KR | adh_short",null,"DEHYDROGENASE/REDUCTASE SDR FAMILY MEMBER 4 EC_1.1.1.184 NADPH DEPENDENT CARBONYL REDUCTASE/NADP RETINOL DEHYDROGENASE CR PHCR NADPH DEPENDENT RETINOL DEHYDROGENASE/REDUCTASE NDRD PEROXISOMAL SHORT CHAIN ALCOHOL DEHYDROGENASE PSCD","ENSFM00500000270135",3,null,null,"DHRS2",1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,138,1,0,"Metabolism > Detoxification > Xenobiotic metabolism","Metabolism "," Detoxification "," Xenobiotic metabolism"]
["hsa:1738",1738,"DLD","DLDH|E3|GCSL|LAD|PHE3",7,"dihydrolipoamide dehydrogenase","oxoglutarate dehydrogenase complex | mitochondrion | flagellum | pyruvate dehydrogenase complex | acrosomal matrix | mitochondrial matrix | cytoplasm","dihydrolipoyl dehydrogenase activity | oxidoreductase activity | NAD binding | flavin adenine dinucleotide binding | lipoamide binding","sperm capacitation | aging | dihydrolipoamide metabolic process | gastrulation | cell redox homeostasis | 2-oxoglutarate metabolic process | branched chain family amino acid catabolic process | regulation of membrane potential | mitochondrial electron transport, NADH to ubiquinone | proteolysis | regulation of acetyl-CoA biosynthetic process from pyruvate | pyruvate metabolic process | lipoate metabolic process | tricarboxylic acid cycle | cellular nitrogen compound metabolic process | lysine catabolic process","DLD","dihydrolipoamide dehydrogenase","MitoCarta3.0","Metabolism > Carbohydrate metabolism > Pyruvate metabolism | Metabolism > Carbohydrate metabolism > TCA cycle | Metabolism > Amino acid metabolism > Branched-chain amino acid metabolism | Metabolism > Amino acid metabolism > Branched-chain amino acid dehydrogenase complex | Metabolism > Amino acid metabolism > Lysine metabolism | Metabolism > Amino acid metabolism > Glycine metabolism | Metabolism > Amino acid metabolism > Glycine cleavage system","Matrix",0,"Pyr_redox | Pyr_redox_dim | Pyr_redox_2 | GIDA",null,null,null,null,"FirstInEnsemblFamily","Maple syrup urine disease, type II","DLD",1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,138,1,1,"Metabolism > Carbohydrate metabolism > Pyruvate metabolism | Metabolism > Carbohydrate metabolism > TCA cycle | Metabolism > Amino acid metabolism > Branched-chain amino acid metabolism | Metabolism > Amino acid metabolism > Branched-chain amino acid dehydrogenase complex | Metabolism > Amino acid metabolism > Lysine metabolism | Metabolism > Amino acid metabolism > Glycine metabolism | Metabolism > Amino acid metabolism > Glycine cleavage system","Metabolism "," Carbohydrate metabolism "," Pyruvate metabolism "," Metabolism "," Carbohydrate metabolism "," TCA cycle "," Metabolism "," Amino acid metabolism "," Branched-chain amino acid metabolism "," Metabolism "," Amino acid metabolism "," Branched-chain amino acid dehydrogenase complex "," Metabolism "," Amino acid metabolism "," Lysine metabolism "]
["hsa:10059",10059,"DNM1L","DLP1|DRP1|DVLP|DYMPLE|DYNIV-11|FLJ41912|HDYNIV|VPS1",12,"dynamin 1-like","endomembrane system | Golgi apparatus | endoplasmic reticulum | cis-Golgi network | cytoplasm | mitochondrial outer membrane | cytosol | membrane","hydrolase activity | protein binding | GTP binding | GTPase activity | nucleotide binding | ubiquitin protein ligase binding","peroxisome fission | cellular component disassembly involved in apoptosis | positive regulation of mitochondrial fission | mitochondrial fragmentation involved in apoptosis | apoptosis | GTP catabolic process | membrane fission involved in mitochondrial fission | dynamin polymerization involved in mitochondrial fission | mitochondrial membrane organization","DNM1L","dynamin 1-like","MitoCarta3.0","Mitochondrial dynamics and surveillance > Fission","MOM",0,"Dynamin_N | GED | Dynamin_M","Endocytosis",null,null,null,"FirstInEnsemblFamily","Encephalopahty, lethal, due to defective mitochondrial peroxisomal fission","DNM1L",0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,138,1,1,"Mitochondrial dynamics and surveillance > Fission","Mitochondrial dynamics and surveillance "," Fission"]
["hsa:2232",2232,"FDXR","ADXR",17,"ferredoxin reductase","mitochondrion | mitochondrial inner membrane | mitochondrial matrix","NADPH-adrenodoxin reductase activity | protein binding | ferredoxin-NADP+ reductase activity | NADPH binding | nucleotide binding","oxidation-reduction process | transport | generation of precursor metabolites and energy | NADPH oxidation | electron transport chain | cholesterol metabolic process | steroid biosynthetic process","FDXR","ferredoxin reductase","MitoCarta3.0","Metabolism > Lipid metabolism > Cholesterol, bile acid, steroid synthesis | Metabolism > Metals and cofactors > Heme synthesis and processing | Metabolism > Metals and cofactors > Fe-S cluster biosynthesis | Metabolism > Vitamin metabolism > Vitamin D metabolism","Matrix",0,"Pyr_redox_2",null,null,null,null,"FirstInEnsemblFamily",null,"FDXR",1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,138,1,1,"Metabolism > Lipid metabolism > Cholesterol, bile acid, steroid synthesis | Metabolism > Metals and cofactors > Heme synthesis and processing | Metabolism > Metals and cofactors > Fe-S cluster biosynthesis | Metabolism > Vitamin metabolism > Vitamin D metabolism","Metabolism "," Lipid metabolism "," Cholesterol, bile acid, steroid synthesis "," Metabolism "," Metals and cofactors "," Heme synthesis and processing "," Metabolism "," Metals and cofactors "," Fe-S cluster biosynthesis "," Metabolism "," Vitamin metabolism "," Vitamin D metabolism"]
["hsa:60681",60681,"FKBP10","FKBP65|FLJ20683|FLJ22041|FLJ23833|OI6|PPIASE|hFKBP65",17,"FK506 binding protein 10, 65 kDa","endoplasmic reticulum | membrane | endoplasmic reticulum lumen","isomerase activity | FK506 binding | peptidyl-prolyl cis-trans isomerase activity | calcium ion binding","protein folding | protein peptidyl-prolyl isomerization | peptidyl-proline modification","FKBP10","FK506 binding protein 10, 65 kDa","MitoCarta3.0","Protein import, sorting and homeostasis > Protein homeostasis > Chaperones","IMS",0,"FKBP_C | efhand",null,"PEPTIDYL PROLYL CIS TRANS ISOMERASE PRECURSOR PPIASE EC_5.2.1.8 KDA FK506 BINDING KDA FKBP FKBP FK506 BINDING FKBP ROTAMASE","ENSFM00500000270138",2,"FirstInEnsemblFamily","Osteogenesis imperfecta, type XI","FKBP10",1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,138,1,0,"Protein import, sorting and homeostasis > Protein homeostasis > Chaperones","Protein import, sorting and homeostasis "," Protein homeostasis "," Chaperones"]
["hsa:3295",3295,"HSD17B4","DBP|MFE-2|MPF-2|SDR8C1",5,"hydroxysteroid (17-beta) dehydrogenase 4","mitochondrion | peroxisomal matrix | peroxisome","isomerase activity | lyase activity | oxidoreductase activity | 3alpha,7alpha,12alpha-trihydroxy-5beta-cholest-24-enoyl-CoA hydratase activity | long-chain-enoyl-CoA hydratase activity | sterol transporter activity | estradiol 17-beta-dehydrogenase activity | 3-hydroxyacyl-CoA dehydrogenase activity | nucleotide binding | sterol binding","Sertoli cell development | fatty acid beta-oxidation using acyl-CoA oxidase | very long-chain fatty acid metabolic process | sterol transport | cellular lipid metabolic process | bile acid biosynthetic process | bile acid metabolic process","HSD17B4","hydroxysteroid (17-beta) dehydrogenase 4","MitoCarta3.0","Metabolism > Lipid metabolism","unknown",0,"SCP2 | MaoC_dehydratas | KR | adh_short","Primary bile acid biosynthesis | Peroxisome",null,null,null,"FirstInEnsemblFamily","D-bifunctional protein deficiency|Perrault syndrome","HSD17B4",1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,138,1,0,"Metabolism > Lipid metabolism","Metabolism "," Lipid metabolism"]
["hsa:3313",3313,"HSPA9","CSA|GRP-75|GRP75|HSPA9B|MGC4500|MOT|MOT2|MTHSP75|PBP74",5,"heat shock 70kDa protein 9 (mortalin)","mitochondrial nucleoid | mitochondrion | cell surface | cytoplasm","unfolded protein binding | protein binding | nucleotide binding | ATP binding","protein folding | protein export from nucleus | anti-apoptosis","HSPA9","heat shock 70kDa protein 9 (mortalin)","MitoCarta3.0","Metabolism > Metals and cofactors > Fe-S cluster biosynthesis","Matrix",0,"HSP70","RNA degradation",null,null,null,"FirstInEnsemblFamily",null,"HSPA9",1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,138,1,1,"Metabolism > Metals and cofactors > Fe-S cluster biosynthesis","Metabolism "," Metals and cofactors "," Fe-S cluster biosynthesis"]
["hsa:55699",55699,"IARS2","FLJ10326|ILERS",1,"isoleucyl-tRNA synthetase 2, mitochondrial","mitochondrion | mitochondrial matrix | cytoplasm","isoleucine-tRNA ligase activity | nucleotide binding | ligase activity | ATP binding","isoleucyl-tRNA aminoacylation | gene expression | tRNA aminoacylation for protein translation","IARS2","isoleucine-tRNA synthetase 2, mitochondrial","MitoCarta3.0","Mitochondrial central dogma > Translation > mt-tRNA synthetases","Matrix",0,"tRNA-synt_1g | Anticodon_1 | tRNA-synt_1 | zf-FPG_IleRS","Aminoacyl-tRNA biosynthesis | \"Valine, leucine and isoleucine biosynthesis\"",null,null,null,"FirstInEnsemblFamily",null,"IARS2",1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,138,1,1,"Mitochondrial central dogma > Translation > mt-tRNA synthetases","Mitochondrial central dogma "," Translation "," mt-tRNA synthetases"]
["hsa:23395",23395,"LARS2","KIAA0028|LEURS|MGC26121",3,"leucyl-tRNA synthetase 2, mitochondrial","mitochondrion | mitochondrial matrix | cytoplasm","leucine-tRNA ligase activity | nucleotide binding | ligase activity | ATP binding","gene expression | leucyl-tRNA aminoacylation | tRNA aminoacylation for protein translation","LARS2","leucyl-tRNA synthetase 2, mitochondrial","MitoCarta3.0","Mitochondrial central dogma > Translation > mt-tRNA synthetases","Matrix",0,"tRNA-synt_1g | Anticodon_1 | tRNA-synt_1","Aminoacyl-tRNA biosynthesis | \"Valine, leucine and isoleucine biosynthesis\"",null,null,null,"FirstInEnsemblFamily",null,"LARS2",1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,138,1,1,"Mitochondrial central dogma > Translation > mt-tRNA synthetases","Mitochondrial central dogma "," Translation "," mt-tRNA synthetases"]
["hsa:3980",3980,"LIG3","LIG2",17,"ligase III, DNA, ATP-dependent","nucleoplasm | nucleus | intracellular","DNA ligase activity | DNA binding | nucleotide binding | ligase activity | ATP binding | protein binding | DNA ligase (ATP) activity | zinc ion binding | metal ion binding","base-excision repair | DNA repair | spermatogenesis | DNA ligation | V(D)J recombination | cell cycle | reciprocal meiotic recombination | cell division | DNA ligation involved in DNA repair | DNA replication | DNA recombination","LIG3","ligase III, DNA, ATP-dependent","MitoCarta3.0","Mitochondrial central dogma > mtDNA maintenance > mtDNA replication | Mitochondrial central dogma > mtDNA maintenance > mtDNA repair","Matrix",0,"DNA_ligase_A_C | zf-PARP | DNA_ligase_A_N | DNA_ligase_A_M","Base excision repair",null,null,null,"FirstInEnsemblFamily",null,"LIG3",1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,138,1,0,"Mitochondrial central dogma > mtDNA maintenance > mtDNA replication | Mitochondrial central dogma > mtDNA maintenance > mtDNA repair","Mitochondrial central dogma "," mtDNA maintenance "," mtDNA replication "," Mitochondrial central dogma "," mtDNA maintenance "," mtDNA repair"]
["hsa:92935",92935,"MARS2","MetRS|mtMetRS",2,"methionyl-tRNA synthetase 2, mitochondrial","mitochondrion | mitochondrial matrix | cytoplasm","methionine-tRNA ligase activity | nucleotide binding | ligase activity | ATP binding","gene expression | methionyl-tRNA aminoacylation | tRNA aminoacylation for protein translation","MARS2","methionine-tRNA synthetase 2 (mitochondrial)","MitoCarta3.0","Mitochondrial central dogma > Translation > mt-tRNA synthetases","Matrix",0,"tRNA-synt_1g","Selenocompound metabolism | Aminoacyl-tRNA biosynthesis",null,null,null,"FirstInEnsemblFamily",null,"MARS2",1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,138,1,1,"Mitochondrial central dogma > Translation > mt-tRNA synthetases","Mitochondrial central dogma "," Translation "," mt-tRNA synthetases"]
["hsa:9054",9054,"NFS1","IscS|NIFS",20,"NFS1 nitrogen fixation 1 homolog (S. cerevisiae)","cytosol | mitochondrion | nucleus | mitochondrial matrix | cytoplasm","protein binding | pyridoxal phosphate binding | protein homodimerization activity | transferase activity | catalytic activity | cysteine desulfurase activity","iron incorporation into metallo-sulfur cluster | sulfur amino acid metabolic process | Mo-molybdopterin cofactor biosynthetic process | water-soluble vitamin metabolic process | vitamin metabolic process | protein complex assembly | cysteine metabolic process | molybdopterin cofactor biosynthetic process","NFS1","NFS1 nitrogen fixation 1 homolog (S. cerevisiae)","MitoCarta3.0","Metabolism > Metals and cofactors > Fe-S cluster biosynthesis | Metabolism > Metals and cofactors > Fe-S-containing proteins","Matrix",0,"Beta_elim_lyase | Aminotran_5","Sulfur relay system | Thiamine metabolism",null,null,null,"FirstInEnsemblFamily",null,"NFS1",1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,138,1,1,"Metabolism > Metals and cofactors > Fe-S cluster biosynthesis | Metabolism > Metals and cofactors > Fe-S-containing proteins","Metabolism "," Metals and cofactors "," Fe-S cluster biosynthesis "," Metabolism "," Metals and cofactors "," Fe-S-containing proteins"]
["hsa:4976",4976,"OPA1","FLJ12460|KIAA0567|MGM1|NPG|NTG|largeG",3,"optic atrophy 1 (autosomal dominant)","mitochondrion | mitochondrial inner membrane | mitochondrial crista | mitochondrial intermembrane space | mitochondrial outer membrane | integral to membrane | membrane | dendrite","protein binding | GTP binding | magnesium ion binding | GTPase activity | nucleotide binding","mitochondrial fission | mitochondrion organization | positive regulation of anti-apoptosis | apoptosis | GTP catabolic process | response to stimulus | visual perception | axon transport of mitochondrion | inner mitochondrial membrane organization | mitochondrial fusion","OPA1","optic atrophy 1 (autosomal dominant)","MitoCarta3.0","Mitochondrial dynamics and surveillance > Fusion | Mitochondrial dynamics and surveillance > Cristae formation","MIM",0,"Dynamin_N",null,null,null,null,"FirstInEnsemblFamily","Optic atrophy with or without deafness, ophthalmoplegia, myopathy, ataxia, and neuropathy|Optic atrophy 1","OPA1",0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,138,1,0,"Mitochondrial dynamics and surveillance > Fusion | Mitochondrial dynamics and surveillance > Cristae formation","Mitochondrial dynamics and surveillance "," Fusion ",null," Mitochondrial dynamics and surveillance "," Cristae formation"]
["hsa:5071",5071,"PARK2","AR-JP|LPRS2|PDJ|PRKN",6,"parkinson protein 2, E3 ubiquitin protein ligase (parkin)","mitochondrion | aggresome | Golgi apparatus | endoplasmic reticulum | protein complex | perinuclear region of cytoplasm | cytoplasm | cytosol | neuron projection | nucleus","PDZ domain binding | ubiquitin-protein ligase activity | protein kinase binding | ligase activity | protein binding | kinase binding | chaperone binding | zinc ion binding | acid-amino acid ligase activity | metal ion binding | ubiquitin protein ligase binding","regulation of autophagy | protein K63-linked ubiquitination | startle response | regulation of neurotransmitter secretion | protein autoubiquitination | protein ubiquitination involved in ubiquitin-dependent protein catabolic process | protein monoubiquitination | regulation of reactive oxygen species metabolic process | neuron death | protein K48-linked ubiquitination | positive regulation of I-kappaB kinase/NF-kappaB cascade | norepinephrine metabolic process | cell death | negative regulation of release of cytochrome c from mitochondria | negative regulation of cell death | protein metabolic process | aggresome assembly | negative regulation of protein phosphorylation | dopamine uptake | negative regulation of actin filament bundle assembly | ubiquitin-dependent protein catabolic process | mitochondrion degradation | central nervous system development | dopamine metabolic process | learning | protein polyubiquitination | adult locomotory behavior | synaptic transmission, glutamatergic","PARK2","Parkinson disease (autosomal recessive, juvenile) 2, parkin","MitoCarta3.0","Mitochondrial dynamics and surveillance > Mitophagy | Mitochondrial dynamics and surveillance > Autophagy","MOM",0,"IBR | ubiquitin","Ubiquitin mediated proteolysis | Protein processing in endoplasmic reticulum",null,null,null,"FirstInEnsemblFamily","Parkinson disease, juvenile, type 2|Lung cancer|Ovarian cancer","PARK2",0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,138,1,0,"Mitochondrial dynamics and surveillance > Mitophagy | Mitochondrial dynamics and surveillance > Autophagy","Mitochondrial dynamics and surveillance "," Mitophagy ",null," Mitochondrial dynamics and surveillance "," Autophagy"]
["hsa:9512",9512,"PMPCB","Beta-MPP|MPP11|MPPB|MPPP52|P-52",7,"peptidase (mitochondrial processing) beta","mitochondrion | mitochondrial inner membrane | mitochondrial matrix","peptidase activity | metalloendopeptidase activity | zinc ion binding | metal ion binding","proteolysis","PMPCB","peptidase (mitochondrial processing) beta","MitoCarta3.0","Protein import, sorting and homeostasis > Protein import and sorting > Preprotein cleavage | Protein import, sorting and homeostasis > Protein homeostasis > Proteases","Matrix",0,"Peptidase_M16 | Peptidase_M16_C",null,"MITOCHONDRIAL PROCESSING PEPTIDASE SUBUNIT BETA PRECURSOR EC_3.4.24.64 BETA MPP","ENSFM00500000269799",2,null,null,"PMPCB",1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,138,1,1,"Protein import, sorting and homeostasis > Protein import and sorting > Preprotein cleavage | Protein import, sorting and homeostasis > Protein homeostasis > Proteases","Protein import, sorting and homeostasis "," Protein import and sorting "," Preprotein cleavage "," Protein import, sorting and homeostasis "," Protein homeostasis "," Proteases"]
["hsa:10721",10721,"POLQ","DKFZp781A0112|POLH|PRO0327",3,"polymerase (DNA directed), theta","nucleoplasm","damaged DNA binding | single-stranded DNA-dependent ATPase activity | helicase activity | transferase activity | nucleotide binding | ATP binding | DNA-directed DNA polymerase activity | ATP-dependent helicase activity | nucleotidyltransferase activity","DNA repair | DNA replication","POLQ","polymerase (DNA directed), theta","MitoCarta3.0","Mitochondrial central dogma > mtDNA maintenance","Matrix",0,"DNA_pol_A | Helicase_C | DEAD",null,"HELICASE POLQ  EC_3.6.4.12 MUS308  HELICASE POLQ  HELICASE","ENSFM00420000140575",2,null,null,"POLQ",1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,138,1,0,"Mitochondrial central dogma > mtDNA maintenance","Mitochondrial central dogma "," mtDNA maintenance"]
["hsa:152926",152926,"PPM1K","DKFZp667B084|DKFZp761G058|PP2Ckappa|PP2Cm|PTMP|UG0882E07|hPTMP",4,"protein phosphatase, Mg2+/Mn2+ dependent, 1K","mitochondrion | protein serine/threonine phosphatase complex | mitochondrial matrix","hydrolase activity | protein serine/threonine phosphatase activity | metal ion binding","protein dephosphorylation","PPM1K","protein phosphatase 1K (PP2C domain containing)","MitoCarta3.0","Metabolism > Amino acid metabolism > Branched-chain amino acid metabolism | Metabolism > Amino acid metabolism > Branched-chain amino acid dehydrogenase complex","Matrix",0,"PP2C",null,null,null,null,"FirstInEnsemblFamily",null,"PPM1K",1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,138,1,0,"Metabolism > Amino acid metabolism > Branched-chain amino acid metabolism | Metabolism > Amino acid metabolism > Branched-chain amino acid dehydrogenase complex","Metabolism "," Amino acid metabolism "," Branched-chain amino acid metabolism "," Metabolism "," Amino acid metabolism "," Branched-chain amino acid dehydrogenase complex"]
["hsa:7001",7001,"PRDX2","MGC4104|NKEFB|PRP|PRX2|PRXII|TDPX1|TPX1|TSA",19,"peroxiredoxin 2","mitochondrion | cytoplasm","oxidoreductase activity | thioredoxin peroxidase activity | antioxidant activity","response to oxidative stress | positive regulation of blood coagulation | negative regulation of NF-kappaB transcription factor activity | homeostasis of number of cells | negative regulation of T cell differentiation | T cell proliferation | negative regulation of reactive oxygen species metabolic process | removal of superoxide radicals | response to lipopolysaccharide | anti-apoptosis | negative regulation of lipopolysaccharide-mediated signaling pathway | activation of MAPK activity | hydrogen peroxide catabolic process | regulation of apoptosis | thymus development | regulation of hydrogen peroxide metabolic process | cellular response to oxidative stress | respiratory burst involved in inflammatory response","PRDX2","peroxiredoxin 2","MitoCarta3.0","Metabolism > Detoxification > ROS and glutathione metabolism","unknown",0,"AhpC-TSA | Redoxin",null,"THIOREDOXIN PEROXIDASE THIOREDOXIN DEPENDENT PEROXIDE REDUCTASE","ENSFM00250000000757",4,null,null,"PRDX2",1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,138,1,0,"Metabolism > Detoxification > ROS and glutathione metabolism","Metabolism "," Detoxification "," ROS and glutathione metabolism"]
["hsa:10935",10935,"PRDX3","AOP-1|AOP1|HBC189|MER5|MGC104387|MGC24293|PRO1748|SP-22|prx-III",10,"peroxiredoxin 3","mitochondrion | IkappaB kinase complex | early endosome | cytoplasm","protein kinase binding | peroxidase activity | protein binding | kinase binding | alkyl hydroperoxide reductase activity | oxidoreductase activity | peroxiredoxin activity | protein C-terminus binding | caspase inhibitor activity | identical protein binding","response to oxidative stress | negative regulation of kinase activity | mitochondrion organization | positive regulation of NF-kappaB transcription factor activity | maternal placenta development | hydrogen peroxide catabolic process | negative regulation of caspase activity | negative regulation of neuron apoptosis | negative regulation of apoptosis | response to hydrogen peroxide | myeloid cell differentiation | positive regulation of cell proliferation | regulation of mitochondrial membrane potential | cellular response to reactive oxygen species | response to lipopolysaccharide","PRDX3","peroxiredoxin 3","MitoCarta3.0","Metabolism > Detoxification > ROS and glutathione metabolism","Matrix",0,"AhpC-TSA | Redoxin",null,"THIOREDOXIN PEROXIDASE THIOREDOXIN DEPENDENT PEROXIDE REDUCTASE","ENSFM00250000000757",4,null,null,"PRDX3",1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,138,1,0,"Metabolism > Detoxification > ROS and glutathione metabolism","Metabolism "," Detoxification "," ROS and glutathione metabolism"]
["hsa:10549",10549,"PRDX4","AOE37-2|PRX-4","X","peroxiredoxin 4","mitochondrion | extracellular space | extracellular region | cytoplasm","oxidoreductase activity | thioredoxin peroxidase activity","I-kappaB phosphorylation","PRDX4","peroxiredoxin 4","MitoCarta3.0","Metabolism > Detoxification > ROS and glutathione metabolism","IMS",0,"AhpC-TSA | Redoxin",null,"THIOREDOXIN PEROXIDASE THIOREDOXIN DEPENDENT PEROXIDE REDUCTASE","ENSFM00250000000757",4,null,null,"PRDX4",1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,138,1,0,"Metabolism > Detoxification > ROS and glutathione metabolism","Metabolism "," Detoxification "," ROS and glutathione metabolism"]
["hsa:9588",9588,"PRDX6","1-Cys|AOP2|KIAA0106|MGC46173|NSGPx|PRX|aiPLA2|p29",1,"peroxiredoxin 6","cytosol | cytoplasmic membrane-bounded vesicle | lysosome | cytoplasm","hydrolase activity | protein binding | phospholipase A2 activity | oxidoreductase activity | peroxiredoxin activity | glutathione peroxidase activity | antioxidant activity","response to oxidative stress | lipid catabolic process | phospholipid catabolic process | hydrogen peroxide catabolic process","PRDX6","peroxiredoxin 6","MitoCarta3.0","Metabolism > Lipid metabolism > Phospholipid metabolism | Metabolism > Detoxification > ROS and glutathione metabolism","unknown",0,"AhpC-TSA | Redoxin","Phenylalanine metabolism",null,null,null,"FirstInEnsemblFamily",null,"PRDX6",1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,138,1,1,"Metabolism > Lipid metabolism > Phospholipid metabolism | Metabolism > Detoxification > ROS and glutathione metabolism","Metabolism "," Lipid metabolism "," Phospholipid metabolism "," Metabolism "," Detoxification "," ROS and glutathione metabolism"]
["hsa:112724",112724,"RDH13","DKFZp313H0740|FLJ39479|SDR7C3",19,"retinol dehydrogenase 13 (all-trans/9-cis)","mitochondrion","oxidoreductase activity | nucleotide binding",null,"RDH13","retinol dehydrogenase 13 (all-trans/9-cis)","MitoCarta3.0","Metabolism > Vitamin metabolism > Vitamin A metabolism","MIM",0,null,null,"RETINOL DEHYDROGENASE","ENSFM00500000269664",5,"FirstInEnsemblFamily",null,"RDH13",1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,138,1,0,"Metabolism > Vitamin metabolism > Vitamin A metabolism","Metabolism "," Vitamin metabolism "," Vitamin A metabolism"]
["hsa:57665",57665,"RDH14","PAN2|SDR7C4",2,"retinol dehydrogenase 14 (all-trans/9-cis/11-cis)","mitochondrion | endoplasmic reticulum","oxidoreductase activity | nucleotide binding",null,"RDH14","retinol dehydrogenase 14 (all-trans/9-cis/11-cis)","MitoCarta3.0","Metabolism > Vitamin metabolism > Vitamin A metabolism","Membrane",1,"adh_short",null,"RETINOL DEHYDROGENASE","ENSFM00500000269664",5,null,null,"RDH14",1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,138,1,0,"Metabolism > Vitamin metabolism > Vitamin A metabolism","Metabolism "," Vitamin metabolism "," Vitamin A metabolism"]
["hsa:54938",54938,"SARS2","FLJ20450|SARS|SARSM|SERS|SYS|SerRS|SerRSmt|mtSerRS",19,"seryl-tRNA synthetase 2, mitochondrial","mitochondrion | mitochondrial matrix | cytoplasm","serine-tRNA ligase activity | nucleotide binding | ligase activity | ATP binding","gene expression | seryl-tRNA aminoacylation | tRNA aminoacylation for protein translation","SARS2","seryl-tRNA synthetase 2","MitoCarta3.0","Mitochondrial central dogma > Translation > mt-tRNA synthetases","Matrix",0,"tRNA-synt_2b | Seryl_tRNA_N","Aminoacyl-tRNA biosynthesis","SERYL TRNA SYNTHETASE MITOCHONDRIAL EC_6.1.1.11 SERINE TRNA LIGASE SERRS SERYL TRNA SER/SEC SYNTHETASE","ENSFM00500000270506",2,"FirstInEnsemblFamily","Hyperuricemia, pulmonary hypertension, renal failure, and alkalosis","SARS2",1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,138,1,1,"Mitochondrial central dogma > Translation > mt-tRNA synthetases","Mitochondrial central dogma "," Translation "," mt-tRNA synthetases"]
["hsa:23410",23410,"SIRT3","SIR2L3",11,"sirtuin 3","mitochondrion | membrane | mitochondrial matrix | cytoplasm","hydrolase activity | protein binding | NAD+ ADP-ribosyltransferase activity | NAD+ binding | zinc ion binding | metal ion binding | hydrolase activity, acting on carbon-nitrogen (but not peptide) bonds, in linear amides","protein ADP-ribosylation | protein deacetylation","SIRT3","sirtuin (silent mating type information regulation 2 homolog) 3 (S. cerevisiae)","MitoCarta3.0","Metabolism > Metals and cofactors > NAD biosynthesis and metabolism | Signaling","Matrix",0,"SIR2",null,"NAD DEPENDENT DEACETYLASE SIRTUIN 2 EC_3.5.1.- SIR2  2","ENSFM00500000269972",2,"FirstInEnsemblFamily",null,"SIRT3",1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,138,1,1,"Metabolism > Metals and cofactors > NAD biosynthesis and metabolism | Signaling","Metabolism "," Metals and cofactors "," NAD biosynthesis and metabolism "," Signaling"]
["hsa:29957",29957,"SLC25A24","APC1|DKFZp586G0123|SCAMC-1",1,"solute carrier family 25 (mitochondrial carrier; phosphate carrier), member 24","mitochondrion | integral to membrane | mitochondrial inner membrane | membrane | cytoplasm","binding | calcium ion binding","transmembrane transport","SLC25A24","solute carrier family 25 (mitochondrial carrier; phosphate carrier), member 24","MitoCarta3.0","Metabolism > Nucleotide metabolism > Nucleotide import | Signaling > Calcium homeostasis > EF hand proteins | Small molecule transport > SLC25A family","MIM",0,"efhand | Mito_carr",null,"CALCIUM BINDING MITOCHONDRIAL CARRIER SCAMC SMALL CALCIUM BINDING MITOCHONDRIAL CARRIER SOLUTE CARRIER FAMILY 25 MEMBER","ENSFM00500000269757",4,"FirstInEnsemblFamily",null,"SLC25A24",1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,138,1,0,"Metabolism > Nucleotide metabolism > Nucleotide import | Signaling > Calcium homeostasis > EF hand proteins | Small molecule transport > SLC25A family","Metabolism "," Nucleotide metabolism "," Nucleotide import "," Signaling "," Calcium homeostasis "," EF hand proteins "," Small molecule transport "," SLC25A family"]
["hsa:81892",81892,"SLIRP","C14orf156|DC50|PD04872",14,"SRA stem-loop interacting RNA binding protein","mitochondrion | nucleus","RNA binding | nucleotide binding","regulation of transcription, DNA-dependent","C14orf156","chromosome 14 open reading frame 156","MitoCarta3.0","Mitochondrial central dogma > mtRNA metabolism > mtRNA stability and decay | Mitochondrial central dogma > Translation","Matrix",0,"RRM_1",null,null,null,null,"FirstInEnsemblFamily",null,"SLIRP",1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,138,1,0,"Mitochondrial central dogma > mtRNA metabolism > mtRNA stability and decay | Mitochondrial central dogma > Translation","Mitochondrial central dogma "," mtRNA metabolism "," mtRNA stability and decay "," Mitochondrial central dogma "," Translation"]
["hsa:80222",80222,"TARS2","FLJ12528|TARSL1",1,"threonyl-tRNA synthetase 2, mitochondrial (putative)","mitochondrion | mitochondrial matrix | cellular_component | cytoplasm","molecular_function | threonine-tRNA ligase activity | nucleotide binding | ligase activity | ATP binding","threonyl-tRNA aminoacylation | mitochondrial threonyl-tRNA aminoacylation | gene expression | tRNA aminoacylation for protein translation","TARSL1","threonyl-tRNA synthetase-like 1","MitoCarta3.0","Mitochondrial central dogma > Translation > mt-tRNA synthetases","Matrix",0,"tRNA-synt_2b | TGS | HGTP_anticodon | tRNA_SAD","Aminoacyl-tRNA biosynthesis","THREONYL TRNA SYNTHETASE EC_6.1.1.3 THREONINE TRNA LIGASE THRRS","ENSFM00250000001453",3,"FirstInEnsemblFamily",null,"TARS2",1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,138,1,0,"Mitochondrial central dogma > Translation > mt-tRNA synthetases","Mitochondrial central dogma "," Translation "," mt-tRNA synthetases"]
["hsa:92609",92609,"TIMM50","MGC102733|TIM50|TIM50L",19,"translocase of inner mitochondrial membrane 50 homolog (S. cerevisiae)","mitochondrion | integral to membrane | nuclear speck | mitochondrial inner membrane | nucleus | membrane | mitochondrial inner membrane presequence translocase complex","protein binding | interleukin-2 receptor binding | protein tyrosine phosphatase activity | RNA binding | ribonucleoprotein complex binding | protein serine/threonine phosphatase activity | phosphoprotein phosphatase activity","protein transport | protein dephosphorylation | release of cytochrome c from mitochondria | peptidyl-tyrosine dephosphorylation | mitochondrial membrane organization","TIMM50","translocase of inner mitochondrial membrane 50 homolog (S. cerevisiae)","MitoCarta3.0","Protein import, sorting and homeostasis > Protein import and sorting > TIM23 presequence pathway","MIM",0,"NIF",null,null,null,null,"FirstInEnsemblFamily",null,"TIMM50",0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,138,1,1,"Protein import, sorting and homeostasis > Protein import and sorting > TIM23 presequence pathway","Protein import, sorting and homeostasis "," Protein import and sorting "," TIM23 presequence pathway"]
["hsa:10131",10131,"TRAP1","HSP75|HSP90L",16,"TNF receptor-associated protein 1","mitochondrion | cellular_component","unfolded protein binding | tumor necrosis factor receptor binding | nucleotide binding | ATP binding","protein folding | cellular response to oxidative stress | response to stress","TRAP1","TNF receptor-associated protein 1","MitoCarta3.0","Protein import, sorting and homeostasis > Protein homeostasis > Chaperones","Matrix",0,"HSP90 | HATPase_c",null,null,null,null,"FirstInEnsemblFamily",null,"TRAP1",1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,138,1,0,"Protein import, sorting and homeostasis > Protein homeostasis > Chaperones","Protein import, sorting and homeostasis "," Protein homeostasis "," Chaperones"]
["hsa:7384",7384,"UQCRC1","D3S3191|QCR1|UQCR1",3,"ubiquinol-cytochrome c reductase core protein I","mitochondrial respiratory chain | mitochondrion | mitochondrial inner membrane | membrane | mitochondrial respiratory chain complex III","ubiquinol-cytochrome-c reductase activity | protein complex binding | metalloendopeptidase activity | zinc ion binding | catalytic activity | metal ion binding","oxidation-reduction process | transport | mitochondrial electron transport, ubiquinol to cytochrome c | respiratory electron transport chain | aerobic respiration | proteolysis | oxidative phosphorylation | response to activity | response to alkaloid","UQCRC1","ubiquinol-cytochrome c reductase core protein I","MitoCarta3.0","Protein import, sorting and homeostasis > Protein import and sorting > Preprotein cleavage | OXPHOS > Complex III > CIII subunits | OXPHOS > OXPHOS subunits","MIM",0,"Peptidase_M16 | Peptidase_M16_C","Oxidative phosphorylation","MITOCHONDRIAL PROCESSING PEPTIDASE SUBUNIT BETA PRECURSOR EC_3.4.24.64 BETA MPP","ENSFM00500000269799",2,"FirstInEnsemblFamily",null,"UQCRC1",1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,138,1,0,"Protein import, sorting and homeostasis > Protein import and sorting > Preprotein cleavage | OXPHOS > Complex III > CIII subunits | OXPHOS > OXPHOS subunits","Protein import, sorting and homeostasis "," Protein import and sorting "," Preprotein cleavage "," OXPHOS "," Complex III "," CIII subunits "," OXPHOS "," OXPHOS subunits"]
["hsa:81554",81554,"WBSCR16","DKFZp434D0421|MGC189739|MGC44931",7,"Williams-Beuren syndrome chromosome region 16","mitochondrion | cellular_component",null,"biological_process","WBSCR16","Williams-Beuren syndrome chromosome region 16","MitoCarta3.0","Mitochondrial central dogma > mtRNA metabolism > mt-rRNA modifications","Matrix",0,"RCC1",null,"WILLIAMS BEUREN SYNDROME CHROMOSOMAL REGION 16","ENSFM00250000003840",2,"FirstInEnsemblFamily",null,"WBSCR16",1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,138,1,0,"Mitochondrial central dogma > mtRNA metabolism > mt-rRNA modifications","Mitochondrial central dogma "," mtRNA metabolism "," mt-rRNA modifications"]
["hsa:150209",150209,"AIFM3","AIFL|FLJ30473|FLJ45137",22,"apoptosis-inducing factor, mitochondrion-associated, 3","cytosol | mitochondrion | endoplasmic reticulum | mitochondrial inner membrane | plasma membrane | nucleus | cytoplasm","oxidoreductase activity | caspase activator activity | 2 iron, 2 sulfur cluster binding | flavin adenine dinucleotide binding | metal ion binding","transport | electron transport chain | activation of caspase activity by cytochrome c | mitochondrial depolarization | cell redox homeostasis | apoptosis | induction of apoptosis","AIFM3","apoptosis-inducing factor, mitochondrion-associated, 3","MitoCarta3.0","Metabolism > Metals and cofactors > Fe-S-containing proteins | Mitochondrial dynamics and surveillance > Apoptosis","MIM",0,"Pyr_redox | Pyr_redox_2 | Rieske",null,null,null,null,"FirstInEnsemblFamily",null,"AIFM3",1,1,1,1,1,1,1,1,1,1,1,1,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,137,1,1,"Metabolism > Metals and cofactors > Fe-S-containing proteins | Mitochondrial dynamics and surveillance > Apoptosis","Metabolism "," Metals and cofactors "," Fe-S-containing proteins "," Mitochondrial dynamics and surveillance "," Apoptosis"]
["hsa:874",874,"CBR3","SDR21C2|hCBR3",21,"carbonyl reductase 3","cytosol | nucleolus | nucleus | cytoplasm","oxidoreductase activity | NADPH binding | 3-keto sterol reductase activity | nucleotide binding | carbonyl reductase (NADPH) activity","phylloquinone catabolic process","CBR3","carbonyl reductase 3","MitoCarta3.0","Metabolism > Detoxification > Xenobiotic metabolism","Matrix",0,"adh_short","Arachidonic acid metabolism","CARBONYL REDUCTASE [NADPH] 1 EC_1.1.1.184 15 HYDROXYPROSTAGLANDIN DEHYDROGENASE [NADP+] EC_1.1.1.- 197 20 BETA HYDROXYSTEROID DEHYDROGENASE NADPH DEPENDENT CARBONYL REDUCTASE 1 PROSTAGLANDIN 9 KETOREDUCTASE PROSTAGLANDIN E 2 9 REDUCTASE EC_1.1.-.- 1 189","ENSFM00250000002301",2,null,null,"CBR3",1,0,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,137,1,1,"Metabolism > Detoxification > Xenobiotic metabolism","Metabolism "," Detoxification "," Xenobiotic metabolism"]
["hsa:80347",80347,"COASY","DPCK|FLJ35179|NBP|PPAT|UKR1|pOV-2",17,"CoA synthase","mitochondrial outer membrane | cellular_component | cytoplasm","pantetheine-phosphate adenylyltransferase activity | transferase activity | nucleotide binding | nucleotidyltransferase activity | ATP binding | dephospho-CoA kinase activity","pantothenate metabolic process | coenzyme A biosynthetic process | biosynthetic process | water-soluble vitamin metabolic process | vitamin metabolic process | coenzyme biosynthetic process","COASY","Coenzyme A synthase","MitoCarta3.0","Metabolism > Metals and cofactors > Coenzyme A metabolism","Matrix",0,"CoaE | CTP_transf_2","Pantothenate and CoA biosynthesis",null,null,null,"FirstInEnsemblFamily",null,"COASY",1,0,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,137,1,1,"Metabolism > Metals and cofactors > Coenzyme A metabolism","Metabolism "," Metals and cofactors "," Coenzyme A metabolism"]
["hsa:79877",79877,"DCAKD","FLJ22955",17,"dephospho-CoA kinase domain containing","mitochondrion","nucleotide binding | ATP binding | dephospho-CoA kinase activity","coenzyme A biosynthetic process","DCAKD","dephospho-CoA kinase domain containing","MitoCarta3.0",0,"Membrane",1,"CoaE",null,null,null,null,"FirstInEnsemblFamily",null,"DCAKD",1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,137,1,1,0,0]
["hsa:1666",1666,"DECR1","DECR|NADPH|SDR18C1",8,"2,4-dienoyl CoA reductase 1, mitochondrial","mitochondrion | nucleolus | plasma membrane | nucleus | mitochondrial matrix | cytoplasm","2,4-dienoyl-CoA reductase (NADPH) activity | NADPH binding | nucleotide binding | oxidoreductase activity, acting on NADH or NADPH","protein homotetramerization | fatty acid beta-oxidation | cellular lipid metabolic process","DECR1","2,4-dienoyl CoA reductase 1, mitochondrial","MitoCarta3.0","Metabolism > Lipid metabolism > Fatty acid oxidation","Matrix",0,"adh_short",null,null,null,null,"FirstInEnsemblFamily",null,"DECR1",1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,137,1,0,"Metabolism > Lipid metabolism > Fatty acid oxidation","Metabolism "," Lipid metabolism "," Fatty acid oxidation"]
["hsa:10901",10901,"DHRS4","CR|FLJ11008|NRDR|PHCR|PSCD|SCAD-SRL|SDR-SRL|SDR25C1|SDR25C2",14,"dehydrogenase/reductase (SDR family) member 4","nuclear membrane | intracellular membrane-bounded organelle | mitochondrion | peroxisome","oxidoreductase activity | nucleotide binding | carbonyl reductase (NADPH) activity","oxidation-reduction process","DHRS4","dehydrogenase/reductase (SDR family) member 4","MitoCarta3.0","Metabolism > Vitamin metabolism > Vitamin A metabolism","Matrix",0,"KR | adh_short","Retinol metabolism | Peroxisome","DEHYDROGENASE/REDUCTASE SDR FAMILY MEMBER 4 EC_1.1.1.184 NADPH DEPENDENT CARBONYL REDUCTASE/NADP RETINOL DEHYDROGENASE CR PHCR NADPH DEPENDENT RETINOL DEHYDROGENASE/REDUCTASE NDRD PEROXISOMAL SHORT CHAIN ALCOHOL DEHYDROGENASE PSCD","ENSFM00500000270135",3,"FirstInEnsemblFamily",null,"DHRS4",1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,137,1,0,"Metabolism > Vitamin metabolism > Vitamin A metabolism","Metabolism "," Vitamin metabolism "," Vitamin A metabolism"]
["hsa:131118",131118,"DNAJC19","Pam18|TIM14|TIMM14",3,"DnaJ (Hsp40) homolog, subfamily C, member 19","mitochondrion | integral to membrane | mitochondrial inner membrane | membrane","heat shock protein binding","protein folding | protein transport | visual perception | protein targeting to mitochondrion | genitalia development",null,null,"MitoCarta3.0","Protein import, sorting and homeostasis > Protein import and sorting > Import motor","MIM",null,"DnaJ",null,null,null,null,"FirstInEnsemblFamily","3-methylglutaconic aciduria, type V","DNAJC19",1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,137,1,1,"Protein import, sorting and homeostasis > Protein import and sorting > Import motor","Protein import, sorting and homeostasis "," Protein import and sorting "," Import motor"]
["hsa:60528",60528,"ELAC2","ELC2|FLJ10530|FLJ36693|FLJ42848|HPC2",17,"elaC homolog 2 (E. coli)","mitochondrion | nucleus","hydrolase activity | metal ion binding | endonuclease activity","tRNA processing","ELAC2","elaC homolog 2 (E. coli)","MitoCarta3.0","Mitochondrial central dogma > mtRNA metabolism > Polycistronic mtRNA processing","Matrix",0,"Lactamase_B","RNA transport",null,null,null,"FirstInEnsemblFamily","Prostate cancer","ELAC2",1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,137,1,1,"Mitochondrial central dogma > mtRNA metabolism > Polycistronic mtRNA processing","Mitochondrial central dogma "," mtRNA metabolism "," Polycistronic mtRNA processing"]
["hsa:23474",23474,"ETHE1","HSCO|YF13H12",19,"ethylmalonic encephalopathy 1","mitochondrion | nucleus | mitochondrial matrix | cytoplasm","hydrolase activity | metal ion binding",null,"ETHE1","ethylmalonic encephalopathy 1","MitoCarta3.0","Metabolism > Sulfur metabolism","Matrix",0,"Lactamase_B",null,null,null,null,"FirstInEnsemblFamily","Ethylmalonic encephalopathy","ETHE1",1,1,1,1,1,1,1,1,1,1,1,1,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,137,1,0,"Metabolism > Sulfur metabolism","Metabolism "," Sulfur metabolism"]
["hsa:2224",2224,"FDPS","FPPS|FPS",1,"farnesyl diphosphate synthase","cytosol | mitochondrion | nucleolus | nucleus | cytoplasm","transferase activity | geranyltranstransferase activity | dimethylallyltranstransferase activity | metal ion binding","isoprenoid biosynthetic process | cholesterol biosynthetic process | interspecies interaction between organisms","FDPS","farnesyl diphosphate synthase (farnesyl pyrophosphate synthetase, dimethylallyltranstransferase, geranyltranstransferase)","MitoCarta3.0","Metabolism > Lipid metabolism > Cholesterol-associated","MIM",0,"polyprenyl_synt","Terpenoid backbone biosynthesis",null,null,null,"FirstInEnsemblFamily",null,"FDPS",1,1,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,137,1,1,"Metabolism > Lipid metabolism > Cholesterol-associated","Metabolism "," Lipid metabolism "," Cholesterol-associated"]
["hsa:3029",3029,"HAGH","GLO2|GLX2|GLXII|HAGH1",16,"hydroxyacylglutathione hydrolase","mitochondrion | mitochondrial matrix | cytoplasm","hydrolase activity | zinc ion binding | hydroxyacylglutathione hydrolase activity | metal ion binding","carbohydrate metabolic process | glutathione biosynthetic process","HAGH","hydroxyacylglutathione hydrolase","MitoCarta3.0","Metabolism > Detoxification > ROS and glutathione metabolism","Matrix",0,"Lactamase_B","Pyruvate metabolism","HYDROXYACYLGLUTATHIONE HYDROLASE MITOCHONDRIAL PRECURSOR EC_3.1.2.6 GLYOXALASE II GLX II","ENSFM00500000270397",2,"FirstInEnsemblFamily",null,"HAGH",1,1,1,1,1,1,1,1,1,1,1,1,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,137,1,1,"Metabolism > Detoxification > ROS and glutathione metabolism","Metabolism "," Detoxification "," ROS and glutathione metabolism"]
["hsa:92483",92483,"LDHAL6B","LDH6B|LDHAL6|LDHL",15,"lactate dehydrogenase A-like 6B","cytoplasm","protein binding | oxidoreductase activity | nucleotide binding | L-lactate dehydrogenase activity","cellular carbohydrate metabolic process | glycolysis","LDHAL6B","lactate dehydrogenase A-like 6B","MitoCarta3.0","Metabolism > Carbohydrate metabolism","Matrix",0,"Ldh_1_N | Ldh_1_C",null,"L LACTATE DEHYDROGENASE CHAIN LDH EC_1.1.1.27","ENSFM00250000000475",5,null,null,"LDHAL6B",1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,137,1,0,"Metabolism > Carbohydrate metabolism","Metabolism "," Carbohydrate metabolism"]
["hsa:51069",51069,"MRPL2","MRP-L14|RPML14",6,"mitochondrial ribosomal protein L2","mitochondrion | ribosome | intracellular","structural constituent of ribosome","translation","MRPL2","mitochondrial ribosomal protein L2","MitoCarta3.0","Mitochondrial central dogma > Translation > Mitochondrial ribosome","Matrix",0,"Ribosomal_L2 | Ribosomal_L2_C",null,null,null,null,"FirstInEnsemblFamily",null,"MRPL2",1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,137,1,1,"Mitochondrial central dogma > Translation > Mitochondrial ribosome","Mitochondrial central dogma "," Translation "," Mitochondrial ribosome"]
["hsa:54148",54148,"MRPL39","C21orf92|FLJ20451|L39mt|MGC104174|MGC3400|MRP-L5|MRPL5|PRED22|PRED66|RPML5",21,"mitochondrial ribosomal protein L39","mitochondrion | ribosome | mitochondrial ribosome","molecular_function | nucleotide binding","biological_process","MRPL39","mitochondrial ribosomal protein L39","MitoCarta3.0","Mitochondrial central dogma > Translation > Mitochondrial ribosome","Matrix",0,"TGS",null,null,null,null,"FirstInEnsemblFamily",null,"MRPL39",1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,137,1,0,"Mitochondrial central dogma > Translation > Mitochondrial ribosome","Mitochondrial central dogma "," Translation "," Mitochondrial ribosome"]
["hsa:4832",4832,"NME3","DR-nm23|KIAA0516|NDPK-C|NDPKC|NM23-H3|NM23H3|c371H6.2",16,"non-metastatic cells 3, protein expressed in","mitochondrion","nucleoside diphosphate kinase activity | transferase activity | kinase activity | nucleotide binding | metal ion binding | ATP binding","UTP biosynthetic process | GTP biosynthetic process | apoptosis | CTP biosynthetic process | nucleotide metabolic process | induction of apoptosis","NME3","non-metastatic cells 3, protein expressed in","MitoCarta3.0","Metabolism > Nucleotide metabolism > Nucleotide synthesis and processing","MOM",0,"NDK","Purine metabolism | Pyrimidine metabolism","NUCLEOSIDE DIPHOSPHATE KINASE NDK NDP KINASE EC_2.7.4.6","ENSFM00250000000895",4,null,null,"NME3",1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,137,1,0,"Metabolism > Nucleotide metabolism > Nucleotide synthesis and processing","Metabolism "," Nucleotide metabolism "," Nucleotide synthesis and processing"]
["hsa:4833",4833,"NME4","NDPK-D|NM23H4|nm23-H4",16,"non-metastatic cells 4, protein expressed in","mitochondrion | mitochondrial inner membrane | mitochondrial intermembrane space","nucleoside diphosphate kinase activity | transferase activity | kinase activity | nucleotide binding | metal ion binding | ATP binding","nucleobase-containing small molecule metabolic process | nucleoside metabolic process | nucleotide metabolic process | UTP biosynthetic process | nucleoside diphosphate phosphorylation | GTP biosynthetic process | CTP biosynthetic process | nucleobase-containing small molecule interconversion","NME4","non-metastatic cells 4, protein expressed in","MitoCarta3.0","Metabolism > Nucleotide metabolism > Nucleotide synthesis and processing","MIM",0,"NDK","Purine metabolism | Pyrimidine metabolism",null,null,null,"FirstInEnsemblFamily",null,"NME4",1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,137,1,0,"Metabolism > Nucleotide metabolism > Nucleotide synthesis and processing","Metabolism "," Nucleotide metabolism "," Nucleotide synthesis and processing"]
["hsa:10201",10201,"NME6","IPIA-ALPHA|NDK 6|NM23-H6",3,"non-metastatic cells 6, protein expressed in (nucleoside-diphosphate kinase)","mitochondrion","nucleoside diphosphate kinase activity | transferase activity | kinase activity | nucleotide binding | metal ion binding | ATP binding","apoptosis | nucleotide metabolic process | negative regulation of cell growth | UTP biosynthetic process | negative regulation of mitosis | nucleoside diphosphate phosphorylation | nucleoside triphosphate biosynthetic process | GTP biosynthetic process | CTP biosynthetic process | anti-apoptosis","NME6","non-metastatic cells 6, protein expressed in (nucleoside-diphosphate kinase)","MitoCarta3.0","Metabolism > Nucleotide metabolism > Nucleotide synthesis and processing","Matrix",0,"NDK","Purine metabolism | Pyrimidine metabolism",null,null,null,"FirstInEnsemblFamily",null,"NME6",1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,137,1,0,"Metabolism > Nucleotide metabolism > Nucleotide synthesis and processing","Metabolism "," Nucleotide metabolism "," Nucleotide synthesis and processing"]
["hsa:5091",5091,"PC","PCB",11,"pyruvate carboxylase","mitochondrion | mitochondrial inner membrane | soluble fraction | mitochondrial matrix","biotin carboxylase activity | carboxylic acid binding | nucleotide binding | ligase activity | ATP binding | pyruvate carboxylase activity | biotin binding | metal ion binding","lipid biosynthetic process | gluconeogenesis | pyruvate metabolic process | oxaloacetate metabolic process | glucose metabolic process | carbohydrate metabolic process","PC","pyruvate carboxylase","MitoCarta3.0","Metabolism > Carbohydrate metabolism > Gluconeogenesis | Metabolism > Carbohydrate metabolism > TCA-associated | Metabolism > Vitamin metabolism > Biotin utilizing proteins","Matrix",0,"ATP-grasp | Biotin_carb_C | CPSase_L_D2 | HMGL-like | PYC_OADA | Biotin_lipoyl | CPSase_L_chain","Pyruvate metabolism | Citrate cycle (TCA cycle)",null,null,null,"FirstInEnsemblFamily","Pyruvate carboxylase deficiency","PC",1,0,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,137,1,0,"Metabolism > Carbohydrate metabolism > Gluconeogenesis | Metabolism > Carbohydrate metabolism > TCA-associated | Metabolism > Vitamin metabolism > Biotin utilizing proteins","Metabolism "," Carbohydrate metabolism "," Gluconeogenesis "," Metabolism "," Carbohydrate metabolism "," TCA-associated "," Metabolism "," Vitamin metabolism "," Biotin utilizing proteins"]
["hsa:54704",54704,"PDP1","FLJ32517|FLJ56179|MGC119646|PDH|PDP|PDPC|PPM2C",8,"pyruvate dehyrogenase phosphatase catalytic subunit 1","mitochondrion | protein serine/threonine phosphatase complex | mitochondrial matrix","hydrolase activity | [pyruvate dehydrogenase (lipoamide)] phosphatase activity | magnesium-dependent protein serine/threonine phosphatase activity | protein complex binding | calcium ion binding","regulation of acetyl-CoA biosynthetic process from pyruvate | pyruvate metabolic process | protein dephosphorylation","PPM2C","protein phosphatase 2C, magnesium-dependent, catalytic subunit","MitoCarta3.0","Metabolism > Carbohydrate metabolism > Pyruvate metabolism","MIM",0,"PP2C",null,"[PYRUVATE DEHYDROGENASE [ACETYL TRANSFERRING]] PHOSPHATASE 1 MITOCHONDRIAL PRECURSOR PDP 1 EC_3.1.3.43 PHOSPHATASE 2C PYRUVATE DEHYDROGENASE PHOSPHATASE CATALYTIC SUBUNIT 1 PDPC 1","ENSFM00250000002844",2,"FirstInEnsemblFamily","Pyruvate dehydrogenase phosphatase deficiency","PDP1",0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,137,1,0,"Metabolism > Carbohydrate metabolism > Pyruvate metabolism","Metabolism "," Carbohydrate metabolism "," Pyruvate metabolism"]
["hsa:57546",57546,"PDP2","KIAA1348|PPM2C2",16,"pyruvate dehyrogenase phosphatase catalytic subunit 2","mitochondrion | protein serine/threonine phosphatase complex | mitochondrial matrix","hydrolase activity | [pyruvate dehydrogenase (lipoamide)] phosphatase activity | magnesium-dependent protein serine/threonine phosphatase activity | metal ion binding","regulation of acetyl-CoA biosynthetic process from pyruvate | pyruvate metabolic process | protein dephosphorylation","PDP2","pyruvate dehydrogenase phosphatase isoenzyme 2","MitoCarta3.0","Metabolism > Carbohydrate metabolism > Pyruvate metabolism","Matrix",0,"PP2C",null,"[PYRUVATE DEHYDROGENASE [ACETYL TRANSFERRING]] PHOSPHATASE 1 MITOCHONDRIAL PRECURSOR PDP 1 EC_3.1.3.43 PHOSPHATASE 2C PYRUVATE DEHYDROGENASE PHOSPHATASE CATALYTIC SUBUNIT 1 PDPC 1","ENSFM00250000002844",2,null,null,"PDP2",0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,137,1,0,"Metabolism > Carbohydrate metabolism > Pyruvate metabolism","Metabolism "," Carbohydrate metabolism "," Pyruvate metabolism"]
["hsa:10531",10531,"PITRM1","KIAA1104|MGC138192|MGC141929|MP1|PreP",10,"pitrilysin metallopeptidase 1","mitochondrion | nucleus | mitochondrial matrix | cytoplasm","peptidase activity | enzyme activator activity | metalloendopeptidase activity | zinc ion binding | metal ion binding","proteolysis | positive regulation of catalytic activity","PITRM1","pitrilysin metallopeptidase 1","MitoCarta3.0","Protein import, sorting and homeostasis > Protein homeostasis > Proteases","Matrix",0,"M16C_assoc | Peptidase_M16_C",null,null,null,null,"FirstInEnsemblFamily",null,"PITRM1",1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,0,1,1,1,1,137,1,1,"Protein import, sorting and homeostasis > Protein homeostasis > Proteases","Protein import, sorting and homeostasis "," Protein homeostasis "," Proteases"]
["hsa:27068",27068,"PPA2","FLJ20459|MGC49850|SID6-306",4,"pyrophosphatase (inorganic) 2","mitochondrion | mitochondrial matrix | cytoplasm","hydrolase activity | inorganic diphosphatase activity | magnesium ion binding","gene expression | tRNA aminoacylation for protein translation | diphosphate metabolic process","PPA2","pyrophosphatase (inorganic) 2","MitoCarta3.0","Mitochondrial central dogma > mtDNA maintenance | Mitochondrial central dogma > mtRNA metabolism | Mitochondrial central dogma > Translation | Metabolism > Nucleotide metabolism","Matrix",0,"Pyrophosphatase","Oxidative phosphorylation","INORGANIC PYROPHOSPHATASE EC_3.6.1.1 PYROPHOSPHATE PHOSPHO HYDROLASE PPASE","ENSFM00250000001403",2,"FirstInEnsemblFamily",null,"PPA2",1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,137,1,0,"Mitochondrial central dogma > mtDNA maintenance | Mitochondrial central dogma > mtRNA metabolism | Mitochondrial central dogma > Translation | Metabolism > Nucleotide metabolism","Mitochondrial central dogma "," mtDNA maintenance ",null," Mitochondrial central dogma "," mtRNA metabolism ",null," Mitochondrial central dogma "," Translation ",null," Metabolism "," Nucleotide metabolism"]
["hsa:57038",57038,"RARS2","ArgRS|DALRD2|MGC14993|MGC23778|PCH6|PRO1992|RARSL|dJ382I10.6",6,"arginyl-tRNA synthetase 2, mitochondrial","mitochondrion | mitochondrial matrix | cytoplasm","arginine-tRNA ligase activity | nucleotide binding | ligase activity | ATP binding","arginyl-tRNA aminoacylation | gene expression | tRNA aminoacylation for protein translation","RARSL","arginyl-tRNA synthetase-like","MitoCarta3.0","Mitochondrial central dogma > Translation > mt-tRNA synthetases","Matrix",0,"tRNA-synt_1d | DALR_1","Aminoacyl-tRNA biosynthesis",null,null,null,"FirstInEnsemblFamily","Pontocerebellar hypoplasia, type 6","RARS2",1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,137,1,0,"Mitochondrial central dogma > Translation > mt-tRNA synthetases","Mitochondrial central dogma "," Translation "," mt-tRNA synthetases"]
["hsa:79085",79085,"SLC25A23","APC2|MCSC2|MGC2615|SCaMC-3",19,"solute carrier family 25 (mitochondrial carrier; phosphate carrier), member 23","mitochondrion | integral to membrane | mitochondrial inner membrane | membrane","binding | calcium ion binding","transmembrane transport","SLC25A23","solute carrier family 25 (mitochondrial carrier; phosphate carrier), member 23","MitoCarta3.0","Metabolism > Nucleotide metabolism > Nucleotide import | Signaling > Calcium homeostasis > EF hand proteins | Small molecule transport > SLC25A family","MIM",0,"efhand | Mito_carr",null,"CALCIUM BINDING MITOCHONDRIAL CARRIER SCAMC SMALL CALCIUM BINDING MITOCHONDRIAL CARRIER SOLUTE CARRIER FAMILY 25 MEMBER","ENSFM00500000269757",4,null,null,"SLC25A23",0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,137,1,1,"Metabolism > Nucleotide metabolism > Nucleotide import | Signaling > Calcium homeostasis > EF hand proteins | Small molecule transport > SLC25A family","Metabolism "," Nucleotide metabolism "," Nucleotide import "," Signaling "," Calcium homeostasis "," EF hand proteins "," Small molecule transport "," SLC25A family"]
["hsa:114789",114789,"SLC25A25","KIAA1896|MCSC|MGC105138|MGC119514|MGC119515|MGC119516|MGC119517|PCSCL|RP11-395P17.4|SCAMC-2",9,"solute carrier family 25 (mitochondrial carrier; phosphate carrier), member 25","mitochondrion | integral to membrane | mitochondrial inner membrane | membrane","binding | calcium ion binding","transmembrane transport","SLC25A25","solute carrier family 25 (mitochondrial carrier; phosphate carrier), member 25","MitoCarta3.0","Metabolism > Nucleotide metabolism > Nucleotide import | Signaling > Calcium homeostasis > EF hand proteins | Small molecule transport > SLC25A family","MIM",0,"efhand | Mito_carr",null,"CALCIUM BINDING MITOCHONDRIAL CARRIER SCAMC SMALL CALCIUM BINDING MITOCHONDRIAL CARRIER SOLUTE CARRIER FAMILY 25 MEMBER","ENSFM00500000269757",4,null,null,"SLC25A25",0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,137,1,0,"Metabolism > Nucleotide metabolism > Nucleotide import | Signaling > Calcium homeostasis > EF hand proteins | Small molecule transport > SLC25A family","Metabolism "," Nucleotide metabolism "," Nucleotide import "," Signaling "," Calcium homeostasis "," EF hand proteins "," Small molecule transport "," SLC25A family"]
["hsa:6648",6648,"SOD2","IPOB|MNSOD|MVCD6",6,"superoxide dismutase 2, mitochondrial","mitochondrial nucleoid | mitochondrion | mitochondrial inner membrane | soluble fraction | mitochondrial matrix | cytoplasm","manganese ion binding | oxidoreductase activity | superoxide dismutase activity | DNA binding | identical protein binding | oxygen binding | metal ion binding","response to cadmium ion | negative regulation of cell proliferation | neuron development | regulation of catalytic activity | liver development | negative regulation of fibroblast proliferation | negative regulation of fat cell differentiation | negative regulation of neuron apoptosis | response to selenium ion | removal of superoxide radicals | response to electrical stimulus | response to L-ascorbic acid | response to lipopolysaccharide | protein homotetramerization | response to nutrient levels | response to zinc ion | post-embryonic development | mitochondrion organization | response to hyperoxia | iron ion homeostasis | negative regulation of apoptosis | response to hydrogen peroxide | hemopoiesis | heart development | erythrophore differentiation | aging | locomotory behavior | age-dependent response to reactive oxygen species | response to drug | response to axon injury | release of cytochrome c from mitochondria | vasodilation by acetylcholine involved in regulation of systemic arterial blood pressure | oxygen homeostasis | glutathione metabolic process | response to activity | hydrogen peroxide biosynthetic process | anti-apoptosis | superoxide metabolic process | detection of oxygen | response to superoxide | response to silicon dioxide | regulation of transcription from RNA polymerase II promoter | response to hypoxia | respiratory electron transport chain | superoxide anion generation | cellular response to ethanol | regulation of mitochondrial membrane potential | positive regulation of nitric oxide biosynthetic process | regulation of blood pressure | response to manganese ion","SOD2","superoxide dismutase 2, mitochondrial","MitoCarta3.0","Metabolism > Detoxification > ROS and glutathione metabolism","Matrix",0,"Sod_Fe_N | Sod_Fe_C","Peroxisome",null,null,null,"FirstInEnsemblFamily",null,"SOD2",1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,137,1,1,"Metabolism > Detoxification > ROS and glutathione metabolism","Metabolism "," Detoxification "," ROS and glutathione metabolism"]
["hsa:51106",51106,"TFB1M","CGI75|mtTFB|mtTFB1",6,"transcription factor B1, mitochondrial","mitochondrial nucleoid | mitochondrion","protein binding | methyltransferase activity | rRNA (adenine-N6,N6-)-dimethyltransferase activity | transferase activity | DNA binding","regulation of transcription, DNA-dependent","TFB1M","transcription factor B1, mitochondrial","MitoCarta3.0","Mitochondrial central dogma > mtRNA metabolism > mtRNA granules | Mitochondrial central dogma > mtRNA metabolism > mt-rRNA modifications | Mitochondrial central dogma > Translation > Mitochondrial ribosome assembly","Matrix",0,"RrnaAD",null,null,null,null,"FirstInEnsemblFamily",null,"TFB1M",1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,137,1,0,"Mitochondrial central dogma > mtRNA metabolism > mtRNA granules | Mitochondrial central dogma > mtRNA metabolism > mt-rRNA modifications | Mitochondrial central dogma > Translation > Mitochondrial ribosome assembly","Mitochondrial central dogma "," mtRNA metabolism "," mtRNA granules "," Mitochondrial central dogma "," mtRNA metabolism "," mt-rRNA modifications "," Mitochondrial central dogma "," Translation "," Mitochondrial ribosome assembly"]
["hsa:7296",7296,"TXNRD1","GRIM-12|MGC9145|TR|TR1|TRXR1|TXNR",12,"thioredoxin reductase 1","cytosol | nucleolus | nucleus | cytoplasm","electron carrier activity | oxidoreductase activity | thioredoxin-disulfide reductase activity | NADP binding | protein disulfide oxidoreductase activity | flavin adenine dinucleotide binding","nucleobase-containing small molecule metabolic process | signal transduction | transport | electron transport chain | cellular lipid metabolic process | cell redox homeostasis | nucleobase-containing small molecule interconversion","TXNRD1","thioredoxin reductase 1","MitoCarta3.0","Metabolism > Detoxification > ROS and glutathione metabolism | Metabolism > Detoxification > Selenoproteins","unknown",0,"Pyr_redox | Pyr_redox_dim | Pyr_redox_2","Selenocompound metabolism | Pyrimidine metabolism",null,null,null,"FirstInEnsemblFamily",null,"TXNRD1",1,1,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,137,1,0,"Metabolism > Detoxification > ROS and glutathione metabolism | Metabolism > Detoxification > Selenoproteins","Metabolism "," Detoxification "," ROS and glutathione metabolism "," Metabolism "," Detoxification "," Selenoproteins"]
["hsa:10587",10587,"TXNRD2","SELZ|TR|TR-BETA|TR3|TRXR2",22,"thioredoxin reductase 2","mitochondrion | cytoplasm","protein binding | oxidoreductase activity | thioredoxin-disulfide reductase activity | NADP binding | protein homodimerization activity | flavin adenine dinucleotide binding","response to selenium ion | response to oxygen radical | hemopoiesis | heart development | cell redox homeostasis","TXNRD2","thioredoxin reductase 2","MitoCarta3.0","Metabolism > Detoxification > ROS and glutathione metabolism | Metabolism > Detoxification > Selenoproteins","Matrix",0,"Pyr_redox | Pyr_redox_dim | Pyr_redox_2","Selenocompound metabolism | Pyrimidine metabolism",null,null,null,"FirstInEnsemblFamily",null,"TXNRD2",1,1,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,137,1,0,"Metabolism > Detoxification > ROS and glutathione metabolism | Metabolism > Detoxification > Selenoproteins","Metabolism "," Detoxification "," ROS and glutathione metabolism "," Metabolism "," Detoxification "," Selenoproteins"]
["hsa:55187",55187,"VPS13D","FLJ23066",1,"vacuolar protein sorting 13 homolog D (S. cerevisiae)",null,null,"protein localization",null,null,"MitoCarta3.0","Mitochondrial dynamics and surveillance > Mitophagy | Mitochondrial dynamics and surveillance > Autophagy","MOM",null,"UBA | DUF1162",null,null,null,null,"FirstInEnsemblFamily",null,"VPS13D",0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,137,1,0,"Mitochondrial dynamics and surveillance > Mitophagy | Mitochondrial dynamics and surveillance > Autophagy","Mitochondrial dynamics and surveillance "," Mitophagy ",null," Mitochondrial dynamics and surveillance "," Autophagy"]
["hsa:10768",10768,"AHCYL1","DCAL|IRBIT|PRO0233|XPVKONA",1,"adenosylhomocysteinase-like 1","endoplasmic reticulum","hydrolase activity | adenosylhomocysteinase activity","positive regulation of sodium ion transport | one-carbon metabolic process | protein export from nucleus | regulation of ion transmembrane transporter activity | mRNA polyadenylation | regulation of mRNA 3'-end processing","AHCYL1","S-adenosylhomocysteine hydrolase-like 1","MitoCarta3.0","Mitochondrial dynamics and surveillance > Organelle contact sites","MOM",0,"AdoHcyase_NAD | AdoHcyase","Cysteine and methionine metabolism","ADENOSYLHOMOCYSTEINASE 3 ADOHCYASE 3 EC_3.3.1.1 S ADENOSYL L HOMOCYSTEINE HYDROLASE 3 S ADENOSYLHOMOCYSTEINE HYDROLASE  2","ENSFM00640001103056",2,"FirstInEnsemblFamily",null,"AHCYL1",1,0,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,136,1,0,"Mitochondrial dynamics and surveillance > Organelle contact sites","Mitochondrial dynamics and surveillance "," Organelle contact sites"]
["hsa:29103",29103,"DNAJC15","DNAJD1|HSD18|MCJ",13,"DnaJ (Hsp40) homolog, subfamily C, member 15","mitochondrion | integral to membrane | membrane","heat shock protein binding",null,"DNAJC15","DnaJ (Hsp40) homolog, subfamily C, member 15","MitoCarta3.0","Protein import, sorting and homeostasis > Protein import and sorting > Import motor","MIM",1,"DnaJ",null,null,null,null,"FirstInEnsemblFamily",null,"DNAJC15",1,1,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,136,1,0,"Protein import, sorting and homeostasis > Protein import and sorting > Import motor","Protein import, sorting and homeostasis "," Protein import and sorting "," Import motor"]
["hsa:10667",10667,"FARS2","FARS1|HSPC320|PheRS|dJ520B18.2",6,"phenylalanyl-tRNA synthetase 2, mitochondrial","mitochondrion | soluble fraction | mitochondrial matrix | cytoplasm","phenylalanine-tRNA ligase activity | magnesium ion binding | tRNA binding | nucleotide binding | ligase activity | ATP binding","tRNA processing | phenylalanyl-tRNA aminoacylation | gene expression | tRNA aminoacylation for protein translation","FARS2","phenylalanine-tRNA synthetase 2 (mitochondrial)","MitoCarta3.0","Mitochondrial central dogma > Translation > mt-tRNA synthetases","Matrix",0,"FDX-ACB | tRNA-synt_2d","Aminoacyl-tRNA biosynthesis",null,null,null,"FirstInEnsemblFamily",null,"FARS2",1,1,1,1,1,0,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,136,1,1,"Mitochondrial central dogma > Translation > mt-tRNA synthetases","Mitochondrial central dogma "," Translation "," mt-tRNA synthetases"]
["hsa:51218",51218,"GLRX5","C14orf87|FLB4739|GRX5|MGC14129|PR01238|PRO1238",14,"glutaredoxin 5","mitochondrion","electron carrier activity | 2 iron, 2 sulfur cluster binding | protein disulfide oxidoreductase activity | metal ion binding","hemopoiesis | cell redox homeostasis","GLRX5","glutaredoxin 5 homolog (S. cerevisiae)","MitoCarta3.0","Metabolism > Metals and cofactors > Fe-S cluster biosynthesis | Metabolism > Metals and cofactors > Fe-S-containing proteins","Matrix",0,"Glutaredoxin",null,null,null,null,"FirstInEnsemblFamily",null,"GLRX5",1,1,0,0,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,136,1,1,"Metabolism > Metals and cofactors > Fe-S cluster biosynthesis | Metabolism > Metals and cofactors > Fe-S-containing proteins","Metabolism "," Metals and cofactors "," Fe-S cluster biosynthesis "," Metabolism "," Metals and cofactors "," Fe-S-containing proteins"]
["hsa:3028",3028,"HSD17B10","17b-HSD10|ABAD|CAMR|DUPXp11.22|ERAB|HADH2|HCD2|MHBD|MRPP2|MRX17|MRX31|MRXS10|SCHAD|SDR5C1","X","hydroxysteroid (17-beta) dehydrogenase 10","mitochondrion | endoplasmic reticulum | mitochondrial inner membrane | plasma membrane | mitochondrial matrix | cytoplasm","cholate 7-alpha-dehydrogenase activity | beta-amyloid binding | 3-hydroxy-2-methylbutyryl-CoA dehydrogenase activity | estrogen receptor binding | nucleotide binding | protein binding | oxidoreductase activity | NAD binding | steroid binding | identical protein binding | acetoacetyl-CoA reductase activity | 3-hydroxyacyl-CoA dehydrogenase activity | estradiol 17-beta-dehydrogenase activity","branched chain family amino acid catabolic process | tRNA processing | protein homotetramerization | cellular nitrogen compound metabolic process | Leydig cell differentiation | cell aging | lipid metabolic process","HSD17B10","hydroxysteroid (17-beta) dehydrogenase 10","MitoCarta3.0","Mitochondrial central dogma > mtRNA metabolism > mtRNA granules | Mitochondrial central dogma > mtRNA metabolism > Polycistronic mtRNA processing | Mitochondrial central dogma > mtRNA metabolism > mt-tRNA modifications | Metabolism > Lipid metabolism > Fatty acid oxidation | Metabolism > Lipid metabolism > Cholesterol, bile acid, steroid synthesis | Metabolism > Amino acid metabolism > Branched-chain amino acid metabolism","Matrix",0,"KR | adh_short | Epimerase","Valine, leucine and isoleucine degradation",null,null,null,"FirstInEnsemblFamily","Mental retardation, X-linked 17/31, microduplication|\"Mental retardation, X-linked syndromic 10\"|17-beta-hydroxysteroid dehydrogenase X deficiency","HSD17B10",1,0,1,1,0,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,136,1,0,"Mitochondrial central dogma > mtRNA metabolism > mtRNA granules | Mitochondrial central dogma > mtRNA metabolism > Polycistronic mtRNA processing | Mitochondrial central dogma > mtRNA metabolism > mt-tRNA modifications | Metabolism > Lipid metabolism > Fatty acid oxidation | Metabolism > Lipid metabolism > Cholesterol, bile acid, steroid synthesis | Metabolism > Amino acid metabolism > Branched-chain amino acid metabolism","Mitochondrial central dogma "," mtRNA metabolism "," mtRNA granules "," Mitochondrial central dogma "," mtRNA metabolism "," Polycistronic mtRNA processing "," Mitochondrial central dogma "," mtRNA metabolism "," mt-tRNA modifications "," Metabolism "," Lipid metabolism "," Fatty acid oxidation "," Metabolism "," Lipid metabolism "," Cholesterol, bile acid, steroid synthesis "]
["hsa:83693",83693,"HSDL1","MGC125994|MGC125995|MGC126032|SDR12C3",16,"hydroxysteroid dehydrogenase like 1","mitochondrion","oxidoreductase activity | nucleotide binding",null,"HSDL1","hydroxysteroid dehydrogenase like 1","MitoCarta3.0",0,"unknown",0,"adh_short",null,null,null,null,"FirstInEnsemblFamily",null,"HSDL1",1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,136,1,0,0,0]
["hsa:56922",56922,"MCCC1","DKFZp686B20267|FLJ25545|MCC-B|MCCA",3,"methylcrotonoyl-CoA carboxylase 1 (alpha)","mitochondrion | mitochondrial inner membrane | mitochondrial matrix","biotin carboxylase activity | biotin binding | methylcrotonoyl-CoA carboxylase activity | nucleotide binding | metal ion binding | ligase activity | ATP binding","branched chain family amino acid catabolic process | biotin metabolic process | cellular nitrogen compound metabolic process | leucine catabolic process","MCCC1","methylcrotonoyl-Coenzyme A carboxylase 1 (alpha)","MitoCarta3.0","Metabolism > Amino acid metabolism > Branched-chain amino acid metabolism | Metabolism > Vitamin metabolism > Biotin utilizing proteins","Matrix",0,"ATP-grasp | Biotin_carb_C | CPSase_L_D2 | RimK | Biotin_lipoyl | CPSase_L_chain","Valine, leucine and isoleucine degradation","PROPIONYL COA CARBOXYLASE ALPHA CHAIN MITOCHONDRIAL PRECURSOR PCCASE SUBUNIT ALPHA EC_6.4.1.3 PROPANOYL COA:CARBON DIOXIDE LIGASE SUBUNIT ALPHA","ENSFM00570000851021",2,"FirstInEnsemblFamily","3-Methylcrotonyl-CoA carboxylase 1 deficiency","MCCC1",1,0,1,1,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,136,1,1,"Metabolism > Amino acid metabolism > Branched-chain amino acid metabolism | Metabolism > Vitamin metabolism > Biotin utilizing proteins","Metabolism "," Amino acid metabolism "," Branched-chain amino acid metabolism "," Metabolism "," Vitamin metabolism "," Biotin utilizing proteins"]
["hsa:4191",4191,"MDH2","M-MDH|MDH|MGC:3559|MOR1",7,"malate dehydrogenase 2, NAD (mitochondrial)","mitochondrion | mitochondrial inner membrane | nucleolus | plasma membrane | nucleus | mitochondrial matrix","L-malate dehydrogenase activity | oxidoreductase activity | protein self-association | nucleotide binding | malate dehydrogenase (NADP+) activity","internal protein amino acid acetylation | oxaloacetate metabolic process | carbohydrate metabolic process | NADH metabolic process | cellular carbohydrate metabolic process | gluconeogenesis | tricarboxylic acid cycle | malate metabolic process | glucose metabolic process","MDH2","malate dehydrogenase 2, NAD (mitochondrial)","MitoCarta3.0","Metabolism > Carbohydrate metabolism > Gluconeogenesis | Metabolism > Carbohydrate metabolism > Malate-aspartate shuttle | Metabolism > Carbohydrate metabolism > TCA cycle","Matrix",0,"Ldh_1_N | Ldh_1_C","Glyoxylate and dicarboxylate metabolism | Pyruvate metabolism | Citrate cycle (TCA cycle)",null,null,null,"FirstInEnsemblFamily",null,"MDH2",1,0,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,136,1,0,"Metabolism > Carbohydrate metabolism > Gluconeogenesis | Metabolism > Carbohydrate metabolism > Malate-aspartate shuttle | Metabolism > Carbohydrate metabolism > TCA cycle","Metabolism "," Carbohydrate metabolism "," Gluconeogenesis "," Metabolism "," Carbohydrate metabolism "," Malate-aspartate shuttle "," Metabolism "," Carbohydrate metabolism "," TCA cycle"]
["hsa:254042",254042,"METAP1D","MAP1D|Metap1l",2,"methionyl aminopeptidase type 1D (mitochondrial)","mitochondrion","peptidase activity | metalloexopeptidase activity | aminopeptidase activity | metal ion binding","peptidyl-methionine modification | proteolysis | N-terminal protein amino acid modification | cellular process","MAP1D","methionine aminopeptidase 1D","MitoCarta3.0","Mitochondrial central dogma > Translation > fMet processing | Protein import, sorting and homeostasis > Protein homeostasis > Proteases","Matrix",0,"Peptidase_M24",null,"METHIONINE AMINOPEPTIDASE 1 MAP 1 METAP 1 EC_3.4.11.18 PEPTIDASE M 1","ENSFM00250000001965",2,"FirstInEnsemblFamily",null,"METAP1D",1,0,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,136,1,0,"Mitochondrial central dogma > Translation > fMet processing | Protein import, sorting and homeostasis > Protein homeostasis > Proteases","Mitochondrial central dogma "," Translation "," fMet processing "," Protein import, sorting and homeostasis "," Protein homeostasis "," Proteases"]
["hsa:6183",6183,"MRPS12","MPR-S12|MT-RPS12|RPMS12|RPS12|RPSM12",19,"mitochondrial ribosomal protein S12","mitochondrion | small ribosomal subunit | mitochondrial ribosome | intracellular","protein binding | structural constituent of ribosome","translation","MRPS12","mitochondrial ribosomal protein S12","MitoCarta3.0","Mitochondrial central dogma > Translation > Mitochondrial ribosome","Matrix",0,"Ribosomal_S12",null,null,null,null,"FirstInEnsemblFamily",null,"MRPS12",1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,136,1,1,"Mitochondrial central dogma > Translation > Mitochondrial ribosome","Mitochondrial central dogma "," Translation "," Mitochondrial ribosome"]
["hsa:80025",80025,"PANK2","C20orf48|FLJ17232|HARP|HSS|MGC15053|NBIA1|PKAN",20,"pantothenate kinase 2","mitochondrion | nucleolus | nucleus | mitochondrial intermembrane space | cytoplasm","transferase activity | pantothenate kinase activity | nucleotide binding | ATP binding","pantothenate metabolic process | cell death | coenzyme A biosynthetic process | water-soluble vitamin metabolic process | vitamin metabolic process | coenzyme biosynthetic process","PANK2","pantothenate kinase 2 (Hallervorden-Spatz syndrome)","MitoCarta3.0","Metabolism > Metals and cofactors > Coenzyme A metabolism","IMS",0,"Fumble","Pantothenate and CoA biosynthesis","PANTOTHENATE KINASE EC_2.7.1.33 PANTOTHENIC ACID KINASE","ENSFM00260000050538",3,"FirstInEnsemblFamily","HARP syndrome|Neurodegeneration with brain iron accumulation 1","PANK2",0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,136,1,0,"Metabolism > Metals and cofactors > Coenzyme A metabolism","Metabolism "," Metals and cofactors "," Coenzyme A metabolism"]
["hsa:23203",23203,"PMPCA","Alpha-MPP|FLJ26258|INPP5E|KIAA0123|MGC104197",9,"peptidase (mitochondrial processing) alpha","mitochondrion | mitochondrial inner membrane | mitochondrial matrix","peptidase activity | metalloendopeptidase activity | zinc ion binding | catalytic activity | metal ion binding","proteolysis","PMPCA","peptidase (mitochondrial processing) alpha","MitoCarta3.0","Protein import, sorting and homeostasis > Protein import and sorting > Preprotein cleavage | Protein import, sorting and homeostasis > Protein homeostasis > Proteases","Matrix",0,"Peptidase_M16 | Peptidase_M16_C",null,null,null,null,"FirstInEnsemblFamily",null,"PMPCA",1,1,0,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,136,1,1,"Protein import, sorting and homeostasis > Protein import and sorting > Preprotein cleavage | Protein import, sorting and homeostasis > Protein homeostasis > Proteases","Protein import, sorting and homeostasis "," Protein import and sorting "," Preprotein cleavage "," Protein import, sorting and homeostasis "," Protein homeostasis "," Proteases"]
["hsa:51651",51651,"PTRH2","BIT1|FLJ32471|PTH2",17,"peptidyl-tRNA hydrolase 2","mitochondrion","hydrolase activity | aminoacyl-tRNA hydrolase activity","apoptosis","PTRH2","peptidyl-tRNA hydrolase 2","MitoCarta3.0",0,"MOM",1,"PTH2",null,null,null,null,"FirstInEnsemblFamily",null,"PTRH2",1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,136,1,1,0,0]
["hsa:54931",54931,"RG9MTD1","FLJ20432|MRPP1",3,"RNA (guanine-9-) methyltransferase domain containing 1","mitochondrion","protein binding | methyltransferase activity | transferase activity","tRNA processing","RG9MTD1","RNA (guanine-9-) methyltransferase domain containing 1","MitoCarta3.0","Mitochondrial central dogma > mtRNA metabolism > mtRNA granules | Mitochondrial central dogma > mtRNA metabolism > Polycistronic mtRNA processing | Mitochondrial central dogma > mtRNA metabolism > mt-tRNA modifications","Matrix",0,"tRNA_m1G_MT",null,null,null,null,"FirstInEnsemblFamily",null,"RG9MTD1",0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,136,1,0,"Mitochondrial central dogma > mtRNA metabolism > mtRNA granules | Mitochondrial central dogma > mtRNA metabolism > Polycistronic mtRNA processing | Mitochondrial central dogma > mtRNA metabolism > mt-tRNA modifications","Mitochondrial central dogma "," mtRNA metabolism "," mtRNA granules "," Mitochondrial central dogma "," mtRNA metabolism "," Polycistronic mtRNA processing "," Mitochondrial central dogma "," mtRNA metabolism "," mt-tRNA modifications"]
["hsa:6576",6576,"SLC25A1","CTP|SEA|SLC20A3",22,"solute carrier family 25 (mitochondrial carrier; citrate transporter), member 1","mitochondrion | integral to membrane | mitochondrial inner membrane | membrane","citrate transmembrane transporter activity | binding","long-chain fatty-acyl-CoA biosynthetic process | triglyceride biosynthetic process | gluconeogenesis | mitochondrial citrate transport | cellular lipid metabolic process | glucose metabolic process | carbohydrate metabolic process | transmembrane transport","SLC25A1","solute carrier family 25 (mitochondrial carrier; citrate transporter), member 1","MitoCarta3.0","Metabolism > Carbohydrate metabolism > Gluconeogenesis | Metabolism > Carbohydrate metabolism > TCA-associated | Metabolism > Lipid metabolism | Small molecule transport > SLC25A family","MIM",0,"Mito_carr",null,null,null,null,"FirstInEnsemblFamily",null,"SLC25A1",0,0,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,136,1,0,"Metabolism > Carbohydrate metabolism > Gluconeogenesis | Metabolism > Carbohydrate metabolism > TCA-associated | Metabolism > Lipid metabolism | Small molecule transport > SLC25A family","Metabolism "," Carbohydrate metabolism "," Gluconeogenesis "," Metabolism "," Carbohydrate metabolism "," TCA-associated "," Metabolism "," Lipid metabolism ",null," Small molecule transport "," SLC25A family"]
["hsa:1468",1468,"SLC25A10","DIC",17,"solute carrier family 25 (mitochondrial carrier; dicarboxylate transporter), member 10","mitochondrion | integral to membrane | mitochondrial inner membrane | nucleolus | nucleus | membrane","thiosulfate transmembrane transporter activity | phosphate ion transmembrane transporter activity | sulfate transmembrane transporter activity | succinate transmembrane transporter activity | antiporter activity | protein binding | dicarboxylic acid transmembrane transporter activity | malate transmembrane transporter activity","dicarboxylic acid transport | transport | gluconeogenesis | mitochondrial transport | glucose metabolic process | carbohydrate metabolic process","SLC25A10","solute carrier family 25 (mitochondrial carrier; dicarboxylate transporter), member 10","MitoCarta3.0","Metabolism > Carbohydrate metabolism > Gluconeogenesis | Metabolism > Carbohydrate metabolism > Malate-aspartate shuttle | Small molecule transport > SLC25A family","MIM",0,"Mito_carr",null,"39S RIBOSOMAL L12 MITOCHONDRIAL PRECURSOR L12MT MRP L12","ENSFM00500000270452",2,"FirstInEnsemblFamily",null,"SLC25A10",0,0,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,136,1,0,"Metabolism > Carbohydrate metabolism > Gluconeogenesis | Metabolism > Carbohydrate metabolism > Malate-aspartate shuttle | Small molecule transport > SLC25A family","Metabolism "," Carbohydrate metabolism "," Gluconeogenesis "," Metabolism "," Carbohydrate metabolism "," Malate-aspartate shuttle "," Small molecule transport "," SLC25A family"]
["hsa:8402",8402,"SLC25A11","OGC|SLC20A4",17,"solute carrier family 25 (mitochondrial carrier; oxoglutarate carrier), member 11","mitochondrion | mitochondrial inner membrane | integral to plasma membrane | membrane","oxoglutarate:malate antiporter activity | transporter activity","transport | gluconeogenesis | alpha-ketoglutarate transport | glucose metabolic process | carbohydrate metabolic process","SLC25A11","solute carrier family 25 (mitochondrial carrier; oxoglutarate carrier), member 11","MitoCarta3.0","Metabolism > Carbohydrate metabolism > Gluconeogenesis | Metabolism > Carbohydrate metabolism > Malate-aspartate shuttle | Metabolism > Carbohydrate metabolism > TCA-associated | Small molecule transport > SLC25A family","MIM",0,"Mito_carr",null,null,null,null,"FirstInEnsemblFamily",null,"SLC25A11",0,0,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,136,1,1,"Metabolism > Carbohydrate metabolism > Gluconeogenesis | Metabolism > Carbohydrate metabolism > Malate-aspartate shuttle | Metabolism > Carbohydrate metabolism > TCA-associated | Small molecule transport > SLC25A family","Metabolism "," Carbohydrate metabolism "," Gluconeogenesis "," Metabolism "," Carbohydrate metabolism "," Malate-aspartate shuttle "," Metabolism "," Carbohydrate metabolism "," TCA-associated "," Small molecule transport "," SLC25A family"]
["hsa:8604",8604,"SLC25A12","AGC1|ARALAR",2,"solute carrier family 25 (mitochondrial carrier, Aralar), member 12","mitochondrion | integral to membrane | mitochondrial inner membrane | membrane","L-aspartate transmembrane transporter activity | binding | L-glutamate transmembrane transporter activity | calcium ion binding","L-glutamate transport | response to calcium ion | gluconeogenesis | malate-aspartate shuttle | aspartate transport | glucose metabolic process | carbohydrate metabolic process | transmembrane transport","SLC25A12","solute carrier family 25 (mitochondrial carrier, Aralar), member 12","MitoCarta3.0","Metabolism > Carbohydrate metabolism > Malate-aspartate shuttle | Metabolism > Amino acid metabolism > Glutamate metabolism | Signaling > Calcium homeostasis > EF hand proteins | Small molecule transport > SLC25A family","MIM",0,"efhand | Mito_carr",null,"CALCIUM BINDING MITOCHONDRIAL CARRIER MITOCHONDRIAL ASPARTATE GLUTAMATE CARRIER SOLUTE CARRIER FAMILY 25 MEMBER","ENSFM00500000269959",2,null,"Hypomyelination, global cerebral","SLC25A12",0,0,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,136,1,1,"Metabolism > Carbohydrate metabolism > Malate-aspartate shuttle | Metabolism > Amino acid metabolism > Glutamate metabolism | Signaling > Calcium homeostasis > EF hand proteins | Small molecule transport > SLC25A family","Metabolism "," Carbohydrate metabolism "," Malate-aspartate shuttle "," Metabolism "," Amino acid metabolism "," Glutamate metabolism "," Signaling "," Calcium homeostasis "," EF hand proteins "," Small molecule transport "," SLC25A family"]
["hsa:10165",10165,"SLC25A13","ARALAR2|CITRIN|CTLN2",7,"solute carrier family 25, member 13 (citrin)","mitochondrion | integral to membrane | mitochondrial inner membrane | integral to plasma membrane | membrane","L-aspartate transmembrane transporter activity | binding | transporter activity | L-glutamate transmembrane transporter activity | calcium ion binding","transport | response to calcium ion | aspartate transport | carbohydrate metabolic process | transmembrane transport | L-glutamate transport | cellular respiration | gluconeogenesis | malate-aspartate shuttle | glucose metabolic process | ATP biosynthetic process","SLC25A13","solute carrier family 25, member 13 (citrin)","MitoCarta3.0","Metabolism > Carbohydrate metabolism > Malate-aspartate shuttle | Metabolism > Amino acid metabolism > Urea cycle | Signaling > Calcium homeostasis > EF hand proteins | Small molecule transport > SLC25A family","MIM",0,"efhand | Mito_carr",null,"CALCIUM BINDING MITOCHONDRIAL CARRIER MITOCHONDRIAL ASPARTATE GLUTAMATE CARRIER SOLUTE CARRIER FAMILY 25 MEMBER","ENSFM00500000269959",2,"FirstInEnsemblFamily","Citrullinemia, adult-onset type II|\"Citrullinemia, type II, neonatal-onset\"","SLC25A13",0,0,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,136,1,0,"Metabolism > Carbohydrate metabolism > Malate-aspartate shuttle | Metabolism > Amino acid metabolism > Urea cycle | Signaling > Calcium homeostasis > EF hand proteins | Small molecule transport > SLC25A family","Metabolism "," Carbohydrate metabolism "," Malate-aspartate shuttle "," Metabolism "," Amino acid metabolism "," Urea cycle "," Signaling "," Calcium homeostasis "," EF hand proteins "," Small molecule transport "," SLC25A family"]
["hsa:9016",9016,"SLC25A14","BMCP1|MGC149543|UCP5","X","solute carrier family 25 (mitochondrial carrier, brain), member 14","mitochondrial envelope | mitochondrion | integral to membrane | mitochondrial inner membrane | integral to plasma membrane | membrane","binding","transport | mitochondrial transport | aerobic respiration","SLC25A14","solute carrier family 25 (mitochondrial carrier, brain), member 14","MitoCarta3.0","Metabolism > Sulfur metabolism | Small molecule transport > SLC25A family","MIM",0,"Mito_carr",null,"KIDNEY MITOCHONDRIAL CARRIER 1 SOLUTE CARRIER FAMILY 25 MEMBER 30","ENSFM00500000270162",2,"FirstInEnsemblFamily",null,"SLC25A14",0,0,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,136,1,0,"Metabolism > Sulfur metabolism | Small molecule transport > SLC25A family","Metabolism "," Sulfur metabolism ",null," Small molecule transport "," SLC25A family"]
["hsa:10166",10166,"SLC25A15","D13S327|HHH|ORC1|ORNT1",13,"solute carrier family 25 (mitochondrial carrier; ornithine transporter) member 15","mitochondrion | integral to membrane | mitochondrial inner membrane | membrane","L-ornithine transmembrane transporter activity","transport | urea cycle | mitochondrial ornithine transport | cellular nitrogen compound metabolic process | cellular amino acid metabolic process","SLC25A15","solute carrier family 25 (mitochondrial carrier; ornithine transporter) member 15","MitoCarta3.0","Metabolism > Amino acid metabolism > Urea cycle | Small molecule transport > SLC25A family","MIM",0,"Mito_carr",null,"MITOCHONDRIAL ORNITHINE TRANSPORTER SOLUTE CARRIER FAMILY 25 MEMBER","ENSFM00470000251456",2,"FirstInEnsemblFamily","Hyperornithinemia-hyperammonemia-homocitrullinemia syndrome","SLC25A15",0,0,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,136,1,0,"Metabolism > Amino acid metabolism > Urea cycle | Small molecule transport > SLC25A family","Metabolism "," Amino acid metabolism "," Urea cycle "," Small molecule transport "," SLC25A family"]
["hsa:8034",8034,"SLC25A16","D10S105E|GDA|GDC|HGT.1|MGC39851|ML7|hML7",10,"solute carrier family 25 (mitochondrial carrier; Graves disease autoantigen), member 16","mitochondrion | integral to membrane | mitochondrial inner membrane | membrane","solute:solute antiporter activity | binding","pantothenate metabolic process | transport | water-soluble vitamin metabolic process | vitamin metabolic process | coenzyme biosynthetic process | transmembrane transport","SLC25A16","solute carrier family 25 (mitochondrial carrier; Graves disease autoantigen), member 16","MitoCarta3.0","Small molecule transport > SLC25A family","MIM",0,"Mito_carr",null,null,null,null,"FirstInEnsemblFamily",null,"SLC25A16",0,0,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,136,1,1,"Small molecule transport > SLC25A family","Small molecule transport "," SLC25A family"]
["hsa:83733",83733,"SLC25A18","GC2",22,"solute carrier family 25 (mitochondrial carrier), member 18","mitochondrion | integral to membrane | mitochondrial inner membrane | membrane","binding | symporter activity","transmembrane transport","SLC25A18","solute carrier family 25 (mitochondrial carrier), member 18","MitoCarta3.0","Small molecule transport > SLC25A family","MIM",2,"Mito_carr",null,"MITOCHONDRIAL GLUTAMATE CARRIER 1 GC 1 GLUTAMATE/H + SYMPORTER 1 SOLUTE CARRIER FAMILY 25 MEMBER 22","ENSFM00500000270170",2,null,null,"SLC25A18",0,0,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,136,1,0,"Small molecule transport > SLC25A family","Small molecule transport "," SLC25A family"]
["hsa:60386",60386,"SLC25A19","DNC|MCPHA|MUP1|TPC",17,"solute carrier family 25 (mitochondrial thiamine pyrophosphate carrier), member 19","mitochondrion | integral to membrane | mitochondrial inner membrane | membrane","deoxynucleotide transmembrane transporter activity | binding","deoxynucleotide transport | transmembrane transport","SLC25A19","solute carrier family 25 (mitochondrial deoxynucleotide carrier), member 19","MitoCarta3.0","Metabolism > Vitamin metabolism > Vitamin B1 metabolism | Small molecule transport > SLC25A family","MIM",0,"Mito_carr",null,null,null,null,"FirstInEnsemblFamily","Microcephaly, Amish type|Thiamine metabolism dysfunction syndrome 4 (progressive polyneuropathy type)","SLC25A19",0,0,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,136,1,1,"Metabolism > Vitamin metabolism > Vitamin B1 metabolism | Small molecule transport > SLC25A family","Metabolism "," Vitamin metabolism "," Vitamin B1 metabolism "," Small molecule transport "," SLC25A family"]
["hsa:788",788,"SLC25A20","CAC|CACT",3,"solute carrier family 25 (carnitine/acylcarnitine translocase), member 20","mitochondrion | integral to membrane | mitochondrial inner membrane | membrane",null,"transport | regulation of fatty acid oxidation | cellular lipid metabolic process | carnitine shuttle","SLC25A20","solute carrier family 25 (carnitine/acylcarnitine translocase), member 20","MitoCarta3.0","Metabolism > Lipid metabolism > Fatty acid oxidation | Metabolism > Metals and cofactors > Carnitine synthesis and transport | Metabolism > Metals and cofactors > Carnitine shuttle | Small molecule transport > SLC25A family","MIM",3,"Mito_carr",null,null,null,null,"FirstInEnsemblFamily","Carnitine-acylcarnitine translocase deficiency","SLC25A20",0,0,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,136,1,0,"Metabolism > Lipid metabolism > Fatty acid oxidation | Metabolism > Metals and cofactors > Carnitine synthesis and transport | Metabolism > Metals and cofactors > Carnitine shuttle | Small molecule transport > SLC25A family","Metabolism "," Lipid metabolism "," Fatty acid oxidation "," Metabolism "," Metals and cofactors "," Carnitine synthesis and transport "," Metabolism "," Metals and cofactors "," Carnitine shuttle "," Small molecule transport "," SLC25A family"]
["hsa:89874",89874,"SLC25A21","MGC126570|ODC|ODC1",14,"solute carrier family 25 (mitochondrial oxodicarboxylate carrier), member 21","mitochondrion | integral to membrane | mitochondrial inner membrane | membrane","binding","cellular nitrogen compound metabolic process | lysine catabolic process | transmembrane transport","SLC25A21","solute carrier family 25 (mitochondrial oxodicarboxylate carrier), member 21","MitoCarta3.0","Metabolism > Amino acid metabolism | Small molecule transport > SLC25A family","MIM",0,"Mito_carr",null,null,null,null,"FirstInEnsemblFamily",null,"SLC25A21",0,0,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,136,1,0,"Metabolism > Amino acid metabolism | Small molecule transport > SLC25A family","Metabolism "," Amino acid metabolism ",null," Small molecule transport "," SLC25A family"]
["hsa:79751",79751,"SLC25A22","EIEE3|FLJ13044|GC1|NET44",11,"solute carrier family 25 (mitochondrial carrier: glutamate), member 22","mitochondrion | integral to membrane | mitochondrial inner membrane | nucleolus | nucleus | membrane","binding | symporter activity | L-glutamate transmembrane transporter activity","L-glutamate transport | transmembrane transport","SLC25A22","solute carrier family 25 (mitochondrial carrier: glutamate), member 22","MitoCarta3.0","Small molecule transport > SLC25A family","MIM",1,"Mito_carr",null,"MITOCHONDRIAL GLUTAMATE CARRIER 1 GC 1 GLUTAMATE/H + SYMPORTER 1 SOLUTE CARRIER FAMILY 25 MEMBER 22","ENSFM00500000270170",2,"FirstInEnsemblFamily","Epileptic encephalopathy, early infantile, 3","SLC25A22",0,0,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,136,1,0,"Small molecule transport > SLC25A family","Small molecule transport "," SLC25A family"]
["hsa:115286",115286,"SLC25A26","DKFZp434E079|FLJ77340|SAMC",3,"solute carrier family 25, member 26","mitochondrion | integral to membrane | mitochondrial inner membrane | nucleolus | nucleus | membrane","S-adenosylmethionine transmembrane transporter activity","transport | S-adenosylmethionine transport","SLC25A26","solute carrier family 25, member 26","MitoCarta3.0","Small molecule transport > SLC25A family","MIM",3,"Mito_carr",null,null,null,null,"FirstInEnsemblFamily",null,"SLC25A26",0,0,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,136,1,1,"Small molecule transport > SLC25A family","Small molecule transport "," SLC25A family"]
["hsa:81894",81894,"SLC25A28","DKFZp547C109|MFRN2|MRS3/4|MRS4L",10,"solute carrier family 25, member 28","mitochondrion | integral to membrane | mitochondrial inner membrane | membrane",null,"ion transport | iron ion homeostasis","SLC25A28","solute carrier family 25, member 28","MitoCarta3.0","Metabolism > Metals and cofactors > Iron homeostasis | Small molecule transport > SLC25A family","MIM",0,"Mito_carr",null,"MITOFERRIN MITOCHONDRIAL IRON TRANSPORTER SOLUTE CARRIER FAMILY 25 MEMBER","ENSFM00250000002181",2,"FirstInEnsemblFamily",null,"SLC25A28",0,0,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,136,1,0,"Metabolism > Metals and cofactors > Iron homeostasis | Small molecule transport > SLC25A family","Metabolism "," Metals and cofactors "," Iron homeostasis "," Small molecule transport "," SLC25A family"]
["hsa:123096",123096,"SLC25A29","C14orf69|CACL|FLJ38975",14,"solute carrier family 25, member 29","mitochondrion | integral to membrane | mitochondrial inner membrane | membrane","acyl carnitine transporter activity | binding","transmembrane transport","SLC25A29","solute carrier family 25, member 29","MitoCarta3.0","Metabolism > Amino acid metabolism > Lysine metabolism | Small molecule transport > SLC25A family","MIM",0,"Mito_carr",null,null,null,null,"FirstInEnsemblFamily",null,"SLC25A29",0,0,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,136,1,0,"Metabolism > Amino acid metabolism > Lysine metabolism | Small molecule transport > SLC25A family","Metabolism "," Amino acid metabolism "," Lysine metabolism "," Small molecule transport "," SLC25A family"]
["hsa:5250",5250,"SLC25A3","PHC|PTP",12,"solute carrier family 25 (mitochondrial carrier; phosphate carrier), member 3","mitochondrion | mitochondrial inner membrane | integral to plasma membrane | membrane","phosphate ion carrier activity | symporter activity","transport | generation of precursor metabolites and energy","SLC25A3","solute carrier family 25 (mitochondrial carrier; phosphate carrier), member 3","MitoCarta3.0","Metabolism > Metals and cofactors > Copper metabolism | Signaling > Calcium homeostasis > Calcium cycle | Small molecule transport > SLC25A family","MIM",2,"Mito_carr",null,null,null,null,"FirstInEnsemblFamily","Micochondrial phosphate carrier deficiency","SLC25A3",0,0,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,136,1,1,"Metabolism > Metals and cofactors > Copper metabolism | Signaling > Calcium homeostasis > Calcium cycle | Small molecule transport > SLC25A family","Metabolism "," Metals and cofactors "," Copper metabolism "," Signaling "," Calcium homeostasis "," Calcium cycle "," Small molecule transport "," SLC25A family"]
["hsa:253512",253512,"SLC25A30","KMCP1",13,"solute carrier family 25, member 30","mitochondrion | integral to membrane | mitochondrial inner membrane | membrane","binding | molecular_function","transport | mitochondrial transport | biological_process","SLC25A30","solute carrier family 25, member 30","MitoCarta3.0","Metabolism > Sulfur metabolism | Small molecule transport > SLC25A family","MIM",0,"Mito_carr",null,"KIDNEY MITOCHONDRIAL CARRIER 1 SOLUTE CARRIER FAMILY 25 MEMBER 30","ENSFM00500000270162",2,null,null,"SLC25A30",0,0,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,136,1,1,"Metabolism > Sulfur metabolism | Small molecule transport > SLC25A family","Metabolism "," Sulfur metabolism ",null," Small molecule transport "," SLC25A family"]
["hsa:83447",83447,"SLC25A31","AAC4|ANT4|DKFZp434N1235|SFEC35kDa",4,"solute carrier family 25 (mitochondrial carrier; adenine nucleotide translocator), member 31","mitochondrion | integral to membrane | flagellum | mitochondrial inner membrane | cilium | membrane","binding | transporter activity","transmembrane transport","SLC25A31","solute carrier family 25 (mitochondrial carrier; adenine nucleotide translocator), member 31","MitoCarta3.0","Metabolism > Nucleotide metabolism > Nucleotide import | Signaling > Calcium homeostasis > Mitochondrial permeability transition pore | Small molecule transport > SLC25A family","MIM",2,"Mito_carr",null,"ADP/ATP TRANSLOCASE ADP ATP CARRIER ADENINE NUCLEOTIDE TRANSLOCATOR ANT SOLUTE CARRIER FAMILY 25 MEMBER","ENSFM00250000000816",4,null,null,"SLC25A31",0,0,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,136,1,0,"Metabolism > Nucleotide metabolism > Nucleotide import | Signaling > Calcium homeostasis > Mitochondrial permeability transition pore | Small molecule transport > SLC25A family","Metabolism "," Nucleotide metabolism "," Nucleotide import "," Signaling "," Calcium homeostasis "," Mitochondrial permeability transition pore "," Small molecule transport "," SLC25A family"]
["hsa:81034",81034,"SLC25A32","FLJ23872|MFT|MFTC",8,"solute carrier family 25, member 32","mitochondrion | integral to membrane | mitochondrial inner membrane | membrane","folic acid transporter activity | binding","transport | folic acid metabolic process | water-soluble vitamin metabolic process | mitochondrial transport | vitamin metabolic process | folic acid transport","SLC25A32","solute carrier family 25, member 32","MitoCarta3.0","Metabolism > Vitamin metabolism > Vitamin B2 metabolism | Metabolism > Vitamin metabolism > Folate and 1-C metabolism | Small molecule transport > SLC25A family","MIM",0,"Mito_carr",null,null,null,null,"FirstInEnsemblFamily",null,"SLC25A32",0,0,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,136,1,1,"Metabolism > Vitamin metabolism > Vitamin B2 metabolism | Metabolism > Vitamin metabolism > Folate and 1-C metabolism | Small molecule transport > SLC25A family","Metabolism "," Vitamin metabolism "," Vitamin B2 metabolism "," Metabolism "," Vitamin metabolism "," Folate and 1-C metabolism "," Small molecule transport "," SLC25A family"]
["hsa:84275",84275,"SLC25A33","BMSC-MCP|MGC4399",1,"solute carrier family 25, member 33","mitochondrion | integral to membrane | mitochondrial inner membrane | membrane",null,"transport","SLC25A33","solute carrier family 25, member 33","MitoCarta3.0","Metabolism > Nucleotide metabolism > Nucleotide import | Small molecule transport > SLC25A family","MIM",1,"Mito_carr",null,"SOLUTE CARRIER FAMILY 25 MEMBER","ENSFM00260000050581",2,null,null,"SLC25A33",0,0,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,136,1,0,"Metabolism > Nucleotide metabolism > Nucleotide import | Small molecule transport > SLC25A family","Metabolism "," Nucleotide metabolism "," Nucleotide import "," Small molecule transport "," SLC25A family"]
["hsa:284723",284723,"SLC25A34","DKFZp781A10161|RP11-169K16.2",1,"solute carrier family 25, member 34","mitochondrion | integral to membrane | mitochondrial inner membrane | membrane",null,"transport","SLC25A34","solute carrier family 25, member 34","MitoCarta3.0","Small molecule transport > SLC25A family","MIM",2,"Mito_carr",null,"SOLUTE CARRIER FAMILY 25 MEMBER","ENSFM00400000131818",2,null,null,"SLC25A34",0,0,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,136,1,0,"Small molecule transport > SLC25A family","Small molecule transport "," SLC25A family"]
["hsa:55186",55186,"SLC25A36","DKFZp564C053|FLJ10618",3,"solute carrier family 25, member 36","mitochondrion | integral to membrane | mitochondrial inner membrane | membrane","binding","transmembrane transport | response to estradiol stimulus","SLC25A36","solute carrier family 25, member 36","MitoCarta3.0","Metabolism > Nucleotide metabolism > Nucleotide import | Small molecule transport > SLC25A family","MIM",2,"Mito_carr",null,"SOLUTE CARRIER FAMILY 25 MEMBER","ENSFM00260000050581",2,"FirstInEnsemblFamily",null,"SLC25A36",0,0,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,136,1,1,"Metabolism > Nucleotide metabolism > Nucleotide import | Small molecule transport > SLC25A family","Metabolism "," Nucleotide metabolism "," Nucleotide import "," Small molecule transport "," SLC25A family"]
["hsa:51312",51312,"SLC25A37","MFRN|MFRN1|MSC|MSCP|PRO1278|PRO1584|PRO2217",8,"solute carrier family 25, member 37","mitochondrion | integral to membrane | mitochondrial inner membrane | membrane",null,"ion transport | iron ion homeostasis","SLC25A37","solute carrier family 25, member 37","MitoCarta3.0","Metabolism > Metals and cofactors > Iron homeostasis | Small molecule transport > SLC25A family","MIM",0,"Mito_carr",null,"MITOFERRIN MITOCHONDRIAL IRON TRANSPORTER SOLUTE CARRIER FAMILY 25 MEMBER","ENSFM00250000002181",2,null,null,"SLC25A37",0,0,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,136,1,1,"Metabolism > Metals and cofactors > Iron homeostasis | Small molecule transport > SLC25A family","Metabolism "," Metals and cofactors "," Iron homeostasis "," Small molecule transport "," SLC25A family"]
["hsa:54977",54977,"SLC25A38","FLJ20551|FLJ22703",3,"solute carrier family 25, member 38","mitochondrion | integral to membrane | mitochondrial inner membrane | membrane",null,"transport | heme biosynthetic process | erythrocyte differentiation","SLC25A38","solute carrier family 25, member 38","MitoCarta3.0","Metabolism > Amino acid metabolism > Glycine metabolism | Metabolism > Metals and cofactors > Heme synthesis and processing | Metabolism > Vitamin metabolism > Folate and 1-C metabolism | Small molecule transport > SLC25A family","MIM",0,"Mito_carr",null,null,null,null,"FirstInEnsemblFamily","Anemia, sideroblastic, pyridoxine-refractory, autosomal recessive","SLC25A38",0,0,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,136,1,1,"Metabolism > Amino acid metabolism > Glycine metabolism | Metabolism > Metals and cofactors > Heme synthesis and processing | Metabolism > Vitamin metabolism > Folate and 1-C metabolism | Small molecule transport > SLC25A family","Metabolism "," Amino acid metabolism "," Glycine metabolism "," Metabolism "," Metals and cofactors "," Heme synthesis and processing "," Metabolism "," Vitamin metabolism "," Folate and 1-C metabolism "," Small molecule transport "," SLC25A family"]
["hsa:51629",51629,"SLC25A39","CGI69|FLJ22407",17,"solute carrier family 25, member 39","mitochondrion | integral to membrane | mitochondrial inner membrane | membrane",null,"transport | heme biosynthetic process","SLC25A39","solute carrier family 25, member 39","MitoCarta3.0","Small molecule transport > SLC25A family","MIM",2,"Mito_carr",null,"SOLUTE CARRIER FAMILY 25 MEMBER","ENSFM00250000002826",2,null,null,"SLC25A39",0,0,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,136,1,1,"Small molecule transport > SLC25A family","Small molecule transport "," SLC25A family"]
["hsa:291",291,"SLC25A4","AAC1|ANT|ANT1|PEO2|PEO3|T1",4,"solute carrier family 25 (mitochondrial carrier; adenine nucleotide translocator), member 4","mitochondrial outer membrane | mitochondrion | mitochondrial inner membrane | integral to plasma membrane | membrane","protein binding | transporter activity | enzyme binding | adenine transmembrane transporter activity | ATP:ADP antiporter activity","glutamate uptake involved in synaptic transmission | transport | energy reserve metabolic process | mitochondrial genome maintenance | transmembrane transport | adenine transport | regulation of insulin secretion | generation of precursor metabolites and energy | negative regulation of necrotic cell death | ATP transport | ADP transport | viral reproduction | interspecies interaction between organisms","SLC25A4","solute carrier family 25 (mitochondrial carrier; adenine nucleotide translocator), member 4","MitoCarta3.0","Metabolism > Nucleotide metabolism > Nucleotide import | Signaling > Calcium homeostasis > Mitochondrial permeability transition pore | Small molecule transport > SLC25A family","MIM",3,"Mito_carr",null,"ADP/ATP TRANSLOCASE ADP ATP CARRIER ADENINE NUCLEOTIDE TRANSLOCATOR ANT SOLUTE CARRIER FAMILY 25 MEMBER","ENSFM00250000000816",4,null,"Progressive external ophthalmoplegia with mitochondrial DNA deletions 3","SLC25A4",0,0,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,136,1,1,"Metabolism > Nucleotide metabolism > Nucleotide import | Signaling > Calcium homeostasis > Mitochondrial permeability transition pore | Small molecule transport > SLC25A family","Metabolism "," Nucleotide metabolism "," Nucleotide import "," Signaling "," Calcium homeostasis "," Mitochondrial permeability transition pore "," Small molecule transport "," SLC25A family"]
["hsa:55972",55972,"SLC25A40","MCFP",7,"solute carrier family 25, member 40","mitochondrion | integral to membrane | mitochondrial inner membrane | membrane","binding","transmembrane transport","SLC25A40","solute carrier family 25, member 40","MitoCarta3.0","Small molecule transport > SLC25A family","MIM",0,"Mito_carr",null,"SOLUTE CARRIER FAMILY 25 MEMBER","ENSFM00250000002826",2,"FirstInEnsemblFamily",null,"SLC25A40",0,0,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,136,1,0,"Small molecule transport > SLC25A family","Small molecule transport "," SLC25A family"]
["hsa:284427",284427,"SLC25A41","FLJ40442|MGC34725",19,"solute carrier family 25, member 41","mitochondrion | integral to membrane | mitochondrial inner membrane | membrane","binding","transmembrane transport",null,null,"MitoCarta3.0","Metabolism > Nucleotide metabolism > Nucleotide import | Small molecule transport > SLC25A family","MIM",null,"Mito_carr",null,"CALCIUM BINDING MITOCHONDRIAL CARRIER SCAMC SMALL CALCIUM BINDING MITOCHONDRIAL CARRIER SOLUTE CARRIER FAMILY 25 MEMBER","ENSFM00500000269757",4,null,null,"SLC25A41",0,0,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,136,1,0,"Metabolism > Nucleotide metabolism > Nucleotide import | Small molecule transport > SLC25A family","Metabolism "," Nucleotide metabolism "," Nucleotide import "," Small molecule transport "," SLC25A family"]
["hsa:284439",284439,"SLC25A42","MGC26694",19,"solute carrier family 25, member 42","mitochondrion | integral to membrane | mitochondrial inner membrane | membrane","binding","transmembrane transport","SLC25A42","solute carrier family 25, member 42","MitoCarta3.0","Metabolism > Metals and cofactors > Coenzyme A metabolism | Small molecule transport > SLC25A family","MIM",0,"Mito_carr",null,null,null,null,"FirstInEnsemblFamily",null,"SLC25A42",0,0,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,136,1,0,"Metabolism > Metals and cofactors > Coenzyme A metabolism | Small molecule transport > SLC25A family","Metabolism "," Metals and cofactors "," Coenzyme A metabolism "," Small molecule transport "," SLC25A family"]
["hsa:203427",203427,"SLC25A43","-","X","solute carrier family 25, member 43","mitochondrion | integral to membrane | mitochondrial inner membrane | membrane","binding","transmembrane transport","SLC25A43","solute carrier family 25, member 43","MitoCarta3.0","Small molecule transport > SLC25A family","MIM",3,"Mito_carr",null,null,null,null,"FirstInEnsemblFamily",null,"SLC25A43",0,0,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,136,1,0,"Small molecule transport > SLC25A family","Small molecule transport "," SLC25A family"]
["hsa:9673",9673,"SLC25A44","FLJ90431|KIAA0446|RP11-54H19.3",1,"solute carrier family 25, member 44","mitochondrion | integral to membrane | mitochondrial inner membrane | membrane","binding","transmembrane transport","SLC25A44","solute carrier family 25, member 44","MitoCarta3.0","Metabolism > Amino acid metabolism > Branched-chain amino acid metabolism | Small molecule transport > SLC25A family","MIM",1,"Mito_carr",null,null,null,null,"FirstInEnsemblFamily",null,"SLC25A44",0,0,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,136,1,0,"Metabolism > Amino acid metabolism > Branched-chain amino acid metabolism | Small molecule transport > SLC25A family","Metabolism "," Amino acid metabolism "," Branched-chain amino acid metabolism "," Small molecule transport "," SLC25A family"]
["hsa:283130",283130,"SLC25A45","-",11,"solute carrier family 25, member 45","mitochondrion | integral to membrane | mitochondrial inner membrane | membrane","binding","transmembrane transport",null,null,"MitoCarta3.0","Small molecule transport > SLC25A family","MIM",null,"Mito_carr",null,"SOLUTE CARRIER FAMILY 25 MEMBER 47 HEPATOCELLULAR CARCINOMA DOWN REGULATED MITOCHONDRIAL CARRIER","ENSFM00500000270645",3,null,null,"SLC25A45",0,0,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,136,1,0,"Small molecule transport > SLC25A family","Small molecule transport "," SLC25A family"]
["hsa:283600",283600,"SLC25A47","C14orf68|HDMCP",14,"solute carrier family 25, member 47","mitochondrion | integral to membrane | mitochondrial inner membrane | membrane","binding","transmembrane transport","C14orf68","chromosome 14 open reading frame 68","MitoCarta3.0","Small molecule transport > SLC25A family","MIM",0,"Mito_carr",null,"SOLUTE CARRIER FAMILY 25 MEMBER 47 HEPATOCELLULAR CARCINOMA DOWN REGULATED MITOCHONDRIAL CARRIER","ENSFM00500000270645",3,null,null,"SLC25A47",0,0,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,136,1,0,"Small molecule transport > SLC25A family","Small molecule transport "," SLC25A family"]
["hsa:153328",153328,"SLC25A48","HDMCP",5,"solute carrier family 25, member 48","mitochondrion | integral to membrane | mitochondrial inner membrane | membrane","binding","transmembrane transport","LOC153328","similar to CG4995 gene product","MitoCarta3.0","Small molecule transport > SLC25A family","MIM",0,"Mito_carr",null,"SOLUTE CARRIER FAMILY 25 MEMBER 47 HEPATOCELLULAR CARCINOMA DOWN REGULATED MITOCHONDRIAL CARRIER","ENSFM00500000270645",3,"FirstInEnsemblFamily",null,"SLC25A48",0,0,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,136,1,0,"Small molecule transport > SLC25A family","Small molecule transport "," SLC25A family"]
["hsa:292",292,"SLC25A5","2F1|AAC2|ANT2|T2|T3","X","solute carrier family 25 (mitochondrial carrier; adenine nucleotide translocator), member 5","mitochondrial nucleoid | mitochondrion | mitochondrial inner membrane | MMXD complex | integral to plasma membrane | membrane","protein binding | transporter activity | adenine transmembrane transporter activity","transport | chromosome segregation | energy reserve metabolic process | transmembrane transport | adenine transport | regulation of insulin secretion | viral reproduction | interspecies interaction between organisms","SLC25A5","solute carrier family 25 (mitochondrial carrier; adenine nucleotide translocator), member 5","MitoCarta3.0","Metabolism > Nucleotide metabolism > Nucleotide import | Signaling > Calcium homeostasis > Mitochondrial permeability transition pore | Small molecule transport > SLC25A family","MIM",2,"Mito_carr",null,"ADP/ATP TRANSLOCASE ADP ATP CARRIER ADENINE NUCLEOTIDE TRANSLOCATOR ANT SOLUTE CARRIER FAMILY 25 MEMBER","ENSFM00250000000816",4,null,null,"SLC25A5",0,0,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,136,1,0,"Metabolism > Nucleotide metabolism > Nucleotide import | Signaling > Calcium homeostasis > Mitochondrial permeability transition pore | Small molecule transport > SLC25A family","Metabolism "," Nucleotide metabolism "," Nucleotide import "," Signaling "," Calcium homeostasis "," Mitochondrial permeability transition pore "," Small molecule transport "," SLC25A family"]
["hsa:293",293,"SLC25A6","AAC3|ANT3|ANT3Y|MGC17525","X|Y","solute carrier family 25 (mitochondrial carrier; adenine nucleotide translocator), member 6","mitochondrion | integral to membrane | mitochondrial inner membrane | membrane | mitochondrial inner membrane presequence translocase complex","binding | transporter activity | ATP:ADP antiporter activity","energy reserve metabolic process | apoptosis | transmembrane transport | regulation of insulin secretion | viral reproduction | interspecies interaction between organisms | viral infectious cycle | active induction of host immune response by virus | virus-host interaction","SLC25A6","solute carrier family 25 (mitochondrial carrier; adenine nucleotide translocator), member 6","MitoCarta3.0","Metabolism > Nucleotide metabolism > Nucleotide import | Signaling > Calcium homeostasis > Mitochondrial permeability transition pore | Small molecule transport > SLC25A family","MIM",2,"Mito_carr",null,"ADP/ATP TRANSLOCASE ADP ATP CARRIER ADENINE NUCLEOTIDE TRANSLOCATOR ANT SOLUTE CARRIER FAMILY 25 MEMBER","ENSFM00250000000816",4,"FirstInEnsemblFamily",null,"SLC25A6",0,0,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,136,1,0,"Metabolism > Nucleotide metabolism > Nucleotide import | Signaling > Calcium homeostasis > Mitochondrial permeability transition pore | Small molecule transport > SLC25A family","Metabolism "," Nucleotide metabolism "," Nucleotide import "," Signaling "," Calcium homeostasis "," Mitochondrial permeability transition pore "," Small molecule transport "," SLC25A family"]
["hsa:8802",8802,"SUCLG1","FLJ21114|FLJ43513|GALPHA|MTDPS9|SUCLA1",2,"succinate-CoA ligase, alpha subunit","mitochondrion | mitochondrial inner membrane | succinate-CoA ligase complex (GDP-forming) | mitochondrial matrix","succinate-CoA ligase (ADP-forming) activity | protein heterodimerization activity | nucleotide binding | ligase activity | GDP binding | succinate-CoA ligase (GDP-forming) activity | GTP binding | ATP citrate synthase activity","tricarboxylic acid cycle | succinate metabolic process | succinyl-CoA metabolic process","SUCLG1","succinate-CoA ligase, GDP-forming, alpha subunit","MitoCarta3.0","Metabolism > Carbohydrate metabolism > TCA cycle | Metabolism > Carbohydrate metabolism > Itaconate metabolism | Metabolism > Nucleotide metabolism > Nucleotide synthesis and processing","Matrix",0,"Ligase_CoA | CoA_binding","Propanoate metabolism | Citrate cycle (TCA cycle)",null,null,null,"FirstInEnsemblFamily","Mitochondrial DNA depletion syndrome 9 (encephalomyopathic type with methylmalonic aciduria)","SUCLG1",1,0,1,1,1,1,1,1,1,1,1,1,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,136,1,1,"Metabolism > Carbohydrate metabolism > TCA cycle | Metabolism > Carbohydrate metabolism > Itaconate metabolism | Metabolism > Nucleotide metabolism > Nucleotide synthesis and processing","Metabolism "," Carbohydrate metabolism "," TCA cycle "," Metabolism "," Carbohydrate metabolism "," Itaconate metabolism "," Metabolism "," Nucleotide metabolism "," Nucleotide synthesis and processing"]
["hsa:7350",7350,"UCP1","SLC25A7|UCP",4,"uncoupling protein 1 (mitochondrial, proton carrier)","mitochondrial envelope | mitochondrion | integral to membrane | mitochondrial inner membrane | membrane","anion transmembrane transporter activity | oxidative phosphorylation uncoupler activity | binding","transport | cellular lipid metabolic process | respiratory electron transport chain | cellular response to hormone stimulus | proton transport | brown fat cell differentiation | response to steroid hormone stimulus | mitochondrial transport | anion transport","UCP1","uncoupling protein 1 (mitochondrial, proton carrier)","MitoCarta3.0","Small molecule transport > SLC25A family","MIM",0,"Mito_carr",null,"MITOCHONDRIAL BROWN FAT UNCOUPLING 1 UCP 1 SOLUTE CARRIER FAMILY 25 MEMBER 7 THERMOGENIN","ENSFM00500000269770",3,null,null,"UCP1",0,0,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,136,1,0,"Small molecule transport > SLC25A family","Small molecule transport "," SLC25A family"]
["hsa:7351",7351,"UCP2","BMIQ4|SLC25A8|UCPH",11,"uncoupling protein 2 (mitochondrial, proton carrier)","mitochondrion | integral to membrane | mitochondrial inner membrane | membrane","anion transmembrane transporter activity | binding","transport | response to superoxide | response to hypoxia | respiratory electron transport chain | response to glucose stimulus | cellular response to hormone stimulus | response to fatty acid | cellular response to insulin stimulus | proton transport | mitochondrial transport | anion transport","UCP2","uncoupling protein 2 (mitochondrial, proton carrier)","MitoCarta3.0","Small molecule transport > SLC25A family","MIM",0,"Mito_carr",null,"MITOCHONDRIAL BROWN FAT UNCOUPLING 1 UCP 1 SOLUTE CARRIER FAMILY 25 MEMBER 7 THERMOGENIN","ENSFM00500000269770",3,"FirstInEnsemblFamily",null,"UCP2",0,0,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,136,1,0,"Small molecule transport > SLC25A family","Small molecule transport "," SLC25A family"]
["hsa:7352",7352,"UCP3","SLC25A9",11,"uncoupling protein 3 (mitochondrial, proton carrier)","mitochondrion | integral to membrane | mitochondrial inner membrane | membrane","oxidative phosphorylation uncoupler activity | binding | transporter activity","respiratory gaseous exchange | response to cold | aging | response to superoxide | response to hypoxia | respiratory electron transport chain | lipid metabolic process | cellular response to hormone stimulus | response to glucocorticoid stimulus | response to insulin stimulus | proton transport | response to activity | fatty acid metabolic process | response to steroid hormone stimulus | mitochondrial transport | response to nutrient","UCP3","uncoupling protein 3 (mitochondrial, proton carrier)","MitoCarta3.0","Small molecule transport > SLC25A family","MIM",0,"Mito_carr",null,"MITOCHONDRIAL BROWN FAT UNCOUPLING 1 UCP 1 SOLUTE CARRIER FAMILY 25 MEMBER 7 THERMOGENIN","ENSFM00500000269770",3,null,null,"UCP3",0,0,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,136,1,0,"Small molecule transport > SLC25A family","Small molecule transport "," SLC25A family"]
["hsa:60314",60314,"C12orf10","MST024|MSTP024|MYG|MYG1",12,"chromosome 12 open reading frame 10","mitochondrion | nucleus | cellular_component","molecular_function","pigmentation","C12orf10","chromosome 12 open reading frame 10","MitoCarta3.0","Mitochondrial central dogma > mtRNA metabolism > mt-rRNA modifications | Mitochondrial central dogma > mtRNA metabolism > mt-mRNA modifications","Matrix",0,"UPF0160",null,null,null,null,"FirstInEnsemblFamily",null,"C12orf10",1,1,1,1,1,1,1,1,1,1,1,1,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,0,1,1,1,1,1,135,1,1,"Mitochondrial central dogma > mtRNA metabolism > mt-rRNA modifications | Mitochondrial central dogma > mtRNA metabolism > mt-mRNA modifications","Mitochondrial central dogma "," mtRNA metabolism "," mt-rRNA modifications "," Mitochondrial central dogma "," mtRNA metabolism "," mt-mRNA modifications"]
["hsa:25819",25819,"CCRN4L","CCR4L|MGC142054|MGC142060|MGC4120817|MGC78549|NOC",4,"CCR4 carbon catabolite repression 4-like (S. cerevisiae)","nucleoplasm | nucleus","sequence-specific DNA binding transcription factor activity","regulation of transcription, DNA-dependent | rhythmic process | transcription from RNA polymerase II promoter","CCRN4L","CCR4 carbon catabolite repression 4-like (S. cerevisiae)","MitoCarta3.0","Metabolism > Metals and cofactors > NAD biosynthesis and metabolism","Matrix",0,"Exo_endo_phos",null,null,null,null,"FirstInEnsemblFamily",null,"CCRN4L",1,1,0,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,135,1,0,"Metabolism > Metals and cofactors > NAD biosynthesis and metabolism","Metabolism "," Metals and cofactors "," NAD biosynthesis and metabolism"]
["hsa:56994",56994,"CHPT1","CPT|CPT1",12,"choline phosphotransferase 1","integral to membrane | Golgi apparatus | microsome | membrane | Golgi membrane","phosphotransferase activity, for other substituted phosphate groups | diacylglycerol binding | diacylglycerol cholinephosphotransferase activity | transferase activity | metal ion binding","platelet activating factor biosynthetic process | regulation of cell growth | phosphatidylcholine biosynthetic process | phospholipid biosynthetic process | CDP-choline pathway | lipid metabolic process","CHPT1","choline phosphotransferase 1","MitoCarta3.0","Metabolism > Lipid metabolism > Phospholipid metabolism","MIM",8,"CDP-OH_P_transf","Glycerophospholipid metabolism | Ether lipid metabolism","CHOLINEPHOSPHOTRANSFERASE 1 EC_2.7.8.2 DIACYLGLYCEROL CHOLINEPHOSPHOTRANSFERASE 1","ENSFM00500000270126",2,null,null,"CHPT1",1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,135,1,0,"Metabolism > Lipid metabolism > Phospholipid metabolism","Metabolism "," Lipid metabolism "," Phospholipid metabolism"]
["hsa:51805",51805,"COQ3","DHHBMT|DHHBMTASE|UG0215E05|bA9819.1",6,"coenzyme Q3 homolog, methyltransferase (S. cerevisiae)","mitochondrion | mitochondrial matrix","3-demethylubiquinone-9 3-O-methyltransferase activity | hexaprenyldihydroxybenzoate methyltransferase activity | 2-polyprenyl-6-methoxy-1,4-benzoquinone methyltransferase activity | O-methyltransferase activity | transferase activity","ubiquinone biosynthetic process | glycerol metabolic process | metabolic process","COQ3","coenzyme Q3 homolog, methyltransferase (S. cerevisiae)","MitoCarta3.0","Metabolism > Metals and cofactors > Coenzyme Q metabolism","MIM",0,"Methyltransf_11 | Methyltransf_12","Ubiquinone and other terpenoid-quinone biosynthesis",null,null,null,"FirstInEnsemblFamily",null,"COQ3",1,0,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,135,1,1,"Metabolism > Metals and cofactors > Coenzyme Q metabolism","Metabolism "," Metals and cofactors "," Coenzyme Q metabolism"]
["hsa:3094",3094,"HINT1","FLJ30414|FLJ32340|HINT|PKCI-1|PRKCNH1",5,"histidine triad nucleotide binding protein 1","cytoskeleton | nucleus | cytoplasm","hydrolase activity | protein kinase C binding","signal transduction","HINT1","histidine triad nucleotide binding protein 1","MitoCarta3.0","Metabolism > Nucleotide metabolism > Nucleotide synthesis and processing","unknown",0,"HIT",null,null,null,null,"FirstInEnsemblFamily","Neuromyotonia and axonal neuropathy, autosomal recessive","HINT1",1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,0,0,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,135,1,1,"Metabolism > Nucleotide metabolism > Nucleotide synthesis and processing","Metabolism "," Nucleotide metabolism "," Nucleotide synthesis and processing"]
["hsa:7923",7923,"HSD17B8","D6S2245E|FABG|FABGL|H2-KE6|HKE6|KE6|RING2|SDR30C1|dJ1033B10.9",6,"hydroxysteroid (17-beta) dehydrogenase 8","mitochondrial envelope | mitochondrion | plasma membrane | mitochondrial matrix | membrane fraction","testosterone 17-beta-dehydrogenase (NAD+) activity | protein binding | oxidoreductase activity | estradiol 17-beta-dehydrogenase activity | 3-hydroxyacyl-CoA dehydrogenase activity | nucleotide binding","estrogen biosynthetic process | fatty acid biosynthetic process | androgen metabolic process","HSD17B8","hydroxysteroid (17-beta) dehydrogenase 8","MitoCarta3.0","Metabolism > Lipid metabolism > Type II fatty acid synthesis | Metabolism > Lipid metabolism > Cholesterol, bile acid, steroid synthesis","Matrix",0,"KR | adh_short","Steroid hormone biosynthesis",null,null,null,"FirstInEnsemblFamily",null,"HSD17B8",1,0,1,1,0,1,1,1,1,1,1,1,1,1,1,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,135,1,0,"Metabolism > Lipid metabolism > Type II fatty acid synthesis | Metabolism > Lipid metabolism > Cholesterol, bile acid, steroid synthesis","Metabolism "," Lipid metabolism "," Type II fatty acid synthesis "," Metabolism "," Lipid metabolism "," Cholesterol, bile acid, steroid synthesis"]
["hsa:3945",3945,"LDHB","LDH-H|LDHBD|TRG-5",12,"lactate dehydrogenase B","cytosol | mitochondrion | soluble fraction | cytoplasm","oxidoreductase activity | NAD binding | identical protein binding | L-lactate dehydrogenase activity","cellular carbohydrate metabolic process | lactate metabolic process | pyruvate metabolic process | glycolysis | NAD metabolic process","LDHB","lactate dehydrogenase B","MitoCarta3.0","Metabolism > Amino acid metabolism > Glyoxylate metabolism","unknown",0,"Ldh_1_N | Ldh_1_C",null,"L LACTATE DEHYDROGENASE CHAIN LDH EC_1.1.1.27","ENSFM00250000000475",5,"FirstInEnsemblFamily","Lactate dehydrogenase-B deficiency","LDHB",1,0,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,135,1,0,"Metabolism > Amino acid metabolism > Glyoxylate metabolism","Metabolism "," Amino acid metabolism "," Glyoxylate metabolism"]
["hsa:55621",55621,"TRMT1","FLJ20244|TRM1",19,"TRM1 tRNA methyltransferase 1 homolog (S. cerevisiae)",null,"methyltransferase activity | RNA binding | transferase activity | zinc ion binding | tRNA (guanine-N2-)-methyltransferase activity | metal ion binding","tRNA processing","TRMT1","TRM1 tRNA methyltransferase 1 homolog (S. cerevisiae)","MitoCarta3.0","Mitochondrial central dogma > mtRNA metabolism > mt-tRNA modifications","Matrix",0,"TRM | zf-CCCH",null,null,null,null,"FirstInEnsemblFamily",null,"TRMT1",1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,0,0,1,1,1,1,1,1,1,135,1,1,"Mitochondrial central dogma > mtRNA metabolism > mt-tRNA modifications","Mitochondrial central dogma "," mtRNA metabolism "," mt-tRNA modifications"]
["hsa:7385",7385,"UQCRC2","QCR2|UQCR2",16,"ubiquinol-cytochrome c reductase core protein II","mitochondrion | respiratory chain | mitochondrial inner membrane | membrane | mitochondrial respiratory chain complex III","protein complex binding | metalloendopeptidase activity | zinc ion binding | catalytic activity | metal ion binding","transport | proteolysis | oxidative phosphorylation | respiratory electron transport chain | aerobic respiration","UQCRC2","ubiquinol-cytochrome c reductase core protein II","MitoCarta3.0","Protein import, sorting and homeostasis > Protein import and sorting > Preprotein cleavage | OXPHOS > Complex III > CIII subunits | OXPHOS > OXPHOS subunits","MIM",0,"Peptidase_M16 | Peptidase_M16_C","Oxidative phosphorylation",null,null,null,"FirstInEnsemblFamily",null,"UQCRC2",1,0,0,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,135,1,0,"Protein import, sorting and homeostasis > Protein import and sorting > Preprotein cleavage | OXPHOS > Complex III > CIII subunits | OXPHOS > OXPHOS subunits","Protein import, sorting and homeostasis "," Protein import and sorting "," Preprotein cleavage "," OXPHOS "," Complex III "," CIII subunits "," OXPHOS "," OXPHOS subunits"]
["hsa:79693",79693,"YRDC","DRIP3|FLJ23476|FLJ26165|IRIP|RP11-109P14.4|SUA5",1,"yrdC domain containing (E. coli)","mitochondrion | membrane | membrane fraction",null,"negative regulation of transport","YRDC","yrdC domain containing (E. coli)","MitoCarta3.0","Mitochondrial central dogma > mtRNA metabolism > mt-tRNA modifications","Matrix",0,"Sua5_yciO_yrdC",null,null,null,null,"FirstInEnsemblFamily",null,"YRDC",1,1,1,1,1,1,1,1,1,1,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,135,1,1,"Mitochondrial central dogma > mtRNA metabolism > mt-tRNA modifications","Mitochondrial central dogma "," mtRNA metabolism "," mt-tRNA modifications"]
["hsa:57143",57143,"ADCK1","FLJ39600",14,"aarF domain containing kinase 1","mitochondrion | extracellular region","protein serine/threonine kinase activity | kinase activity | nucleotide binding | ATP binding",null,"ADCK1","aarF domain containing kinase 1","MitoCarta3.0",0,"unknown",0,"ABC1",null,"UNCHARACTERIZED AARF DOMAIN CONTAINING KINASE 1 PRECURSOR EC_2.7.11.-","ENSFM00250000002555",2,"FirstInEnsemblFamily",null,"ADCK1",1,0,0,0,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,134,1,0,0,0]
["hsa:56997",56997,"ADCK3","ARCA2|CABC1|COQ8|MGC4849|SCAR9",1,"aarF domain containing kinase 3","mitochondrion","protein serine/threonine kinase activity | transferase activity, transferring phosphorus-containing groups | kinase activity | nucleotide binding | ATP binding","cell death","CABC1","chaperone, ABC1 activity of bc1 complex homolog (S. pombe)","MitoCarta3.0","Metabolism > Metals and cofactors > Coenzyme Q metabolism","MIM",0,"ABC1",null,"CHAPERONE ACTIVITY OF BC1 COMPLEX  MITOCHONDRIAL PRECURSOR CHAPERONE ABC1  EC_2.7.11.- AARF DOMAIN CONTAINING KINASE 3","ENSFM00250000002062",2,null,"Coenzyme Q10 deficiency, primary, 4","ADCK3",1,0,0,0,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,134,1,1,"Metabolism > Metals and cofactors > Coenzyme Q metabolism","Metabolism "," Metals and cofactors "," Coenzyme Q metabolism"]
["hsa:79934",79934,"ADCK4","FLJ12229",19,"aarF domain containing kinase 4","mitochondrion | integral to membrane | membrane","protein serine/threonine kinase activity | kinase activity",null,"ADCK4","aarF domain containing kinase 4","MitoCarta3.0","Metabolism > Metals and cofactors > Coenzyme Q metabolism","MIM",0,"ABC1",null,"CHAPERONE ACTIVITY OF BC1 COMPLEX  MITOCHONDRIAL PRECURSOR CHAPERONE ABC1  EC_2.7.11.- AARF DOMAIN CONTAINING KINASE 3","ENSFM00250000002062",2,"FirstInEnsemblFamily",null,"ADCK4",1,0,0,0,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,134,1,0,"Metabolism > Metals and cofactors > Coenzyme Q metabolism","Metabolism "," Metals and cofactors "," Coenzyme Q metabolism"]
["hsa:203054",203054,"ADCK5","FLJ35454|MGC126708",8,"aarF domain containing kinase 5","mitochondrion | integral to membrane | membrane","protein serine/threonine kinase activity | kinase activity",null,"ADCK5","aarF domain containing kinase 5","MitoCarta3.0",0,"unknown",0,"ABC1",null,"UNCHARACTERIZED AARF DOMAIN CONTAINING KINASE 1 PRECURSOR EC_2.7.11.-","ENSFM00250000002555",2,null,null,"ADCK5",1,0,0,0,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,134,1,1,0,0]
["hsa:8574",8574,"AKR7A2","AFAR|AFAR1|AFB1-AR1|AKR7",1,"aldo-keto reductase family 7, member A2 (aflatoxin aldehyde reductase)","Golgi apparatus | cytoplasm","electron carrier activity | oxidoreductase activity | alditol:NADP+ 1-oxidoreductase activity | oxidoreductase activity, acting on the CH-OH group of donors, NAD or NADP as acceptor","cellular aldehyde metabolic process | carbohydrate metabolic process","AKR7A2","aldo-keto reductase family 7, member A2 (aflatoxin aldehyde reductase)","MitoCarta3.0","Metabolism > Amino acid metabolism > GABA metabolism | Metabolism > Detoxification > Xenobiotic metabolism","Matrix",0,"Aldo_ket_red",null,"AFLATOXIN B1 ALDEHYDE REDUCTASE MEMBER 2 EC_1.1.1.- N11 SUCCINIC SEMIALDEHYDE REDUCTASE SSA REDUCTASE","ENSFM00250000005212",2,"FirstInEnsemblFamily",null,"AKR7A2",1,0,1,1,1,1,1,1,1,1,1,1,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,134,1,0,"Metabolism > Amino acid metabolism > GABA metabolism | Metabolism > Detoxification > Xenobiotic metabolism","Metabolism "," Amino acid metabolism "," GABA metabolism "," Metabolism "," Detoxification "," Xenobiotic metabolism"]
["hsa:211",211,"ALAS1","ALAS|ALAS3|ALASH|MIG4",3,"aminolevulinate, delta-, synthase 1","mitochondrion | mitochondrial matrix","5-aminolevulinate synthase activity | pyridoxal phosphate binding | transferase activity, transferring nitrogenous groups | transferase activity, transferring acyl groups","porphyrin-containing compound metabolic process | heme biosynthetic process | tetrapyrrole biosynthetic process | biosynthetic process","ALAS1","aminolevulinate, delta-, synthase 1","MitoCarta3.0","Metabolism > Metals and cofactors > Heme synthesis and processing","Matrix",0,"Aminotran_1_2","Porphyrin and chlorophyll metabolism | \"Glycine, serine and threonine metabolism\"","5 AMINOLEVULINATE SYNTHASE MITOCHONDRIAL PRECURSOR EC_2.3.1.37 5 AMINOLEVULINIC ACID SYNTHASE DELTA ALA SYNTHASE DELTA AMINOLEVULINATE SYNTHASE","ENSFM00250000001038",2,null,null,"ALAS1",1,1,1,1,1,1,1,1,1,1,1,1,0,0,0,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,134,1,0,"Metabolism > Metals and cofactors > Heme synthesis and processing","Metabolism "," Metals and cofactors "," Heme synthesis and processing"]
["hsa:212",212,"ALAS2","ALAS-E|ALASE|ANH1|ASB|FLJ93603|XLDPP|XLEPP|XLSA","X","aminolevulinate, delta-, synthase 2","mitochondrion | mitochondrial inner membrane | mitochondrial matrix","coenzyme binding | protein binding | 5-aminolevulinate synthase activity | pyridoxal phosphate binding | transferase activity, transferring nitrogenous groups | glycine binding | transferase activity, transferring acyl groups","hemoglobin biosynthetic process | porphyrin-containing compound metabolic process | cellular iron ion homeostasis | heme biosynthetic process | tetrapyrrole biosynthetic process | response to hypoxia | oxygen homeostasis | erythrocyte differentiation | biosynthetic process","ALAS2","aminolevulinate, delta-, synthase 2 (sideroblastic/hypochromic anemia)","MitoCarta3.0","Metabolism > Metals and cofactors > Heme synthesis and processing","Matrix",0,"Preseq_ALAS | Aminotran_1_2 | Aminotran_5","Porphyrin and chlorophyll metabolism | \"Glycine, serine and threonine metabolism\"","5 AMINOLEVULINATE SYNTHASE MITOCHONDRIAL PRECURSOR EC_2.3.1.37 5 AMINOLEVULINIC ACID SYNTHASE DELTA ALA SYNTHASE DELTA AMINOLEVULINATE SYNTHASE","ENSFM00250000001038",2,"FirstInEnsemblFamily","Anemia, sideroblastic, X-linked|\"Protoporphyria, erythropoietic, X-linked\"","ALAS2",1,1,1,1,1,1,1,1,1,1,1,1,0,0,0,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,134,1,0,"Metabolism > Metals and cofactors > Heme synthesis and processing","Metabolism "," Metals and cofactors "," Heme synthesis and processing"]
["hsa:90806",90806,"ANGEL2","FLJ12793|KIAA0759L",1,"angel homolog 2 (Drosophila)",null,null,null,"ANGEL2","angel homolog 2 (Drosophila)","MitoCarta3.0","Mitochondrial central dogma > mtRNA metabolism > mt-mRNA modifications","Matrix",0,"Exo_endo_phos",null,"ANGEL HOMOLOG","ENSFM00250000002758",2,null,null,"ANGEL2",0,1,0,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,134,1,0,"Mitochondrial central dogma > mtRNA metabolism > mt-mRNA modifications","Mitochondrial central dogma "," mtRNA metabolism "," mt-mRNA modifications"]
["hsa:80777",80777,"CYB5B","CYB5-M|CYPB5M|DKFZp686M0619|OMB5",16,"cytochrome b5 type B (outer mitochondrial membrane)","mitochondrial outer membrane | mitochondrion | integral to membrane | mitochondrial inner membrane | microsome | membrane","enzyme activator activity | heme binding | metal ion binding","transport | electron transport chain","CYB5B","cytochrome b5 type B (outer mitochondrial membrane)","MitoCarta3.0","Metabolism > Metals and cofactors > Heme-containing proteins | Metabolism > Detoxification > Xenobiotic metabolism | Metabolism > Detoxification > Amidoxime reducing complex | Metabolism > Electron carriers > Cytochromes","MOM",1,"Cyt-b5",null,null,null,null,"FirstInEnsemblFamily",null,"CYB5B",0,1,0,0,1,1,1,1,1,1,1,1,1,1,1,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,134,1,1,"Metabolism > Metals and cofactors > Heme-containing proteins | Metabolism > Detoxification > Xenobiotic metabolism | Metabolism > Detoxification > Amidoxime reducing complex | Metabolism > Electron carriers > Cytochromes","Metabolism "," Metals and cofactors "," Heme-containing proteins "," Metabolism "," Detoxification "," Xenobiotic metabolism "," Metabolism "," Detoxification "," Amidoxime reducing complex "," Metabolism "," Electron carriers "," Cytochromes"]
["hsa:51181",51181,"DCXR","DCR|HCR2|HCRII|KIDCR|P34H|SDR20C1|XR",17,"dicarbonyl/L-xylulose reductase","brush border | membrane | microvillus","oxidoreductase activity, acting on CH-OH group of donors | L-xylulose reductase (NADP+) activity | nucleotide binding","NADP metabolic process | protein homotetramerization | xylulose metabolic process | glucose metabolic process | carbohydrate metabolic process | D-xylose metabolic process","DCXR","dicarbonyl/L-xylulose reductase","MitoCarta3.0","Metabolism > Carbohydrate metabolism","Matrix",0,"KR | adh_short","Pentose and glucuronate interconversions",null,null,null,"FirstInEnsemblFamily",null,"DCXR",1,0,1,1,0,1,1,1,1,1,1,1,1,1,0,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,134,1,1,"Metabolism > Carbohydrate metabolism","Metabolism "," Carbohydrate metabolism"]
["hsa:115817",115817,"DHRS1","DKFZp586I0523|FLJ14250|FLJ25430|MGC20204|SDR19C1",14,"dehydrogenase/reductase (SDR family) member 1","endoplasmic reticulum","oxidoreductase activity | nucleotide binding",null,"DHRS1","dehydrogenase/reductase (SDR family) member 1","MitoCarta3.0","Metabolism > Lipid metabolism > Cholesterol, bile acid, steroid synthesis","MIM",0,"adh_short",null,null,null,null,"FirstInEnsemblFamily",null,"DHRS1",1,0,1,1,0,1,1,1,1,1,1,1,1,1,0,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,134,1,0,"Metabolism > Lipid metabolism > Cholesterol, bile acid, steroid synthesis","Metabolism "," Lipid metabolism "," Cholesterol, bile acid, steroid synthesis"]
["hsa:1841",1841,"DTYMK","CDC8|FLJ44192|MGC198617|PP3731|TMPK|TYMK",2,"deoxythymidylate kinase (thymidylate kinase)","cytosol","nucleoside phosphate kinase activity | thymidylate kinase activity | transferase activity | kinase activity | nucleotide binding | ATP binding","nucleoside monophosphate phosphorylation | cell proliferation | nucleobase-containing small molecule metabolic process | dTDP biosynthetic process | cellular response to growth factor stimulus | cell cycle | nucleobase-containing small molecule interconversion","DTYMK","deoxythymidylate kinase (thymidylate kinase)","MitoCarta3.0","Metabolism > Nucleotide metabolism > Nucleotide synthesis and processing","Matrix",0,"Thymidylate_kin","Pyrimidine metabolism",null,null,null,"FirstInEnsemblFamily",null,"DTYMK",1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,134,1,1,"Metabolism > Nucleotide metabolism > Nucleotide synthesis and processing","Metabolism "," Nucleotide metabolism "," Nucleotide synthesis and processing"]
["hsa:23464",23464,"GCAT","KBL|MGC23053",22,"glycine C-acetyltransferase","mitochondrion","pyridoxal phosphate binding | transferase activity, transferring nitrogenous groups | transferase activity, transferring acyl groups | glycine C-acetyltransferase activity","biosynthetic process | cellular amino acid metabolic process","GCAT","glycine C-acetyltransferase (2-amino-3-ketobutyrate coenzyme A ligase)","MitoCarta3.0","Metabolism > Amino acid metabolism > Glycine metabolism","unknown",0,"Aminotran_1_2 | Aminotran_5","Glycine, serine and threonine metabolism",null,null,null,"FirstInEnsemblFamily",null,"GCAT",1,1,1,1,1,1,1,1,1,1,1,1,0,0,0,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,134,1,1,"Metabolism > Amino acid metabolism > Glycine metabolism","Metabolism "," Amino acid metabolism "," Glycine metabolism"]
["hsa:84681",84681,"HINT2","HIT-17",9,"histidine triad nucleotide binding protein 2","mitochondrion","hydrolase activity","steroid biosynthetic process | apoptosis","HINT2","histidine triad nucleotide binding protein 2","MitoCarta3.0","Metabolism > Lipid metabolism > Cholesterol, bile acid, steroid synthesis","Matrix",0,"HIT",null,null,null,null,"FirstInEnsemblFamily",null,"HINT2",1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,0,0,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,134,1,0,"Metabolism > Lipid metabolism > Cholesterol, bile acid, steroid synthesis","Metabolism "," Lipid metabolism "," Cholesterol, bile acid, steroid synthesis"]
["hsa:81689",81689,"ISCA1","HBLD2|ISA1|MGC4276|RP11-507D14.2|hIscA",9,"iron-sulfur cluster assembly 1 homolog (S. cerevisiae)","mitochondrion","iron-sulfur cluster binding | structural molecule activity | metal ion binding","iron-sulfur cluster assembly","HBLD2","HESB like domain containing 2","MitoCarta3.0","Metabolism > Metals and cofactors > Fe-S cluster biosynthesis | Metabolism > Metals and cofactors > Fe-S-containing proteins","Matrix",0,"Fe-S_biosyn",null,null,null,null,"FirstInEnsemblFamily",null,"ISCA1",1,0,0,0,1,1,1,1,1,1,1,1,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,134,1,1,"Metabolism > Metals and cofactors > Fe-S cluster biosynthesis | Metabolism > Metals and cofactors > Fe-S-containing proteins","Metabolism "," Metals and cofactors "," Fe-S cluster biosynthesis "," Metabolism "," Metals and cofactors "," Fe-S-containing proteins"]
["hsa:122961",122961,"ISCA2","HBLD1|ISA2|c14_5557",14,"iron-sulfur cluster assembly 2 homolog (S. cerevisiae)","mitochondrion","iron-sulfur cluster binding | structural molecule activity | metal ion binding","iron-sulfur cluster assembly","HBLD1","HesB like domain containing 1","MitoCarta3.0","Metabolism > Metals and cofactors > Fe-S cluster biosynthesis | Metabolism > Metals and cofactors > Fe-S-containing proteins","Matrix",0,"Fe-S_biosyn",null,null,null,null,"FirstInEnsemblFamily",null,"ISCA2",1,0,0,0,1,1,1,1,1,1,1,1,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,134,1,1,"Metabolism > Metals and cofactors > Fe-S cluster biosynthesis | Metabolism > Metals and cofactors > Fe-S-containing proteins","Metabolism "," Metals and cofactors "," Fe-S cluster biosynthesis "," Metabolism "," Metals and cofactors "," Fe-S-containing proteins"]
["hsa:5095",5095,"PCCA","-",13,"propionyl CoA carboxylase, alpha polypeptide","mitochondrion | mitochondrial matrix","biotin carboxylase activity | enzyme binding | nucleotide binding | ligase activity | ATP binding | propionyl-CoA carboxylase activity | biotin binding | metal ion binding","fatty acid beta-oxidation | cellular lipid metabolic process","PCCA","propionyl Coenzyme A carboxylase, alpha polypeptide","MitoCarta3.0","Metabolism > Carbohydrate metabolism > Propanoate metabolism | Metabolism > Lipid metabolism > Fatty acid oxidation | Metabolism > Vitamin metabolism > Biotin utilizing proteins","Matrix",0,"Biotin_carb_C | CPSase_L_D2 | RimK | Biotin_lipoyl | CPSase_L_chain","Glyoxylate and dicarboxylate metabolism | Propanoate metabolism | \"Valine, leucine and isoleucine degradation\"","PROPIONYL COA CARBOXYLASE ALPHA CHAIN MITOCHONDRIAL PRECURSOR PCCASE SUBUNIT ALPHA EC_6.4.1.3 PROPANOYL COA:CARBON DIOXIDE LIGASE SUBUNIT ALPHA","ENSFM00570000851021",2,null,"Propionicacidemia","PCCA",1,0,0,0,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,134,1,0,"Metabolism > Carbohydrate metabolism > Propanoate metabolism | Metabolism > Lipid metabolism > Fatty acid oxidation | Metabolism > Vitamin metabolism > Biotin utilizing proteins","Metabolism "," Carbohydrate metabolism "," Propanoate metabolism "," Metabolism "," Lipid metabolism "," Fatty acid oxidation "," Metabolism "," Vitamin metabolism "," Biotin utilizing proteins"]
["hsa:201626",201626,"PDE12","2'-PDE",3,"phosphodiesterase 12","mitochondrion","hydrolase activity",null,"2'-PDE","2'-phosphodiesterase","MitoCarta3.0","Mitochondrial central dogma > mtRNA metabolism > mt-mRNA modifications | Mitochondrial central dogma > mtRNA metabolism > mtRNA stability and decay","Matrix",0,"Exo_endo_phos",null,null,null,null,"FirstInEnsemblFamily",null,"PDE12",0,1,0,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,134,1,0,"Mitochondrial central dogma > mtRNA metabolism > mt-mRNA modifications | Mitochondrial central dogma > mtRNA metabolism > mtRNA stability and decay","Mitochondrial central dogma "," mtRNA metabolism "," mt-mRNA modifications "," Mitochondrial central dogma "," mtRNA metabolism "," mtRNA stability and decay"]
["hsa:23590",23590,"PDSS1","COQ1|DPS|MGC70953|RP13-16H11.3|SPS|TPRT|TPT|TPT 1|hDPS1",10,"prenyl (decaprenyl) diphosphate synthase, subunit 1","mitochondrion","trans-octaprenyltranstransferase activity | trans-hexaprenyltranstransferase activity | protein heterodimerization activity | transferase activity | metal ion binding","ubiquinone biosynthetic process | isoprenoid biosynthetic process | protein heterotetramerization","PDSS1","prenyl (decaprenyl) diphosphate synthase, subunit 1","MitoCarta3.0","Metabolism > Metals and cofactors > Coenzyme Q metabolism","MIM",0,"polyprenyl_synt","Terpenoid backbone biosynthesis",null,null,null,"FirstInEnsemblFamily","Coenzyme Q10 deficiency, primary, 2","PDSS1",1,0,1,1,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,134,1,1,"Metabolism > Metals and cofactors > Coenzyme Q metabolism","Metabolism "," Metals and cofactors "," Coenzyme Q metabolism"]
["hsa:5245",5245,"PHB","PHB1",17,"prohibitin","nucleoplasm | mitochondrion | mitochondrial inner membrane | nucleus | integral to plasma membrane | membrane | cytoplasm","histone deacetylase binding | protein binding | transcription regulatory region DNA binding","cellular response to interleukin-6 | negative regulation of cell proliferation | negative regulation of transcription by competitive promoter binding | negative regulation of androgen receptor signaling pathway | negative regulation of transcription, DNA-dependent | regulation of transcription, DNA-dependent | regulation of apoptosis | histone deacetylation | negative regulation of glucocorticoid receptor signaling pathway | positive regulation of transcription, DNA-dependent | signal transduction | negative regulation of cell growth | negative regulation of transcription from RNA polymerase II promoter | DNA replication | progesterone receptor signaling pathway","PHB","prohibitin","MitoCarta3.0","Protein import, sorting and homeostasis > Protein homeostasis > Chaperones","MIM",0,"Band_7",null,"PROHIBITIN","ENSFM00250000001598",2,"FirstInEnsemblFamily",null,"PHB",1,0,0,0,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,134,1,1,"Protein import, sorting and homeostasis > Protein homeostasis > Chaperones","Protein import, sorting and homeostasis "," Protein homeostasis "," Chaperones"]
["hsa:11331",11331,"PHB2","BAP|BCAP37|Bap37|MGC117268|PNAS-141|REA|p22",12,"prohibitin 2","mitochondrion | mitochondrial inner membrane | nucleus | membrane | cytoplasm","protein binding | estrogen receptor binding | receptor activity","negative regulation of transcription, DNA-dependent","PHB2","prohibitin 2","MitoCarta3.0","Protein import, sorting and homeostasis > Protein homeostasis > Chaperones","MIM",0,"Band_7",null,"PROHIBITIN","ENSFM00250000001598",2,null,null,"PHB2",1,0,0,0,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,134,1,1,"Protein import, sorting and homeostasis > Protein homeostasis > Chaperones","Protein import, sorting and homeostasis "," Protein homeostasis "," Chaperones"]
["hsa:6472",6472,"SHMT2","GLYA|SHMT",12,"serine hydroxymethyltransferase 2 (mitochondrial)","mitochondrial nucleoid | microtubule cytoskeleton | mitochondrion | mitochondrial inner membrane | mitochondrial matrix | mitochondrial intermembrane space","pyridoxal phosphate binding | transferase activity | chromatin binding | amino acid binding | methyltransferase activity | glycine hydroxymethyltransferase activity | L-allo-threonine aldolase activity | identical protein binding","glycine biosynthetic process from serine | L-serine biosynthetic process | protein homotetramerization | one-carbon metabolic process | positive regulation of cell proliferation","SHMT2","serine hydroxymethyltransferase 2 (mitochondrial)","MitoCarta3.0","Metabolism > Amino acid metabolism > Serine metabolism | Metabolism > Amino acid metabolism > Glycine metabolism | Metabolism > Vitamin metabolism > Folate and 1-C metabolism","Matrix",0,"SHMT","Glycine, serine and threonine metabolism | One carbon pool by folate | Cyanoamino acid metabolism","SERINE HYDROXYMETHYLTRANSFERASE SHMT EC_2.1.2.1 GLYCINE HYDROXYMETHYLTRANSFERASE SERINE METHYLASE","ENSFM00250000001563",2,"FirstInEnsemblFamily",null,"SHMT2",1,1,0,0,0,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,134,1,1,"Metabolism > Amino acid metabolism > Serine metabolism | Metabolism > Amino acid metabolism > Glycine metabolism | Metabolism > Vitamin metabolism > Folate and 1-C metabolism","Metabolism "," Amino acid metabolism "," Serine metabolism "," Metabolism "," Amino acid metabolism "," Glycine metabolism "," Metabolism "," Vitamin metabolism "," Folate and 1-C metabolism"]
["hsa:9481",9481,"SLC25A27","FLJ33552|UCP4",6,"solute carrier family 25, member 27","mitochondrion | integral to membrane | mitochondrial inner membrane | membrane",null,"transport | generation of precursor metabolites and energy","SLC25A27","solute carrier family 25, member 27","MitoCarta3.0","Small molecule transport > SLC25A family","MIM",0,"Mito_carr",null,null,null,null,"FirstInEnsemblFamily",null,"SLC25A27",0,0,0,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,134,1,0,"Small molecule transport > SLC25A family","Small molecule transport "," SLC25A family"]
["hsa:399512",399512,"SLC25A35","FLJ40217|MGC120446|MGC120448",17,"solute carrier family 25, member 35","mitochondrion | integral to membrane | mitochondrial inner membrane | membrane",null,"transport","SLC25A35","solute carrier family 25, member 35","MitoCarta3.0","Small molecule transport > SLC25A family","MIM",0,"Mito_carr",null,"SOLUTE CARRIER FAMILY 25 MEMBER","ENSFM00400000131818",2,"FirstInEnsemblFamily",null,"SLC25A35",0,0,0,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,134,1,0,"Small molecule transport > SLC25A family","Small molecule transport "," SLC25A family"]
["hsa:10449",10449,"ACAA2","DSAEC|FLJ35992|FLJ95265",18,"acetyl-CoA acyltransferase 2","mitochondrion | mitochondrial inner membrane","transferase activity, transferring acyl groups other than amino-acyl groups | protein binding | acetyl-CoA C-acyltransferase activity","fatty acid beta-oxidation | acetyl-CoA metabolic process | cholesterol biosynthetic process | fatty acid metabolic process | anti-apoptosis | lipid metabolic process","ACAA2","acetyl-Coenzyme A acyltransferase 2 (mitochondrial 3-oxoacyl-Coenzyme A thiolase)","MitoCarta3.0","Metabolism > Carbohydrate metabolism > Ketone metabolism | Metabolism > Lipid metabolism > Fatty acid oxidation | Metabolism > Amino acid metabolism > Lysine metabolism","Matrix",0,"Thiolase_N | Thiolase_C","Valine, leucine and isoleucine degradation | Fatty acid elongation | Fatty acid metabolism","3 KETOACYL COA THIOLASE MITOCHONDRIAL EC_2.3.1.16 ACETYL COA ACYLTRANSFERASE BETA KETOTHIOLASE MITOCHONDRIAL 3 OXOACYL COA THIOLASE","ENSFM00630001050568",2,null,null,"ACAA2",1,1,0,0,1,1,1,1,1,1,1,1,0,0,1,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,133,1,0,"Metabolism > Carbohydrate metabolism > Ketone metabolism | Metabolism > Lipid metabolism > Fatty acid oxidation | Metabolism > Amino acid metabolism > Lysine metabolism","Metabolism "," Carbohydrate metabolism "," Ketone metabolism "," Metabolism "," Lipid metabolism "," Fatty acid oxidation "," Metabolism "," Amino acid metabolism "," Lysine metabolism"]
["hsa:1373",1373,"CPS1","CPSASE1",2,"carbamoyl-phosphate synthase 1, mitochondrial","mitochondrial nucleoid | mitochondrion | mitochondrial inner membrane | protein complex | mitochondrial matrix | cytoplasm","phospholipid binding | endopeptidase activity | glutamate binding | carbamoyl-phosphate synthase (ammonia) activity | catalytic activity | nucleotide binding | ligase activity | ATP binding | protein binding | protein complex binding | calcium ion binding","nitrogen compound metabolic process | glutamine metabolic process | urea cycle | response to toxin | response to growth hormone stimulus | response to drug | nitric oxide metabolic process | response to amino acid stimulus | triglyceride catabolic process | cellular response to fibroblast growth factor stimulus | midgut development | response to lipopolysaccharide | glycogen catabolic process | positive regulation of vasodilation | response to zinc ion | citrulline biosynthetic process | cellular response to cAMP | response to starvation | anion homeostasis | response to dexamethasone stimulus | cellular response to oleic acid | hepatocyte differentiation | response to steroid hormone stimulus | carbamoyl phosphate biosynthetic process | response to food | cellular nitrogen compound metabolic process | cellular response to glucagon stimulus","CPS1","carbamoyl-phosphate synthetase 1, mitochondrial","MitoCarta3.0","Metabolism > Amino acid metabolism > Urea cycle","Matrix",0,"ATP-grasp | CPSase_sm_chain | CPSase_L_D2 | MGS | GATase | CPSase_L_chain | CPSase_L_D3","Alanine, aspartate and glutamate metabolism | Arginine and proline metabolism | Nitrogen metabolism","CARBAMOYL PHOSPHATE SYNTHASE ARGININE SPECIFIC SMALL CHAIN CPS A EC_6.3.5.5 ARGININE SPECIFIC CARBAMOYL PHOSPHATE SYNTHETASE GLUTAMINE CHAIN","ENSFM00570000850982",2,"FirstInEnsemblFamily",null,"CPS1",1,0,0,0,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,133,1,0,"Metabolism > Amino acid metabolism > Urea cycle","Metabolism "," Amino acid metabolism "," Urea cycle"]
["hsa:4285",4285,"MIPEP","HMIP|MIP",13,"mitochondrial intermediate peptidase","intracellular membrane-bounded organelle | mitochondrion | mitochondrial matrix","peptidase activity | metalloendopeptidase activity | metal ion binding","proteolysis | protein processing involved in protein targeting to mitochondrion","MIPEP","mitochondrial intermediate peptidase","MitoCarta3.0","Protein import, sorting and homeostasis > Protein import and sorting > Preprotein cleavage | Protein import, sorting and homeostasis > Protein homeostasis > Proteases","Matrix",0,"Peptidase_M3",null,null,null,null,"FirstInEnsemblFamily",null,"MIPEP",1,0,1,1,0,1,1,1,1,1,1,1,0,0,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,133,1,1,"Protein import, sorting and homeostasis > Protein import and sorting > Preprotein cleavage | Protein import, sorting and homeostasis > Protein homeostasis > Proteases","Protein import, sorting and homeostasis "," Protein import and sorting "," Preprotein cleavage "," Protein import, sorting and homeostasis "," Protein homeostasis "," Proteases"]
["hsa:5162",5162,"PDHB","DKFZp564K0164|PDHBD|PHE1B",3,"pyruvate dehydrogenase (lipoamide) beta","mitochondrion | pyruvate dehydrogenase complex | mitochondrial matrix","oxidoreductase activity | pyruvate dehydrogenase (acetyl-transferring) activity","regulation of acetyl-CoA biosynthetic process from pyruvate | pyruvate metabolic process | glycolysis | tricarboxylic acid cycle","PDHB","pyruvate dehydrogenase (lipoamide) beta","MitoCarta3.0","Metabolism > Carbohydrate metabolism > Pyruvate metabolism","Matrix",0,"Transketolase_C | Transket_pyr",null,null,null,null,"FirstInEnsemblFamily","Pyruvate dehydrogenase E1-beta deficiency","PDHB",1,1,0,0,0,0,1,1,1,1,1,1,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,133,1,1,"Metabolism > Carbohydrate metabolism > Pyruvate metabolism","Metabolism "," Carbohydrate metabolism "," Pyruvate metabolism"]
["hsa:51095",51095,"TRNT1","CCA1|MtCCA",3,"tRNA nucleotidyl transferase, CCA-adding, 1","mitochondrion | intracellular","tRNA adenylyltransferase activity | tRNA binding | transferase activity | nucleotide binding | nucleotidyltransferase activity | ATP binding","tRNA 3'-end processing | protein targeting to mitochondrion | RNA processing","TRNT1","tRNA nucleotidyl transferase, CCA-adding, 1","MitoCarta3.0","Mitochondrial central dogma > mtRNA metabolism > mt-tRNA modifications","Matrix",0,"PolyA_pol","RNA transport",null,null,null,"FirstInEnsemblFamily",null,"TRNT1",1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,133,1,1,"Mitochondrial central dogma > mtRNA metabolism > mt-tRNA modifications","Mitochondrial central dogma "," mtRNA metabolism "," mt-tRNA modifications"]
["hsa:50",50,"ACO2","ACONM|MGC20605|MGC33908",22,"aconitase 2, mitochondrial","mitochondrion | nucleus | mitochondrial matrix","citrate hydro-lyase (cis-aconitate-forming) activity | 4 iron, 4 sulfur cluster binding | aconitate hydratase activity | lyase activity | 3 iron, 4 sulfur cluster binding | iron ion binding | isocitrate hydro-lyase (cis-aconitate-forming) activity | metal ion binding","generation of precursor metabolites and energy | isocitrate metabolic process | tricarboxylic acid cycle | metabolic process | citrate metabolic process","ACO2","aconitase 2, mitochondrial","MitoCarta3.0","Metabolism > Carbohydrate metabolism > TCA cycle | Metabolism > Metals and cofactors > Fe-S-containing proteins","Matrix",0,"Aconitase_C | Aconitase","Glyoxylate and dicarboxylate metabolism | Citrate cycle (TCA cycle)",null,null,null,"FirstInEnsemblFamily","Infantile cerebellar-retinal degeneration","ACO2",1,0,0,0,0,0,1,1,1,1,1,1,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,132,1,1,"Metabolism > Carbohydrate metabolism > TCA cycle | Metabolism > Metals and cofactors > Fe-S-containing proteins","Metabolism "," Carbohydrate metabolism "," TCA cycle "," Metabolism "," Metals and cofactors "," Fe-S-containing proteins"]
["hsa:1622",1622,"DBI","ACBD1|ACBP|CCK-RP|EP|MGC70414",2,"diazepam binding inhibitor (GABA receptor modulator, acyl-CoA binding protein)",null,"lipid binding | benzodiazepine receptor binding | fatty-acyl-CoA binding","transport","DBI","diazepam binding inhibitor (GABA receptor modulator, acyl-Coenzyme A binding protein)","MitoCarta3.0","Metabolism > Lipid metabolism","unknown",0,"ACBP",null,null,null,null,"FirstInEnsemblFamily",null,"DBI",1,0,0,0,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,132,1,1,"Metabolism > Lipid metabolism","Metabolism "," Lipid metabolism"]
["hsa:1743",1743,"DLST","DLTS",14,"dihydrolipoamide S-succinyltransferase (E2 component of 2-oxo-glutarate complex)","oxoglutarate dehydrogenase complex | intracellular membrane-bounded organelle | mitochondrion | plasma membrane | nucleus | microsome | mitochondrial matrix | cytoplasm","dihydrolipoyllysine-residue succinyltransferase activity | heat shock protein binding | chaperone binding | transferase activity, transferring acyl groups","generation of precursor metabolites and energy | tricarboxylic acid cycle | cellular nitrogen compound metabolic process | metabolic process | NADH metabolic process | lysine catabolic process | 2-oxoglutarate metabolic process","DLST","dihydrolipoamide S-succinyltransferase (E2 component of 2-oxo-glutarate complex)","MitoCarta3.0","Metabolism > Carbohydrate metabolism > TCA cycle | Metabolism > Amino acid metabolism > Lysine metabolism","Matrix",0,"Biotin_lipoyl | 2-oxoacid_dh","Lysine degradation | Citrate cycle (TCA cycle)",null,null,null,"FirstInEnsemblFamily",null,"DLST",1,0,0,0,0,0,1,1,1,1,1,1,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,132,1,1,"Metabolism > Carbohydrate metabolism > TCA cycle | Metabolism > Amino acid metabolism > Lysine metabolism","Metabolism "," Carbohydrate metabolism "," TCA cycle "," Metabolism "," Amino acid metabolism "," Lysine metabolism"]
["hsa:3032",3032,"HADHB","ECHB|MGC87480|MTPB|TP-BETA",2,"hydroxyacyl-CoA dehydrogenase/3-ketoacyl-CoA thiolase/enoyl-CoA hydratase (trifunctional protein), beta subunit","mitochondrial nucleoid | mitochondrial envelope | mitochondrion | mitochondrial inner membrane | fatty acid beta-oxidation multienzyme complex | mitochondrial matrix","transferase activity, transferring acyl groups other than amino-acyl groups | long-chain-3-hydroxyacyl-CoA dehydrogenase activity | long-chain-enoyl-CoA hydratase activity | enoyl-CoA hydratase activity | acetyl-CoA C-acyltransferase activity | protein binding | NAD binding | fatty-acyl-CoA binding | 3-hydroxyacyl-CoA dehydrogenase activity","fatty acid beta-oxidation | fatty acid metabolic process | cellular lipid metabolic process","HADHB","hydroxyacyl-Coenzyme A dehydrogenase/3-ketoacyl-Coenzyme A thiolase/enoyl-Coenzyme A hydratase (trifunctional protein), beta subunit","MitoCarta3.0","Metabolism > Carbohydrate metabolism > Ketone metabolism | Metabolism > Lipid metabolism > Fatty acid oxidation | Metabolism > Amino acid metabolism > Lysine metabolism","MIM",0,"Thiolase_N | Thiolase_C","Valine, leucine and isoleucine degradation | Fatty acid elongation | Fatty acid metabolism",null,null,null,"FirstInEnsemblFamily","Trifunctional protein deficiency","HADHB",1,1,0,0,1,1,1,1,1,1,1,1,0,0,1,0,0,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,132,1,0,"Metabolism > Carbohydrate metabolism > Ketone metabolism | Metabolism > Lipid metabolism > Fatty acid oxidation | Metabolism > Amino acid metabolism > Lysine metabolism","Metabolism "," Carbohydrate metabolism "," Ketone metabolism "," Metabolism "," Lipid metabolism "," Fatty acid oxidation "," Metabolism "," Amino acid metabolism "," Lysine metabolism"]
["hsa:9617",9617,"MTRF1","MGC47721|MRF1|MTTRF1|RF1",13,"mitochondrial translational release factor 1","mitochondrion | cytoplasm","translation release factor activity, codon specific | translation release factor activity","regulation of translational termination","MTRF1","mitochondrial translational release factor 1","MitoCarta3.0","Mitochondrial central dogma > Translation > Translation factors","Matrix",0,"RF-1 | PCRF",null,"PEPTIDE CHAIN RELEASE FACTOR 1  MITOCHONDRIAL PRECURSOR MITOCHONDRIAL TRANSLATIONAL RELEASE FACTOR 1","ENSFM00250000003049",2,"FirstInEnsemblFamily",null,"MTRF1",1,0,0,0,0,0,1,1,1,1,1,1,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,132,1,1,"Mitochondrial central dogma > Translation > Translation factors","Mitochondrial central dogma "," Translation "," Translation factors"]
["hsa:4898",4898,"NRD1","hNRD1|hNRD2",1,"nardilysin (N-arginine dibasic convertase)","cytosol | cell surface","epidermal growth factor binding | peptidase activity | protein binding | metalloendopeptidase activity | zinc ion binding | metal ion binding","cell proliferation | neuromuscular junction development | proteolysis | cell migration | regulation of endopeptidase activity | positive regulation of membrane protein ectodomain proteolysis","NRD1","nardilysin (N-arginine dibasic convertase)","MitoCarta3.0","Protein import, sorting and homeostasis > Protein homeostasis > Chaperones | Protein import, sorting and homeostasis > Protein import and sorting","Matrix",0,"Peptidase_M16 | Peptidase_M16_C",null,"INSULIN DEGRADING ENZYME EC_3.4.24.56 INSULIN PROTEASE INSULINASE INSULYSIN","ENSFM00250000001013",2,null,null,"NRD1",1,1,0,0,0,0,1,1,1,1,1,1,1,1,1,1,0,0,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,132,1,1,"Protein import, sorting and homeostasis > Protein homeostasis > Chaperones | Protein import, sorting and homeostasis > Protein import and sorting","Protein import, sorting and homeostasis "," Protein homeostasis "," Chaperones "," Protein import, sorting and homeostasis "," Protein import and sorting"]
["hsa:54708",54708,"2013-03-05","FLJ20445|MARCH-V|MITOL|RNF153",10,"membrane-associated ring finger (C3HC4) 5","mitochondrial outer membrane | mitochondrion | integral to membrane | endoplasmic reticulum | endoplasmic reticulum membrane | membrane","ubiquitin-protein ligase activity | GTPase binding | protein binding | zinc ion binding | metal ion binding | ligase activity","negative regulation of cell aging | protein localization in mitochondrion | positive regulation of mitochondrial fission | protein autoubiquitination | protein polyubiquitination | regulation of mitochondrial fission | protein ubiquitination","2013-03-05","membrane-associated ring finger (C3HC4) 5","MitoCarta3.0","Mitochondrial dynamics and surveillance > Mitophagy | Mitochondrial dynamics and surveillance > Autophagy","MOM",4,"zf-C3HC4",null,null,null,null,"FirstInEnsemblFamily",null,"2013-03-05",0,1,0,0,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,131,1,0,"Mitochondrial dynamics and surveillance > Mitophagy | Mitochondrial dynamics and surveillance > Autophagy","Mitochondrial dynamics and surveillance "," Mitophagy ",null," Mitochondrial dynamics and surveillance "," Autophagy"]
["hsa:32",32,"ACACB","ACC2|ACCB|HACC275",12,"acetyl-CoA carboxylase beta","cytosol | endomembrane system | Golgi apparatus | membrane | cytoplasm","protein binding | biotin carboxylase activity | acetyl-CoA carboxylase activity | nucleotide binding | metal ion binding | ligase activity | ATP binding","protein homotetramerization | acetyl-CoA metabolic process | regulation of fatty acid oxidation | cellular lipid metabolic process | energy reserve metabolic process | carnitine shuttle | fatty acid biosynthetic process | positive regulation of cellular metabolic process","ACACB","acetyl-Coenzyme A carboxylase beta","MitoCarta3.0","Metabolism > Lipid metabolism > Fatty acid oxidation | Metabolism > Vitamin metabolism > Biotin utilizing proteins","MOM",1,"Biotin_carb_C | CPSase_L_D2 | Carboxyl_trans | Biotin_lipoyl | CPSase_L_chain | ACC_central","Propanoate metabolism | Pyruvate metabolism | Fatty acid biosynthesis","ACETYL COA CARBOXYLASE EC_6.4.1.2 [INCLUDES BIOTIN CARBOXYLASE EC_6.3.4.- 14 ]","ENSFM00260000050407",2,"FirstInEnsemblFamily",null,"ACACB",1,0,0,0,0,0,1,1,1,1,1,1,1,1,0,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,131,1,0,"Metabolism > Lipid metabolism > Fatty acid oxidation | Metabolism > Vitamin metabolism > Biotin utilizing proteins","Metabolism "," Lipid metabolism "," Fatty acid oxidation "," Metabolism "," Vitamin metabolism "," Biotin utilizing proteins"]
["hsa:91574",91574,"C12orf65","COXPD7|FLJ38663",12,"chromosome 12 open reading frame 65","mitochondrion","translation release factor activity","translational termination",null,null,"MitoCarta3.0","Mitochondrial central dogma > Translation > Translation factors","Matrix",null,null,null,null,null,null,"FirstInEnsemblFamily","Combined oxidative phosphorylation deficiency 7","C12orf65",1,0,0,0,0,0,1,1,1,1,1,1,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,131,1,1,"Mitochondrial central dogma > Translation > Translation factors","Mitochondrial central dogma "," Translation "," Translation factors"]
["hsa:1727",1727,"CYB5R3","B5R|DIA1",22,"cytochrome b5 reductase 3","mitochondrial outer membrane | mitochondrion | endoplasmic reticulum | endoplasmic reticulum membrane | membrane | hemoglobin complex | cytoplasm","cytochrome-b5 reductase activity | oxidoreductase activity","blood circulation | cholesterol biosynthetic process | water-soluble vitamin metabolic process | vitamin metabolic process","CYB5R3","cytochrome b5 reductase 3","MitoCarta3.0","Metabolism > Detoxification > Xenobiotic metabolism | Metabolism > Detoxification > Amidoxime reducing complex | Metabolism > Lipid metabolism","MOM",0,"NAD_binding_1 | FAD_binding_6","Amino sugar and nucleotide sugar metabolism","NADH CYTOCHROME B5 REDUCTASE B5R EC_1.6.2.2","ENSFM00500000269951",3,null,"Methemoglobinemia, type I","CYB5R3",1,0,0,0,0,0,1,1,1,1,1,1,0,0,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,131,1,0,"Metabolism > Detoxification > Xenobiotic metabolism | Metabolism > Detoxification > Amidoxime reducing complex | Metabolism > Lipid metabolism","Metabolism "," Detoxification "," Xenobiotic metabolism "," Metabolism "," Detoxification "," Amidoxime reducing complex "," Metabolism "," Lipid metabolism"]
["hsa:2271",2271,"FH","HLRCC|LRCC|MCL|MCUL1",1,"fumarate hydratase","mitochondrion | cell junction | tricarboxylic acid cycle enzyme complex | mitochondrial matrix | cytoplasm","fumarate hydratase activity | lyase activity","fumarate metabolic process | homeostasis of number of cells within a tissue | malate metabolic process | tricarboxylic acid cycle","FH","fumarate hydratase","MitoCarta3.0","Metabolism > Carbohydrate metabolism > TCA cycle","Matrix",0,"Lyase_1","Citrate cycle (TCA cycle)",null,null,null,"FirstInEnsemblFamily","Fumarase deficiency|Leiomyomatosis and renal cell cancer","FH",1,0,1,1,0,0,0,0,1,1,1,1,0,0,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,131,1,1,"Metabolism > Carbohydrate metabolism > TCA cycle","Metabolism "," Carbohydrate metabolism "," TCA cycle"]
["hsa:54516",54516,"MTRF1L","HMRF1L|MGC102748|MRF1L|mtRF1a",6,"mitochondrial translational release factor 1-like","mitochondrion | cytoplasm","translation release factor activity, codon specific","translational termination","MTRF1L","mitochondrial translational release factor 1-like","MitoCarta3.0","Mitochondrial central dogma > Translation > Translation factors","Matrix",0,"RF-1 | PCRF",null,"PEPTIDE CHAIN RELEASE FACTOR 1  MITOCHONDRIAL PRECURSOR MITOCHONDRIAL TRANSLATIONAL RELEASE FACTOR 1","ENSFM00250000003049",2,null,null,"MTRF1L",1,0,0,0,0,0,1,1,1,1,1,1,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,131,1,0,"Mitochondrial central dogma > Translation > Translation factors","Mitochondrial central dogma "," Translation "," Translation factors"]
["hsa:55278",55278,"QRSL1","DKFZp564C1278|FLJ10989|FLJ12189|FLJ13447|GatA",6,"glutaminyl-tRNA synthase (glutamine-hydrolyzing)-like 1","mitochondrion | glutamyl-tRNA(Gln) amidotransferase complex","glutaminyl-tRNA synthase (glutamine-hydrolyzing) activity | nucleotide binding | ligase activity | ATP binding","glutaminyl-tRNAGln biosynthesis via transamidation | mitochondrial translation","QRSL1","glutaminyl-tRNA synthase (glutamine-hydrolyzing)-like 1","MitoCarta3.0","Mitochondrial central dogma > Translation > mt-tRNA synthetases","Matrix",0,"Amidase",null,null,null,null,"FirstInEnsemblFamily",null,"QRSL1",1,0,0,0,0,0,1,1,1,1,1,0,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,131,1,1,"Mitochondrial central dogma > Translation > mt-tRNA synthetases","Mitochondrial central dogma "," Translation "," mt-tRNA synthetases"]
["hsa:10352",10352,"WARS2","TrpRS",1,"tryptophanyl tRNA synthetase 2, mitochondrial","mitochondrion | mitochondrial matrix | cytoplasm","tryptophan-tRNA ligase activity | nucleotide binding | ligase activity | ATP binding","vasculogenesis | gene expression | tryptophanyl-tRNA aminoacylation | tRNA aminoacylation for protein translation","WARS2","tryptophanyl tRNA synthetase 2 (mitochondrial)","MitoCarta3.0","Mitochondrial central dogma > Translation > mt-tRNA synthetases","Matrix",0,"tRNA-synt_1b","Tryptophan metabolism | Aminoacyl-tRNA biosynthesis",null,null,null,"FirstInEnsemblFamily",null,"WARS2",1,0,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,0,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,131,1,1,"Mitochondrial central dogma > Translation > mt-tRNA synthetases","Mitochondrial central dogma "," Translation "," mt-tRNA synthetases"]
["hsa:10840",10840,"ALDH1L1","10-fTHF|DKFZp781N0997|FDH|FTHFD",3,"aldehyde dehydrogenase 1 family, member L1","mitochondrion | cytoplasm","formyltetrahydrofolate dehydrogenase activity | oxidoreductase activity | methyltransferase activity | hydroxymethyl-, formyl- and related transferase activity | oxidoreductase activity, acting on the aldehyde or oxo group of donors, NAD or NADP as acceptor | acyl carrier activity | catalytic activity | cofactor binding","biosynthetic process | 10-formyltetrahydrofolate catabolic process","ALDH1L1","aldehyde dehydrogenase 1 family, member L1","MitoCarta3.0","Metabolism > Vitamin metabolism > Folate and 1-C metabolism","Matrix",0,"Formyl_trans_C | Formyl_trans_N | Aldedh","One carbon pool by folate","ALDEHYDE DEHYDROGENASE FAMILY 1 MEMBER L1 EC_1.5.1.6 CYTOSOLIC 10 FORMYLTETRAHYDROFOLATE DEHYDROGENASE 10 FTHFDH FDH","ENSFM00550000743150",2,"FirstInEnsemblFamily",null,"ALDH1L1",1,0,1,1,0,1,1,1,1,1,1,1,0,0,0,0,0,1,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,130,1,0,"Metabolism > Vitamin metabolism > Folate and 1-C metabolism","Metabolism "," Vitamin metabolism "," Folate and 1-C metabolism"]
["hsa:1723",1723,"DHODH","DHOdehase|POADS|URA1",16,"dihydroorotate dehydrogenase (quinone)","mitochondrion | integral to membrane | mitochondrial inner membrane | membrane","oxidoreductase activity | dihydroorotate oxidase activity | dihydroorotate dehydrogenase activity","pyrimidine base metabolic process | nucleobase-containing small molecule metabolic process | UMP biosynthetic process | 'de novo' pyrimidine base biosynthetic process | positive regulation of apoptosis | pyrimidine nucleotide biosynthetic process | pyrimidine nucleoside biosynthetic process | regulation of mitochondrial fission","DHODH","dihydroorotate dehydrogenase","MitoCarta3.0","Metabolism > Nucleotide metabolism > Nucleotide synthesis and processing | Metabolism > Electron carriers > Q-linked reactions, other","MIM",1,"DHO_dh","Pyrimidine metabolism",null,null,null,"FirstInEnsemblFamily","Miller syndrome","DHODH",1,0,1,1,0,1,1,1,0,1,0,1,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,130,1,1,"Metabolism > Nucleotide metabolism > Nucleotide synthesis and processing | Metabolism > Electron carriers > Q-linked reactions, other","Metabolism "," Nucleotide metabolism "," Nucleotide synthesis and processing "," Metabolism "," Electron carriers "," Q-linked reactions, other"]
["hsa:80273",80273,"GRPEL1","FLJ25609|HMGE",4,"GrpE-like 1, mitochondrial (E. coli)","mitochondrion | mitochondrial matrix","unfolded protein binding | chaperone binding | protein homodimerization activity | adenyl-nucleotide exchange factor activity","protein folding | protein import into mitochondrial matrix","GRPEL1","GrpE-like 1, mitochondrial (E. coli)","MitoCarta3.0","Protein import, sorting and homeostasis > Protein import and sorting > Import motor | Metabolism > Metals and cofactors > Fe-S cluster biosynthesis","Matrix",0,"GrpE",null,null,null,null,"FirstInEnsemblFamily",null,"GRPEL1",1,0,0,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,130,1,1,"Protein import, sorting and homeostasis > Protein import and sorting > Import motor | Metabolism > Metals and cofactors > Fe-S cluster biosynthesis","Protein import, sorting and homeostasis "," Protein import and sorting "," Import motor "," Metabolism "," Metals and cofactors "," Fe-S cluster biosynthesis"]
["hsa:3419",3419,"IDH3A","-",15,"isocitrate dehydrogenase 3 (NAD+) alpha","mitochondrion | mitochondrial matrix","oxidoreductase activity | NAD binding | oxidoreductase activity, acting on the CH-OH group of donors, NAD or NADP as acceptor | isocitrate dehydrogenase (NAD+) activity | magnesium ion binding","isocitrate metabolic process | tricarboxylic acid cycle | carbohydrate metabolic process | NADH metabolic process | 2-oxoglutarate metabolic process","IDH3A","isocitrate dehydrogenase 3 (NAD+) alpha","MitoCarta3.0","Metabolism > Carbohydrate metabolism > TCA cycle","Matrix",0,"Iso_dh","Citrate cycle (TCA cycle)",null,null,null,"FirstInEnsemblFamily",null,"IDH3A",1,0,1,1,0,1,1,0,1,1,1,0,0,0,0,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,130,1,1,"Metabolism > Carbohydrate metabolism > TCA cycle","Metabolism "," Carbohydrate metabolism "," TCA cycle"]
["hsa:29920",29920,"PYCR2","FLJ54750|P5CR2",1,"pyrroline-5-carboxylate reductase family, member 2","cytoplasm","oxidoreductase activity | oxidoreductase activity, acting on the CH-OH group of donors, NAD or NADP as acceptor | pyrroline-5-carboxylate reductase activity | nucleotide binding","cellular amino acid biosynthetic process | proline biosynthetic process","PYCR2","pyrroline-5-carboxylate reductase family, member 2","MitoCarta3.0","Metabolism > Amino acid metabolism > Proline metabolism","Matrix",0,"NAD_Gly3P_dh_N | F420_oxidored","Arginine and proline metabolism","PYRROLINE 5 CARBOXYLATE REDUCTASE 3 P5C REDUCTASE 3 P5CR 3 EC_1.5.1.2 PYRROLINE 5 CARBOXYLATE REDUCTASE","ENSFM00500000269896",3,null,null,"PYCR2",1,0,0,0,0,1,1,1,1,1,1,1,1,1,0,0,0,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,130,1,0,"Metabolism > Amino acid metabolism > Proline metabolism","Metabolism "," Amino acid metabolism "," Proline metabolism"]
["hsa:10245",10245,"TIMM17B","DXS9822|TIM17B","X","translocase of inner mitochondrial membrane 17 homolog B (yeast)","microtubule cytoskeleton | mitochondrion | integral to membrane | membrane | mitochondrial inner membrane presequence translocase complex | integral to mitochondrial inner membrane","P-P-bond-hydrolysis-driven protein transmembrane transporter activity","intracellular protein transport | protein targeting to mitochondrion","TIMM17B","translocase of inner mitochondrial membrane 17 homolog B (yeast)","MitoCarta3.0","Protein import, sorting and homeostasis > Protein import and sorting > TIM23 presequence pathway","MIM",1,"Tim17",null,"MITOCHONDRIAL IMPORT INNER MEMBRANE TRANSLOCASE SUBUNIT TIM17 A INNER MEMBRANE PREPROTEIN TRANSLOCASE TIM17A","ENSFM00500000270373",2,"FirstInEnsemblFamily",null,"TIMM17B",0,0,0,0,0,0,1,1,0,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,130,1,0,"Protein import, sorting and homeostasis > Protein import and sorting > TIM23 presequence pathway","Protein import, sorting and homeostasis "," Protein import and sorting "," TIM23 presequence pathway"]
["hsa:549",549,"AUH","-",9,"AU RNA binding protein/enoyl-CoA hydratase","mitochondrion | mitochondrial matrix","lyase activity | mRNA 3'-UTR binding | RNA binding | methylglutaconyl-CoA hydratase activity | enoyl-CoA hydratase activity","branched chain family amino acid catabolic process | mRNA catabolic process | cellular nitrogen compound metabolic process","AUH","AU RNA binding protein/enoyl-Coenzyme A hydratase","MitoCarta3.0","Metabolism > Carbohydrate metabolism > Itaconate metabolism | Metabolism > Amino acid metabolism > Branched-chain amino acid metabolism","Matrix",0,"ECH","Valine, leucine and isoleucine degradation","METHYLGLUTACONYL COA HYDRATASE MITOCHONDRIAL PRECURSOR EC_4.2.1.18 AU SPECIFIC RNA BINDING ENOYL COA HYDRATASE AU BINDING COA HYDRATASE","ENSFM00500000270222",2,"FirstInEnsemblFamily","3-methylglutaconic aciduria, type I","AUH",1,0,0,0,0,0,1,1,1,1,1,1,0,0,0,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,129,1,1,"Metabolism > Carbohydrate metabolism > Itaconate metabolism | Metabolism > Amino acid metabolism > Branched-chain amino acid metabolism","Metabolism "," Carbohydrate metabolism "," Itaconate metabolism "," Metabolism "," Amino acid metabolism "," Branched-chain amino acid metabolism"]
["hsa:54205",54205,"CYCS","CYC|HCS|THC4",7,"cytochrome c, somatic","cytosol | mitochondrion | respiratory chain | mitochondrial inner membrane | nucleus | mitochondrial matrix | protein phosphatase type 2A complex | mitochondrial intermembrane space","electron transporter, transferring electrons from CoQH2-cytochrome c reductase complex and cytochrome c oxidase complex activity | protein binding | protein serine/threonine phosphatase activity | heme binding | metal ion binding","cellular respiration | transport | induction of apoptosis by intracellular signals | DNA fragmentation involved in apoptotic nuclear change | activation of caspase activity by cytochrome c | apoptosis | respiratory electron transport chain","CYCS","cytochrome c, somatic","MitoCarta3.0","Metabolism > Metals and cofactors > Heme-containing proteins | Metabolism > Electron carriers > Cytochromes | OXPHOS > OXPHOS subunits | OXPHOS > Cytochrome C | Mitochondrial dynamics and surveillance > Apoptosis","IMS",0,"Cytochrom_C","p53 signaling pathway | Apoptosis",null,null,null,"FirstInEnsemblFamily","Thrombocytopenia 4","CYCS",1,0,0,0,0,0,1,1,1,1,1,1,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,129,1,1,"Metabolism > Metals and cofactors > Heme-containing proteins | Metabolism > Electron carriers > Cytochromes | OXPHOS > OXPHOS subunits | OXPHOS > Cytochrome C | Mitochondrial dynamics and surveillance > Apoptosis","Metabolism "," Metals and cofactors "," Heme-containing proteins "," Metabolism "," Electron carriers "," Cytochromes "," OXPHOS "," OXPHOS subunits ",null," OXPHOS "," Cytochrome C ",null," Mitochondrial dynamics and surveillance "," Apoptosis"]
["hsa:2272",2272,"FHIT","AP3Aase|FRA3B",3,"fragile histidine triad gene","cytosol | intracellular membrane-bounded organelle | plasma membrane | nucleus | cytoplasm","hydrolase activity | bis(5'-adenosyl)-triphosphatase activity | protein binding | nickel ion binding | catalytic activity","diadenosine triphosphate catabolic process | DNA replication | nucleotide metabolic process","FHIT","fragile histidine triad gene","MitoCarta3.0","Metabolism > Nucleotide metabolism > Nucleotide synthesis and processing","Matrix",0,"HIT","Purine metabolism",null,null,null,"FirstInEnsemblFamily",null,"FHIT",1,1,1,1,1,1,1,1,1,1,1,1,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,0,1,1,1,1,1,0,1,1,1,1,1,1,1,0,0,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,129,1,1,"Metabolism > Nucleotide metabolism > Nucleotide synthesis and processing","Metabolism "," Nucleotide metabolism "," Nucleotide synthesis and processing"]
["hsa:79979",79979,"TRMT2B","CXorf34|FLJ12687|dJ341D10.3","X","TRM2 tRNA methyltransferase 2 homolog B (S. cerevisiae)",null,"tRNA (uracil-5-)-methyltransferase activity | transferase activity | RNA methyltransferase activity","tRNA processing | RNA processing","CXorf34","chromosome X open reading frame 34","MitoCarta3.0","Mitochondrial central dogma > mtRNA metabolism > mt-tRNA modifications | Mitochondrial central dogma > mtRNA metabolism > mt-rRNA modifications","Matrix",0,"tRNA_U5-meth_tr",null,"TRNA URACIL 5 METHYLTRANSFERASE HOMOLOG EC_2.1.1.35 TRM2 HOMOLOG","ENSFM00250000001753",2,"FirstInEnsemblFamily",null,"TRMT2B",1,0,1,1,1,1,1,1,1,1,1,1,1,1,0,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,0,1,1,1,1,1,1,1,1,0,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,129,1,0,"Mitochondrial central dogma > mtRNA metabolism > mt-tRNA modifications | Mitochondrial central dogma > mtRNA metabolism > mt-rRNA modifications","Mitochondrial central dogma "," mtRNA metabolism "," mt-tRNA modifications "," Mitochondrial central dogma "," mtRNA metabolism "," mt-rRNA modifications"]
["hsa:7915",7915,"ALDH5A1","SSADH|SSDH",6,"aldehyde dehydrogenase 5 family, member A1","mitochondrion | soluble fraction | mitochondrial matrix","oxidoreductase activity | protein homodimerization activity | succinate-semialdehyde dehydrogenase activity","glycerophospholipid metabolic process | protein homotetramerization | glutamine metabolic process | glutamate metabolic process | galactosylceramide metabolic process | acetate metabolic process | respiratory electron transport chain | central nervous system development | short-chain fatty acid metabolic process | glutathione metabolic process | neurotransmitter catabolic process | gamma-aminobutyric acid catabolic process | glucose metabolic process | succinate metabolic process | synaptic transmission | neurotransmitter secretion","ALDH5A1","aldehyde dehydrogenase 5 family, member A1 (succinate-semialdehyde dehydrogenase)","MitoCarta3.0","Metabolism > Amino acid metabolism > GABA metabolism","Matrix",0,"Aldedh","Alanine, aspartate and glutamate metabolism | Butanoate metabolism",null,null,null,"FirstInEnsemblFamily","Succinic semialdehyde dehydrogenase deficiency","ALDH5A1",1,0,1,1,0,1,1,1,1,1,1,1,1,1,0,0,0,0,0,0,0,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,128,1,1,"Metabolism > Amino acid metabolism > GABA metabolism","Metabolism "," Amino acid metabolism "," GABA metabolism"]
["hsa:79746",79746,"ECHDC3","FLJ20909",10,"enoyl CoA hydratase domain containing 3","mitochondrion","catalytic activity",null,"ECHDC3","enoyl Coenzyme A hydratase domain containing 3","MitoCarta3.0",0,"Matrix",0,"ECH",null,null,null,null,"FirstInEnsemblFamily",null,"ECHDC3",1,0,0,0,0,0,1,1,1,1,1,1,0,0,0,0,0,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,128,1,0,0,0]
["hsa:27247",27247,"NFU1","HIRIP|HIRIP5|MGC142252|MGC142254|MMDS1|NIFUC|Nfu|NifU",2,"NFU1 iron-sulfur cluster scaffold homolog (S. cerevisiae)","cytosol | mitochondrion | nucleus | cytoplasm","4 iron, 4 sulfur cluster binding | protein binding | iron ion binding","iron-sulfur cluster assembly","NFU1","NFU1 iron-sulfur cluster scaffold homolog (S. cerevisiae)","MitoCarta3.0","Metabolism > Metals and cofactors > Fe-S cluster biosynthesis | Metabolism > Metals and cofactors > Fe-S-containing proteins","Matrix",0,"Nfu_N | NifU",null,null,null,null,"FirstInEnsemblFamily","Multiple mitochondrial dysfunctions syndrome 1","NFU1",1,0,0,0,0,1,1,1,1,1,1,1,0,0,1,1,1,1,1,1,1,0,0,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,128,1,1,"Metabolism > Metals and cofactors > Fe-S cluster biosynthesis | Metabolism > Metals and cofactors > Fe-S-containing proteins","Metabolism "," Metals and cofactors "," Fe-S cluster biosynthesis "," Metabolism "," Metals and cofactors "," Fe-S-containing proteins"]
["hsa:160760",160760,"PPTC7","DKFZp686M07120|MGC133072|TA-PP2C|TAPP2C",12,"PTC7 protein phosphatase homolog (S. cerevisiae)","mitochondrion","hydrolase activity | phosphoprotein phosphatase activity | metal ion binding",null,"PPTC7","PTC7 protein phosphatase homolog (S. cerevisiae)","MitoCarta3.0","Signaling","Matrix",0,"SpoIIE",null,null,null,null,"FirstInEnsemblFamily",null,"PPTC7",0,0,0,0,0,1,1,1,1,1,1,1,1,1,0,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,0,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,128,1,1,"Signaling","Signaling"]
["hsa:217",217,"ALDH2","ALDH-E2|ALDHI|ALDM|MGC1806",12,"aldehyde dehydrogenase 2 family (mitochondrial)","mitochondrion | mitochondrial matrix","electron carrier activity | oxidoreductase activity | aldehyde dehydrogenase [NAD(P)+] activity | aldehyde dehydrogenase (NAD) activity","xenobiotic metabolic process | neurotransmitter biosynthetic process | ethanol oxidation | synaptic transmission | alcohol metabolic process | carbohydrate metabolic process","ALDH2","aldehyde dehydrogenase 2 family (mitochondrial)","MitoCarta3.0","Metabolism > Detoxification > Xenobiotic metabolism","Matrix",0,"Aldedh",null,"ALDEHYDE DEHYDROGENASE","ENSFM00250000000201",5,"FirstInEnsemblFamily",null,"ALDH2",1,0,1,1,1,1,1,1,1,1,1,1,0,0,0,0,0,0,0,0,0,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,127,1,0,"Metabolism > Detoxification > Xenobiotic metabolism","Metabolism "," Detoxification "," Xenobiotic metabolism"]
["hsa:1431",1431,"CS","-",12,"citrate synthase","mitochondrion | mitochondrial matrix","transferase activity | citrate (Si)-synthase activity","cellular carbohydrate metabolic process | acetyl-CoA metabolic process | tricarboxylic acid cycle | oxaloacetate metabolic process | carbohydrate metabolic process | citrate metabolic process","CS","citrate synthase","MitoCarta3.0","Metabolism > Carbohydrate metabolism > TCA cycle","Matrix",0,"Citrate_synt","Glyoxylate and dicarboxylate metabolism | Citrate cycle (TCA cycle)",null,null,null,"FirstInEnsemblFamily",null,"CS",1,0,0,0,0,0,1,1,1,1,1,1,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,0,1,1,0,1,1,1,1,1,127,1,1,"Metabolism > Carbohydrate metabolism > TCA cycle","Metabolism "," Carbohydrate metabolism "," TCA cycle"]
["hsa:3336",3336,"HSPE1","CPN10|EPF|GROES|HSP10",2,"heat shock 10kDa protein 1 (chaperonin 10)","mitochondrion | mitochondrial matrix | cytoplasm","unfolded protein binding | protein binding | chaperone binding | ATP binding","activation of caspase activity | protein folding | response to unfolded protein | response to stress","HSPE1","heat shock 10kDa protein 1 (chaperonin 10)","MitoCarta3.0","Protein import, sorting and homeostasis > Protein homeostasis > Chaperones","Matrix",0,"Cpn10",null,null,null,null,"FirstInEnsemblFamily",null,"HSPE1",1,0,1,0,0,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,0,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,127,1,1,"Protein import, sorting and homeostasis > Protein homeostasis > Chaperones","Protein import, sorting and homeostasis "," Protein homeostasis "," Chaperones"]
["hsa:196294",196294,"IMMP1L","FLJ25059|IMP1|IMP1-LIKE",11,"IMP1 inner mitochondrial membrane peptidase-like (S. cerevisiae)","mitochondrion | mitochondrial inner membrane | membrane","serine-type peptidase activity | peptidase activity | molecular_function","proteolysis | biological_process","IMMP1L","IMP1 inner mitochondrial membrane peptidase-like (S. cerevisiae)","MitoCarta3.0","Protein import, sorting and homeostasis > Protein import and sorting > Preprotein cleavage | Protein import, sorting and homeostasis > Protein homeostasis > Proteases","MIM",0,"Peptidase_S24","Protein export",null,null,null,"FirstInEnsemblFamily",null,"IMMP1L",1,0,0,0,0,0,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,0,0,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,127,1,1,"Protein import, sorting and homeostasis > Protein import and sorting > Preprotein cleavage | Protein import, sorting and homeostasis > Protein homeostasis > Proteases","Protein import, sorting and homeostasis "," Protein import and sorting "," Preprotein cleavage "," Protein import, sorting and homeostasis "," Protein homeostasis "," Proteases"]
["hsa:5442",5442,"POLRMT","APOLMT|MTRNAP|MTRPOL|h-mtRPOL",19,"polymerase (RNA) mitochondrial (DNA directed)","mitochondrial nucleoid | mitochondrion | mitochondrial matrix","protein binding | DNA-directed RNA polymerase activity | transferase activity | DNA binding | nucleotidyltransferase activity","transcription initiation from mitochondrial promoter | transcription from mitochondrial promoter","POLRMT","polymerase (RNA) mitochondrial (DNA directed)","MitoCarta3.0","Mitochondrial central dogma > mtDNA maintenance > mtDNA replication | Mitochondrial central dogma > mtDNA maintenance > mtDNA nucleoid | Mitochondrial central dogma > mtRNA metabolism > Transcription","Matrix",0,"RNA_pol",null,null,null,null,"FirstInEnsemblFamily",null,"POLRMT",0,0,0,0,0,0,1,1,1,1,1,1,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,127,1,1,"Mitochondrial central dogma > mtDNA maintenance > mtDNA replication | Mitochondrial central dogma > mtDNA maintenance > mtDNA nucleoid | Mitochondrial central dogma > mtRNA metabolism > Transcription","Mitochondrial central dogma "," mtDNA maintenance "," mtDNA replication "," Mitochondrial central dogma "," mtDNA maintenance "," mtDNA nucleoid "," Mitochondrial central dogma "," mtRNA metabolism "," Transcription"]
["hsa:6832",6832,"SUPV3L1","SUV3",10,"suppressor of var1, 3-like 1 (S. cerevisiae)","mitochondrial nucleoid | mitochondrion | nucleus | mitochondrial degradosome | mitochondrial matrix","double-stranded RNA binding | 3'-5' RNA helicase activity | hydrolase activity, acting on acid anhydrides | helicase activity | DNA binding | nucleotide binding | ATP binding | DNA helicase activity | protein binding | RNA binding | protein homodimerization activity | ATP-dependent RNA helicase activity","mitochondrial RNA surveillance | mitochondrion morphogenesis | DNA duplex unwinding | mitochondrial mRNA catabolic process | mitochondrial mRNA surveillance | mitochondrial RNA 3'-end processing | positive regulation of mitochondrial RNA catabolic process | RNA catabolic process | positive regulation of cell growth | chromatin maintenance | mitochondrial ncRNA surveillance | DNA recombination | anti-apoptosis","SUPV3L1","suppressor of var1, 3-like 1 (S. cerevisiae)","MitoCarta3.0","Mitochondrial central dogma > mtRNA metabolism > mtRNA granules | Mitochondrial central dogma > mtRNA metabolism > mtRNA stability and decay","Matrix",0,"Helicase_C",null,null,null,null,"FirstInEnsemblFamily",null,"SUPV3L1",1,0,0,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,127,1,1,"Mitochondrial central dogma > mtRNA metabolism > mtRNA granules | Mitochondrial central dogma > mtRNA metabolism > mtRNA stability and decay","Mitochondrial central dogma "," mtRNA metabolism "," mtRNA granules "," Mitochondrial central dogma "," mtRNA metabolism "," mtRNA stability and decay"]
["hsa:8659",8659,"ALDH4A1","ALDH4|DKFZp779M035|P5CD|P5CDh",1,"aldehyde dehydrogenase 4 family, member A1","mitochondrion | mitochondrial matrix","electron carrier activity | oxidoreductase activity | aldehyde dehydrogenase (NAD) activity | 1-pyrroline-5-carboxylate dehydrogenase activity","proline catabolic process | proline biosynthetic process | cellular nitrogen compound metabolic process | proline metabolic process","ALDH4A1","aldehyde dehydrogenase 4 family, member A1","MitoCarta3.0","Metabolism > Amino acid metabolism > Proline metabolism | Metabolism > Amino acid metabolism > Glyoxylate metabolism","Matrix",0,"Aldedh","Alanine, aspartate and glutamate metabolism | Arginine and proline metabolism",null,null,null,"FirstInEnsemblFamily","Hyperprolinemia, type II","ALDH4A1",1,0,1,1,0,1,1,1,1,1,1,1,0,0,0,0,0,0,0,0,0,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,126,1,1,"Metabolism > Amino acid metabolism > Proline metabolism | Metabolism > Amino acid metabolism > Glyoxylate metabolism","Metabolism "," Amino acid metabolism "," Proline metabolism "," Metabolism "," Amino acid metabolism "," Glyoxylate metabolism"]
["hsa:51660",51660,"BRP44L","dJ68L15.3",6,"brain protein 44-like","mitochondrion | cellular_component","molecular_function","biological_process","BRP44L","brain protein 44-like","MitoCarta3.0","Metabolism > Carbohydrate metabolism > Gluconeogenesis | Metabolism > Carbohydrate metabolism > Pyruvate metabolism | Metabolism > Carbohydrate metabolism > TCA-associated | Small molecule transport","MIM",0,"UPF0041",null,null,null,null,"FirstInEnsemblFamily","Mitochondrial pyruvate carrier deficiency","BRP44L",0,0,0,0,0,0,1,1,1,1,1,1,0,0,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,126,1,1,"Metabolism > Carbohydrate metabolism > Gluconeogenesis | Metabolism > Carbohydrate metabolism > Pyruvate metabolism | Metabolism > Carbohydrate metabolism > TCA-associated | Small molecule transport","Metabolism "," Carbohydrate metabolism "," Gluconeogenesis "," Metabolism "," Carbohydrate metabolism "," Pyruvate metabolism "," Metabolism "," Carbohydrate metabolism "," TCA-associated "," Small molecule transport"]
["hsa:65003",65003,"MRPL11","L11MT|MGC111024|MRP-L11",11,"mitochondrial ribosomal protein L11","mitochondrion | ribosome | mitochondrial ribosome","structural constituent of ribosome","translation","MRPL11","mitochondrial ribosomal protein L11","MitoCarta3.0","Mitochondrial central dogma > Translation > Mitochondrial ribosome","Matrix",0,"Ribosomal_L11 | Ribosomal_L11_N",null,null,null,null,"FirstInEnsemblFamily",null,"MRPL11",1,0,0,0,0,0,1,1,1,1,1,1,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,0,0,1,1,1,1,1,1,1,126,1,1,"Mitochondrial central dogma > Translation > Mitochondrial ribosome","Mitochondrial central dogma "," Translation "," Mitochondrial ribosome"]
["hsa:56954",56954,"NIT2","MGC111199",3,"nitrilase family, member 2","mitochondrion | cellular_component | cytoplasm","omega-amidase activity | hydrolase activity, acting on carbon-nitrogen (but not peptide) bonds | molecular_function","nitrogen compound metabolic process | biological_process","NIT2","nitrilase family, member 2","MitoCarta3.0","Metabolism > Detoxification","unknown",0,"CN_hydrolase","Alanine, aspartate and glutamate metabolism",null,null,null,"FirstInEnsemblFamily",null,"NIT2",1,0,1,1,0,1,1,1,1,1,1,1,0,0,0,0,0,0,0,0,0,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,126,1,1,"Metabolism > Detoxification","Metabolism "," Detoxification"]
["hsa:55006",55006,"TRMT61B","DKFZp564I2178|FLJ20628",2,"tRNA methyltransferase 61 homolog B (S. cerevisiae)",null,"tRNA (adenine-N1-)-methyltransferase activity | methyltransferase activity | transferase activity","tRNA processing | tRNA methylation",null,null,"MitoCarta3.0","Mitochondrial central dogma > mtRNA metabolism > mt-tRNA modifications | Mitochondrial central dogma > mtRNA metabolism > mt-rRNA modifications | Mitochondrial central dogma > Translation > Mitochondrial ribosome assembly","Matrix",null,"GCD14",null,null,null,null,"FirstInEnsemblFamily",null,"TRMT61B",1,0,1,1,1,0,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,0,1,1,1,0,1,0,1,1,1,0,0,1,0,1,1,1,1,1,1,1,0,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,126,1,0,"Mitochondrial central dogma > mtRNA metabolism > mt-tRNA modifications | Mitochondrial central dogma > mtRNA metabolism > mt-rRNA modifications | Mitochondrial central dogma > Translation > Mitochondrial ribosome assembly","Mitochondrial central dogma "," mtRNA metabolism "," mt-tRNA modifications "," Mitochondrial central dogma "," mtRNA metabolism "," mt-rRNA modifications "," Mitochondrial central dogma "," Translation "," Mitochondrial ribosome assembly"]
["hsa:2746",2746,"GLUD1","GDH|GDH1|GLUD|MGC132003",10,"glutamate dehydrogenase 1","mitochondrion | mitochondrial matrix | cytoplasm","ADP binding | glutamate dehydrogenase [NAD(P)+] activity | nucleotide binding | ATP binding | leucine binding | protein binding | oxidoreductase activity | glutamate dehydrogenase (NAD+) activity | NAD+ binding | GTP binding | identical protein binding","cellular amino acid biosynthetic process | glutamate catabolic process | glutamate biosynthetic process | cellular nitrogen compound metabolic process | positive regulation of insulin secretion","GLUD1","glutamate dehydrogenase 1","MitoCarta3.0","Metabolism > Amino acid metabolism > Glutamate metabolism | Metabolism > Amino acid metabolism > GABA metabolism","Matrix",0,"ELFV_dehydrog_N | ELFV_dehydrog",null,"GLUTAMATE DEHYDROGENASE MITOCHONDRIAL GDH EC_1.4.1.3","ENSFM00250000001520",2,"FirstInEnsemblFamily","Hyperinsulinism-hyperammonemia syndrome","GLUD1",1,0,0,0,1,1,0,1,1,1,1,1,0,0,1,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,125,1,0,"Metabolism > Amino acid metabolism > Glutamate metabolism | Metabolism > Amino acid metabolism > GABA metabolism","Metabolism "," Amino acid metabolism "," Glutamate metabolism "," Metabolism "," Amino acid metabolism "," GABA metabolism"]
["hsa:11112",11112,"HIBADH","MGC40361|NS5ATP1",7,"3-hydroxyisobutyrate dehydrogenase","mitochondrion | mitochondrial matrix","oxidoreductase activity | 3-hydroxyisobutyrate dehydrogenase activity | NAD binding | phosphogluconate dehydrogenase (decarboxylating) activity","branched chain family amino acid catabolic process | valine metabolic process | pentose-phosphate shunt | cellular nitrogen compound metabolic process","HIBADH","3-hydroxyisobutyrate dehydrogenase","MitoCarta3.0","Metabolism > Amino acid metabolism > Branched-chain amino acid metabolism","Matrix",0,"F420_oxidored | NAD_binding_2","Valine, leucine and isoleucine degradation",null,null,null,"FirstInEnsemblFamily",null,"HIBADH",1,1,0,0,0,1,1,1,1,1,1,1,0,0,0,0,0,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,125,1,0,"Metabolism > Amino acid metabolism > Branched-chain amino acid metabolism","Metabolism "," Amino acid metabolism "," Branched-chain amino acid metabolism"]
["hsa:347411",347411,"LOC347411","-","X","brain protein 44-like protein 2",null,null,null,null,null,"MitoCarta3.0","Metabolism > Carbohydrate metabolism > Gluconeogenesis | Metabolism > Carbohydrate metabolism > Pyruvate metabolism | Metabolism > Carbohydrate metabolism > TCA-associated | Small molecule transport","MIM",null,"UPF0041",null,null,null,null,"FirstInEnsemblFamily",null,"LOC347411",0,0,0,0,0,0,1,1,1,1,1,1,0,0,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,0,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,125,1,0,"Metabolism > Carbohydrate metabolism > Gluconeogenesis | Metabolism > Carbohydrate metabolism > Pyruvate metabolism | Metabolism > Carbohydrate metabolism > TCA-associated | Small molecule transport","Metabolism "," Carbohydrate metabolism "," Gluconeogenesis "," Metabolism "," Carbohydrate metabolism "," Pyruvate metabolism "," Metabolism "," Carbohydrate metabolism "," TCA-associated "," Small molecule transport"]
["hsa:22921",22921,"MSRB2","CBS-1|CBS1|MGC26104|MSRB|PILB",10,"methionine sulfoxide reductase B2","mitochondrion","protein-methionine-R-oxide reductase activity | oxidoreductase activity | peptide-methionine-(S)-S-oxide reductase activity | zinc ion binding | sequence-specific DNA binding transcription factor activity | metal ion binding","regulation of transcription, DNA-dependent | protein repair","MSRB2","methionine sulfoxide reductase B2","MitoCarta3.0","Metabolism > Detoxification > ROS and glutathione metabolism | Metabolism > Sulfur metabolism","Matrix",0,"SelR",null,null,null,null,"FirstInEnsemblFamily",null,"MSRB2",1,0,0,0,1,1,1,1,1,1,1,1,0,0,0,0,0,0,0,0,0,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,125,1,0,"Metabolism > Detoxification > ROS and glutathione metabolism | Metabolism > Sulfur metabolism","Metabolism "," Detoxification "," ROS and glutathione metabolism "," Metabolism "," Sulfur metabolism"]
["hsa:9997",9997,"SCO2","MGC125823|MGC125825|SCO1L",22,"SCO cytochrome oxidase deficient homolog 2 (yeast)","mitochondrion | mitochondrial inner membrane","copper ion binding | metal ion binding","oxidation-reduction process | muscle system process | in utero embryonic development | respiratory electron transport chain | copper ion transport | cellular copper ion homeostasis | respiratory chain complex IV assembly | response to activity",null,null,"MitoCarta3.0","OXPHOS > Complex IV > CIV assembly factors | Metabolism > Metals and cofactors > Copper metabolism | OXPHOS > OXPHOS assembly factors","MIM",null,"SCO1-SenC",null,"HOMOLOG MITOCHONDRIAL PRECURSOR","ENSFM00500000270642",2,null,"Cardioencephalomyopathy, fatal infantile, due to cytochrome c oxidase deficiency","SCO2",1,0,0,0,0,0,1,1,1,1,1,1,0,0,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,125,1,0,"OXPHOS > Complex IV > CIV assembly factors | Metabolism > Metals and cofactors > Copper metabolism | OXPHOS > OXPHOS assembly factors","OXPHOS "," Complex IV "," CIV assembly factors "," Metabolism "," Metals and cofactors "," Copper metabolism "," OXPHOS "," OXPHOS assembly factors"]
["hsa:55862",55862,"ECHDC1","DKFZp762M1110|FLJ40827|dJ351K20.2",6,"enoyl CoA hydratase domain containing 1",null,"isomerase activity | catalytic activity",null,"ECHDC1","enoyl Coenzyme A hydratase domain containing 1","MitoCarta3.0","Metabolism > Lipid metabolism","unknown",0,null,null,null,null,null,"FirstInEnsemblFamily",null,"ECHDC1",1,0,0,0,0,0,1,1,1,1,1,1,0,0,0,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,0,1,1,0,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,124,1,0,"Metabolism > Lipid metabolism","Metabolism "," Lipid metabolism"]
["hsa:2235",2235,"FECH","EPP|FCE",18,"ferrochelatase","mitochondrion | mitochondrial inner membrane | membrane | mitochondrial matrix","protein binding | ferrous iron binding | 2 iron, 2 sulfur cluster binding | ferrochelatase activity | metal ion binding","protoporphyrinogen IX metabolic process | porphyrin-containing compound metabolic process | heme biosynthetic process | generation of precursor metabolites and energy | response to light stimulus","FECH","ferrochelatase (protoporphyria)","MitoCarta3.0","Metabolism > Metals and cofactors > Heme synthesis and processing | Metabolism > Metals and cofactors > Fe-S-containing proteins","MIM",0,"Ferrochelatase","Porphyrin and chlorophyll metabolism",null,null,null,"FirstInEnsemblFamily","Protoporphyria, erythropoietic, autosomal recessive","FECH",1,0,0,0,0,0,0,0,1,1,1,1,0,0,0,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,124,1,1,"Metabolism > Metals and cofactors > Heme synthesis and processing | Metabolism > Metals and cofactors > Fe-S-containing proteins","Metabolism "," Metals and cofactors "," Heme synthesis and processing "," Metabolism "," Metals and cofactors "," Fe-S-containing proteins"]
["hsa:10434",10434,"LYPLA1","APT-1|LPL1|LYSOPLA|hAPT1",8,"lysophospholipase I","cytosol | mitochondrion | cytoplasm","hydrolase activity | lysophospholipase activity | palmitoyl-(protein) hydrolase activity","regulation of nitric-oxide synthase activity | fatty acid metabolic process | nitric oxide metabolic process | lipid metabolic process","LYPLA1","lysophospholipase I","MitoCarta3.0","Metabolism > Lipid metabolism | Signaling","unknown",0,"Abhydrolase_2","Glycerophospholipid metabolism","ACYL THIOESTERASE 1 APT 1 EC_3.1.2.- LYSOPHOSPHOLIPASE 1 LYSOPHOSPHOLIPASE I LPL I LYSOPLA I","ENSFM00500000270136",2,null,null,"LYPLA1",1,0,0,0,0,0,1,1,1,1,1,1,1,1,0,0,0,0,0,0,0,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,124,1,1,"Metabolism > Lipid metabolism | Signaling","Metabolism "," Lipid metabolism ",null," Signaling"]
["hsa:10873",10873,"ME3","FLJ34862|NADP-ME",11,"malic enzyme 3, NADP(+)-dependent, mitochondrial","mitochondrion | mitochondrial matrix","malate dehydrogenase (oxaloacetate-decarboxylating) activity | oxidoreductase activity | malate dehydrogenase (oxaloacetate-decarboxylating) (NADP+) activity | NAD binding | malic enzyme activity | cofactor binding | metal ion binding","oxidation-reduction process | pyruvate metabolic process | malate metabolic process | oxygen metabolic process | aerobic respiration","ME3","malic enzyme 3, NADP(+)-dependent, mitochondrial","MitoCarta3.0","Metabolism > Carbohydrate metabolism > TCA-associated","Matrix",0,"Malic_M | malic","Pyruvate metabolism","NADP DEPENDENT MALIC ENZYME NADP ME EC_1.1.1.40 MALIC ENZYME","ENSFM00250000000979",3,null,null,"ME3",1,0,1,1,1,1,1,1,1,1,1,0,1,1,0,0,0,0,0,0,0,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,124,1,0,"Metabolism > Carbohydrate metabolism > TCA-associated","Metabolism "," Carbohydrate metabolism "," TCA-associated"]
["hsa:4968",4968,"OGG1","HMMH|HOGG1|MUTM|OGH1",3,"8-oxoguanine DNA glycosylase","nucleoplasm | mitochondrion | nuclear speck | nucleolus | nucleus | nuclear matrix | cytoplasm","damaged DNA binding | protein binding | lyase activity | hydrolase activity, acting on glycosyl bonds | oxidized purine base lesion DNA N-glycosylase activity | endonuclease activity","response to oxidative stress | depurination | base-excision repair | regulation of protein import into nucleus, translocation | response to radiation | regulation of transcription, DNA-dependent | nucleotide-excision repair | DNA repair | base-excision repair, AP site formation","OGG1","8-oxoguanine DNA glycosylase","MitoCarta3.0","Mitochondrial central dogma > mtDNA maintenance > mtDNA repair","Matrix",0,"HHH | HhH-GPD | OGG_N","Base excision repair",null,null,null,"FirstInEnsemblFamily",null,"OGG1",1,1,0,0,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,0,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,0,1,1,1,1,1,1,1,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,0,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,124,1,1,"Mitochondrial central dogma > mtDNA maintenance > mtDNA repair","Mitochondrial central dogma "," mtDNA maintenance "," mtDNA repair"]
["hsa:10469",10469,"TIMM44","DKFZp686H05241|TIM44",19,"translocase of inner mitochondrial membrane 44 homolog (yeast)","mitochondrion | mitochondrial inner membrane | membrane | mitochondrial matrix | mitochondrial inner membrane presequence translocase complex","protein binding | chaperone binding | P-P-bond-hydrolysis-driven protein transmembrane transporter activity | nucleotide binding | ATP binding","intracellular protein transport | protein targeting to mitochondrion","TIMM44","translocase of inner mitochondrial membrane 44 homolog (yeast)","MitoCarta3.0","Protein import, sorting and homeostasis > Protein import and sorting > Import motor","MIM",0,"Tim44",null,null,null,null,"FirstInEnsemblFamily",null,"TIMM44",1,0,0,0,0,0,0,0,0,0,0,1,1,1,1,1,1,1,1,1,1,1,1,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,124,1,1,"Protein import, sorting and homeostasis > Protein import and sorting > Import motor","Protein import, sorting and homeostasis "," Protein import and sorting "," Import motor"]
["hsa:29958",29958,"DMGDH","DMGDHD|ME2GLYDH",5,"dimethylglycine dehydrogenase","mitochondrion | mitochondrial matrix | cytoplasm","electron carrier activity | oxidoreductase activity | folic acid binding | dimethylglycine dehydrogenase activity | aminomethyltransferase activity | flavin adenine dinucleotide binding","glycine catabolic process | choline metabolic process | glycine metabolic process","DMGDH","dimethylglycine dehydrogenase","MitoCarta3.0","Metabolism > Amino acid metabolism > Glycine metabolism | Metabolism > Vitamin metabolism > Choline and betaine metabolism | Metabolism > Vitamin metabolism > Folate and 1-C metabolism","Matrix",0,"GCV_T_C | DAO | GCV_T","Glycine, serine and threonine metabolism","DEHYDROGENASE MITOCHONDRIAL PRECURSOR","ENSFM00250000000791",3,"FirstInEnsemblFamily","Dimethylglycine dehydrogenase deficiency","DMGDH",1,0,1,0,0,0,1,1,1,1,1,0,0,0,0,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,0,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,123,1,0,"Metabolism > Amino acid metabolism > Glycine metabolism | Metabolism > Vitamin metabolism > Choline and betaine metabolism | Metabolism > Vitamin metabolism > Folate and 1-C metabolism","Metabolism "," Amino acid metabolism "," Glycine metabolism "," Metabolism "," Vitamin metabolism "," Choline and betaine metabolism "," Metabolism "," Vitamin metabolism "," Folate and 1-C metabolism"]
["hsa:28976",28976,"ACAD9","FLJ23533|MGC14452|NPD002",3,"acyl-CoA dehydrogenase family, member 9","mitochondrion","acyl-CoA dehydrogenase activity | flavin adenine dinucleotide binding",null,"ACAD9","acyl-Coenzyme A dehydrogenase family, member 9","MitoCarta3.0","OXPHOS > Complex I > CI assembly factors | OXPHOS > OXPHOS assembly factors","MIM",0,"Acyl-CoA_dh_1 | Acyl-CoA_dh_N | Acyl-CoA_dh_M | Acyl-CoA_dh_2",null,"VERY LONG CHAIN SPECIFIC ACYL COA DEHYDROGENASE MITOCHONDRIAL PRECURSOR EC_1.3.99.-","ENSFM00500000270065",2,null,"ACAD9 deficiency","ACAD9",1,0,0,0,0,0,1,1,1,1,1,1,0,0,0,0,0,0,0,0,0,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,122,1,0,"OXPHOS > Complex I > CI assembly factors | OXPHOS > OXPHOS assembly factors","OXPHOS "," Complex I "," CI assembly factors "," OXPHOS "," OXPHOS assembly factors"]
["hsa:1584",1584,"CYP11B1","CPN1|CYP11B|DKFZp686B05283|FHI|FLJ36771|P450C11",8,"cytochrome P450, family 11, subfamily B, polypeptide 1","mitochondrion | mitochondrial inner membrane | membrane","electron carrier activity | steroid 11-beta-monooxygenase activity | oxidoreductase activity, acting on paired donors, with incorporation or reduction of molecular oxygen | monooxygenase activity | heme binding | metal ion binding","oxidation-reduction process | cellular response to potassium ion | steroid metabolic process | immune response | C21-steroid hormone biosynthetic process | cortisol biosynthetic process | response to stress | cellular response to hormone stimulus | mineralocorticoid biosynthetic process | xenobiotic metabolic process | glucose homeostasis | aldosterone biosynthetic process | regulation of blood pressure | glucocorticoid biosynthetic process","CYP11B1","cytochrome P450, family 11, subfamily B, polypeptide 1","MitoCarta3.0","Metabolism > Lipid metabolism > Cholesterol, bile acid, steroid synthesis | Metabolism > Metals and cofactors > Heme-containing proteins","MIM",0,"p450","Steroid hormone biosynthesis","CHOLESTEROL SIDE CHAIN CLEAVAGE ENZYME MITOCHONDRIAL PRECURSOR EC_1.14.15.6 CYPXIA1 CHOLESTEROL DESMOLASE CYTOCHROME P450 11A1 CYTOCHROME P450 SCC","ENSFM00500000269868",3,null,"Adrenal hyperplasia, congenital, due to 11-beta-hydroxylase deficiency|\"Aldosteronism, glucocorticoid-remediable\"","CYP11B1",1,0,0,0,0,0,1,1,1,1,1,1,0,0,0,0,0,0,0,0,0,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,122,1,0,"Metabolism > Lipid metabolism > Cholesterol, bile acid, steroid synthesis | Metabolism > Metals and cofactors > Heme-containing proteins","Metabolism "," Lipid metabolism "," Cholesterol, bile acid, steroid synthesis "," Metabolism "," Metals and cofactors "," Heme-containing proteins"]
["hsa:1593",1593,"CYP27A1","CP27|CTX|CYP27",2,"cytochrome P450, family 27, subfamily A, polypeptide 1","mitochondrion | mitochondrial inner membrane | membrane | mitochondrial matrix","electron carrier activity | oxidoreductase activity, acting on paired donors, with incorporation or reduction of molecular oxygen | cholesterol 26-hydroxylase activity | vitamin D3 25-hydroxylase activity | heme binding | steroid hydroxylase activity | cholestanetriol 26-monooxygenase activity | metal ion binding","xenobiotic metabolic process | cholesterol metabolic process | bile acid biosynthetic process | bile acid metabolic process","CYP27A1","cytochrome P450, family 27, subfamily A, polypeptide 1","MitoCarta3.0","Metabolism > Lipid metabolism > Cholesterol, bile acid, steroid synthesis | Metabolism > Metals and cofactors > Heme-containing proteins","MIM",0,"p450","Primary bile acid biosynthesis","25 D HYDROXYLASE MITOCHONDRIAL PRECURSOR D 3 HYDROXYLASE CYTOCHROME P450 CYTOCHROME P450","ENSFM00500000269772",4,"FirstInEnsemblFamily","Cerebrotendinous xanthomatosis","CYP27A1",1,0,0,0,0,0,1,1,1,1,1,1,0,0,0,0,0,0,0,0,0,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,122,1,0,"Metabolism > Lipid metabolism > Cholesterol, bile acid, steroid synthesis | Metabolism > Metals and cofactors > Heme-containing proteins","Metabolism "," Lipid metabolism "," Cholesterol, bile acid, steroid synthesis "," Metabolism "," Metals and cofactors "," Heme-containing proteins"]
["hsa:3421",3421,"IDH3G","H-IDHG","X","isocitrate dehydrogenase 3 (NAD+) gamma","mitochondrion | nucleolus | nucleus | mitochondrial matrix","oxidoreductase activity | NAD binding | oxidoreductase activity, acting on the CH-OH group of donors, NAD or NADP as acceptor | isocitrate dehydrogenase (NAD+) activity | magnesium ion binding | ATP binding","negative regulation of growth | isocitrate metabolic process | tricarboxylic acid cycle | carbohydrate metabolic process | NADH metabolic process | 2-oxoglutarate metabolic process","IDH3G","isocitrate dehydrogenase 3 (NAD+) gamma","MitoCarta3.0","Metabolism > Carbohydrate metabolism > TCA cycle","Matrix",0,"Iso_dh","Citrate cycle (TCA cycle)","ISOCITRATE DEHYDROGENASE [NAD] GAMMA MITOCHONDRIAL PRECURSOR EC_1.1.1.41 ISOCITRIC DEHYDROGENASE SUBUNIT GAMMA NAD + SPECIFIC ICDH SUBUNIT GAMMA","ENSFM00560000770977",2,null,null,"IDH3G",1,0,1,1,0,1,0,0,1,1,1,0,0,0,0,0,0,0,0,0,0,0,0,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,122,1,1,"Metabolism > Carbohydrate metabolism > TCA cycle","Metabolism "," Carbohydrate metabolism "," TCA cycle"]
["hsa:4129",4129,"MAOB","MGC26382","X","monoamine oxidase B","mitochondrial outer membrane | mitochondrial envelope | mitochondrion | integral to membrane | mitochondrial inner membrane | membrane","electron carrier activity | oxidoreductase activity | primary amine oxidase activity | protein homodimerization activity | flavin adenine dinucleotide binding","response to aluminum ion | negative regulation of serotonin secretion | response to toxin | response to drug | response to corticosterone stimulus | xenobiotic metabolic process | response to selenium ion | positive regulation of dopamine metabolic process | response to steroid hormone stimulus | response to lipopolysaccharide | response to ethanol","MAOB","monoamine oxidase B","MitoCarta3.0","Metabolism > Amino acid metabolism > Catechol metabolism | Metabolism > Detoxification > Xenobiotic metabolism","MOM",1,"Amino_oxidase",null,"AMINE OXIDASE [FLAVIN CONTAINING] EC_1.4.3.4 MONOAMINE OXIDASE TYPE MAO","ENSFM00250000002249",2,null,null,"MAOB",1,0,0,1,0,0,0,0,0,0,0,1,0,0,1,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,122,1,0,"Metabolism > Amino acid metabolism > Catechol metabolism | Metabolism > Detoxification > Xenobiotic metabolism","Metabolism "," Amino acid metabolism "," Catechol metabolism "," Metabolism "," Detoxification "," Xenobiotic metabolism"]
["hsa:4482",4482,"MSRA","PMSR",8,"methionine sulfoxide reductase A","mitochondrion | nucleus | cytoplasm","oxidoreductase activity | peptide-methionine-(S)-S-oxide reductase activity","response to oxidative stress | protein modification process | aging | methionine metabolic process | protein metabolic process","MSRA","methionine sulfoxide reductase A","MitoCarta3.0","Metabolism > Detoxification > ROS and glutathione metabolism | Metabolism > Sulfur metabolism","Matrix",0,"PMSR",null,null,null,null,"FirstInEnsemblFamily",null,"MSRA",1,0,0,0,1,1,1,1,1,1,1,1,0,0,0,0,0,0,0,0,0,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,122,1,1,"Metabolism > Detoxification > ROS and glutathione metabolism | Metabolism > Sulfur metabolism","Metabolism "," Detoxification "," ROS and glutathione metabolism "," Metabolism "," Sulfur metabolism"]
["hsa:1371",1371,"CPOX","CPO|CPX|HCP",3,"coproporphyrinogen oxidase","mitochondrion | mitochondrial inner membrane | mitochondrial intermembrane space | cytoplasm","oxidoreductase activity | protein homodimerization activity | coproporphyrinogen oxidase activity","response to lead ion | porphyrin-containing compound metabolic process | heme biosynthetic process | response to insecticide | response to methylmercury | response to arsenic-containing substance | response to iron ion | protoporphyrinogen IX biosynthetic process","CPOX","coproporphyrinogen oxidase","MitoCarta3.0","Metabolism > Metals and cofactors > Heme synthesis and processing","MIM",0,"Coprogen_oxidas","Porphyrin and chlorophyll metabolism",null,null,null,"FirstInEnsemblFamily","Coproporphyria","CPOX",1,0,0,0,0,0,0,0,1,1,1,1,0,0,0,0,0,1,1,1,0,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,0,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,121,1,1,"Metabolism > Metals and cofactors > Heme synthesis and processing","Metabolism "," Metals and cofactors "," Heme synthesis and processing"]
["hsa:3155",3155,"HMGCL","HL",1,"3-hydroxymethyl-3-methylglutaryl-CoA lyase","mitochondrion | mitochondrial inner membrane | mitochondrial matrix","lyase activity | hydroxymethylglutaryl-CoA lyase activity | carboxylic acid binding | fatty-acyl-CoA binding | metal ion binding","acyl-CoA metabolic process | ketone body biosynthetic process | mitochondrion organization | cellular lipid metabolic process | embryo development | liver development | response to starvation | response to fatty acid | cellular ketone body metabolic process | response to nutrient | acetoacetic acid biosynthetic process","HMGCL","3-hydroxymethyl-3-methylglutaryl-Coenzyme A lyase (hydroxymethylglutaricaciduria)","MitoCarta3.0","Metabolism > Carbohydrate metabolism > Ketone metabolism | Metabolism > Amino acid metabolism > Branched-chain amino acid metabolism","Matrix",0,"HMGL-like",null,"HYDROXYMETHYLGLUTARYL COA LYASE MITOCHONDRIAL PRECURSOR HL HMG COA LYASE EC_4.1.3.4 3 HYDROXY 3 METHYLGLUTARATE COA LYASE","ENSFM00250000002331",2,"FirstInEnsemblFamily","HMG-CoA lyase deficiency","HMGCL",1,0,1,1,0,0,1,1,0,0,0,1,0,0,0,0,0,0,0,0,0,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,121,1,1,"Metabolism > Carbohydrate metabolism > Ketone metabolism | Metabolism > Amino acid metabolism > Branched-chain amino acid metabolism","Metabolism "," Carbohydrate metabolism "," Ketone metabolism "," Metabolism "," Amino acid metabolism "," Branched-chain amino acid metabolism"]
["hsa:91419",91419,"XRCC6BP1","KUB3|MGC134817|MGC134818",12,"XRCC6 binding protein 1","DNA-dependent protein kinase-DNA ligase 4 complex","peptidase activity | DNA-dependent protein kinase activity | metalloendopeptidase activity | metal ion binding","proteolysis | double-strand break repair via nonhomologous end joining","XRCC6BP1","XRCC6 binding protein 1","MitoCarta3.0","Protein import, sorting and homeostasis > Protein homeostasis > Proteases","IMS",0,null,null,null,null,null,"FirstInEnsemblFamily",null,"XRCC6BP1",0,0,0,0,0,0,1,1,1,1,1,1,0,0,0,0,0,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,0,0,0,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,121,1,1,"Protein import, sorting and homeostasis > Protein homeostasis > Proteases","Protein import, sorting and homeostasis "," Protein homeostasis "," Proteases"]
["hsa:81889",81889,"FAHD1","C16orf36|DKFZp566J2046|MGC74876|YISKL",16,"fumarylacetoacetate hydrolase domain containing 1","cytosol | mitochondrion","hydrolase activity | acylpyruvate hydrolase activity | metal ion binding | fumarylpyruvate hydrolase activity | acetylpyruvate hydrolase activity",null,"FAHD1","fumarylacetoacetate hydrolase domain containing 1","MitoCarta3.0","Metabolism > Carbohydrate metabolism > TCA-associated","Matrix",0,"FAA_hydrolase",null,null,null,null,"FirstInEnsemblFamily",null,"FAHD1",1,0,0,0,0,0,0,1,1,1,1,0,0,0,0,0,0,0,0,0,0,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,120,1,1,"Metabolism > Carbohydrate metabolism > TCA-associated","Metabolism "," Carbohydrate metabolism "," TCA-associated"]
["hsa:51011",51011,"FAHD2A","FLJ35661|MGC131995",2,"fumarylacetoacetate hydrolase domain containing 2A",null,"hydrolase activity | metal ion binding",null,"FAHD2A","fumarylacetoacetate hydrolase domain containing 2A","MitoCarta3.0",0,"Matrix",0,"FAA_hydrolase",null,"FUMARYLACETOACETATE HYDROLASE DOMAIN CONTAINING EC 3","ENSFM00500000271766",2,"FirstInEnsemblFamily",null,"FAHD2A",1,0,0,0,0,0,0,1,1,1,1,0,0,0,0,0,0,0,0,0,0,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,120,1,0,0,0]
["hsa:92106",92106,"OXNAD1","MGC15763",3,"oxidoreductase NAD-binding domain containing 1","mitochondrion","oxidoreductase activity",null,"OXNAD1","oxidoreductase NAD-binding domain containing 1","MitoCarta3.0",0,"Matrix",0,"NAD_binding_1 | NAD_binding_6",null,null,null,null,"FirstInEnsemblFamily",null,"OXNAD1",1,0,0,0,1,0,1,0,1,1,1,1,0,0,0,0,0,1,1,1,0,1,1,0,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,0,1,0,1,1,1,1,0,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,120,1,0,0,0]
["hsa:5163",5163,"PDK1","-",2,"pyruvate dehydrogenase kinase, isozyme 1","mitochondrion | pyruvate dehydrogenase complex | mitochondrial matrix","two-component sensor activity | protein heterodimerization activity | transferase activity | protein kinase activity | nucleotide binding | ATP binding | pyruvate dehydrogenase (acetyl-transferring) kinase activity | protein complex binding | protein homodimerization activity","cell surface receptor linked signaling pathway | regulation of acetyl-CoA biosynthetic process from pyruvate | pyruvate metabolic process | peptidyl-histidine phosphorylation | small GTPase mediated signal transduction | protein phosphorylation | glucose metabolic process | carbohydrate metabolic process","PDK1","pyruvate dehydrogenase kinase, isozyme 1","MitoCarta3.0","Metabolism > Carbohydrate metabolism > Pyruvate metabolism","Matrix",0,"HATPase_c",null,"[PYRUVATE DEHYDROGENASE [LIPOAMIDE]] KINASE ISOZYME MITOCHONDRIAL PRECURSOR EC_2.7.11.2 PYRUVATE DEHYDROGENASE KINASE","ENSFM00500000269765",4,"FirstInEnsemblFamily",null,"PDK1",1,0,0,0,0,0,1,1,1,1,1,1,0,0,0,0,0,0,0,0,0,0,0,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,120,1,0,"Metabolism > Carbohydrate metabolism > Pyruvate metabolism","Metabolism "," Carbohydrate metabolism "," Pyruvate metabolism"]
["hsa:51657",51657,"STYXL1","DUSP24|MK-STYX",7,"serine/threonine/tyrosine interacting-like 1","intracellular","hydrolase activity | protein tyrosine/serine/threonine phosphatase activity","protein dephosphorylation | intracellular signal transduction","STYXL1","serine/threonine/tyrosine interacting-like 1","MitoCarta3.0","Mitochondrial dynamics and surveillance > Apoptosis","Matrix",0,"DSPc",null,null,null,null,"FirstInEnsemblFamily",null,"STYXL1",0,0,1,1,1,1,1,1,1,1,1,1,1,1,0,0,0,1,1,1,1,1,0,1,1,1,1,0,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,0,1,0,1,1,0,1,1,1,1,1,0,1,1,1,1,0,1,1,1,0,1,0,0,1,1,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,120,1,0,"Mitochondrial dynamics and surveillance > Apoptosis","Mitochondrial dynamics and surveillance "," Apoptosis"]
["hsa:10295",10295,"BCKDK","-",16,"branched chain ketoacid dehydrogenase kinase","mitochondrion | mitochondrial matrix | mitochondrial alpha-ketoglutarate dehydrogenase complex","protein serine/threonine kinase activity | two-component sensor activity | [3-methyl-2-oxobutanoate dehydrogenase (acetyl-transferring)] kinase activity | kinase activity | nucleotide binding | ATP binding","branched chain family amino acid catabolic process | peptidyl-histidine phosphorylation | cellular amino acid catabolic process | phosphorylation","BCKDK","branched chain ketoacid dehydrogenase kinase","MitoCarta3.0","Metabolism > Amino acid metabolism > Branched-chain amino acid metabolism | Metabolism > Amino acid metabolism > Branched-chain amino acid dehydrogenase complex","Matrix",0,"HATPase_c",null,null,null,null,"FirstInEnsemblFamily","Branched-chain ketoacid dehydrogenase kinase deficiency","BCKDK",1,0,0,0,0,0,1,1,1,1,0,1,0,0,0,0,0,0,0,0,0,0,0,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,119,1,0,"Metabolism > Amino acid metabolism > Branched-chain amino acid metabolism | Metabolism > Amino acid metabolism > Branched-chain amino acid dehydrogenase complex","Metabolism "," Amino acid metabolism "," Branched-chain amino acid metabolism "," Metabolism "," Amino acid metabolism "," Branched-chain amino acid dehydrogenase complex"]
["hsa:5827",5827,"PXMP2","FLJ54922|PMP22",12,"peroxisomal membrane protein 2, 22kDa","mitochondrion | integral to membrane | peroxisomal membrane | peroxisome | membrane","protein binding",null,"PXMP2","peroxisomal membrane protein 2, 22kDa","MitoCarta3.0",0,"MIM",4,"Mpv17_PMP22","Peroxisome",null,null,null,"FirstInEnsemblFamily",null,"PXMP2",0,0,0,0,0,0,0,0,0,0,0,1,0,0,0,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,119,1,0,0,0]
["hsa:2639",2639,"GCDH","ACAD5|GCD",19,"glutaryl-CoA dehydrogenase","mitochondrion | mitochondrial inner membrane | mitochondrial matrix","glutaryl-CoA dehydrogenase activity | fatty-acyl-CoA binding | flavin adenine dinucleotide binding","acyl-CoA metabolic process | fatty-acyl-CoA biosynthetic process | cellular nitrogen compound metabolic process | lysine catabolic process | fatty acid oxidation","GCDH","glutaryl-Coenzyme A dehydrogenase","MitoCarta3.0","Metabolism > Amino acid metabolism > Lysine metabolism","Matrix",0,"Acyl-CoA_dh_1 | Acyl-CoA_dh_N | Acyl-CoA_dh_M | Acyl-CoA_dh_2","Tryptophan metabolism | Lysine degradation | Fatty acid metabolism",null,null,null,"FirstInEnsemblFamily","Glutaricaciduria, type I","GCDH",1,0,0,0,0,0,1,1,1,1,1,1,0,0,0,0,0,0,0,0,0,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,0,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,118,1,0,"Metabolism > Amino acid metabolism > Lysine metabolism","Metabolism "," Amino acid metabolism "," Lysine metabolism"]
["hsa:5188",5188,"PET112","PET112L",4,"PET112 homolog (yeast)","mitochondrion | glutamyl-tRNA(Gln) amidotransferase complex","glutaminyl-tRNA synthase (glutamine-hydrolyzing) activity | translation factor activity, nucleic acid binding | nucleotide binding | ligase activity | ATP binding","glutaminyl-tRNAGln biosynthesis via transamidation | translation | mitochondrial translation","PET112L","PET112-like (yeast)","MitoCarta3.0","Mitochondrial central dogma > Translation > mt-tRNA synthetases","Matrix",0,"GatB_N | GatB_Yqey | GatB",null,null,null,null,"FirstInEnsemblFamily",null,"PET112",1,0,0,0,0,0,0,0,0,0,0,1,0,0,1,1,1,1,1,1,1,1,1,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,0,1,1,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,118,1,1,"Mitochondrial central dogma > Translation > mt-tRNA synthetases","Mitochondrial central dogma "," Translation "," mt-tRNA synthetases"]
["hsa:26517",26517,"TIMM13","TIM13|TIM13B|TIMM13A|TIMM13B|ppv1",19,"translocase of inner mitochondrial membrane 13 homolog (yeast)","mitochondrion | mitochondrial inner membrane | mitochondrial intermembrane space protein transporter complex | membrane","zinc ion binding | metal ion binding","chaperone-mediated protein transport | protein import into mitochondrial inner membrane | protein targeting to mitochondrion | sensory perception of sound","TIMM13","translocase of inner mitochondrial membrane 13 homolog (yeast)","MitoCarta3.0","Protein import, sorting and homeostasis > Protein homeostasis > Chaperones | Protein import, sorting and homeostasis > Protein import and sorting","MIM",0,"zf-Tim10_DDP",null,null,null,null,"FirstInEnsemblFamily",null,"TIMM13",0,0,0,0,0,0,0,0,0,0,0,1,0,0,1,1,1,1,1,1,1,1,1,0,0,1,1,0,1,0,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,118,1,1,"Protein import, sorting and homeostasis > Protein homeostasis > Chaperones | Protein import, sorting and homeostasis > Protein import and sorting","Protein import, sorting and homeostasis "," Protein homeostasis "," Chaperones "," Protein import, sorting and homeostasis "," Protein import and sorting"]
["hsa:5825",5825,"ABCD3","ABC43|PMP70|PXMP1|ZWS2",1,"ATP-binding cassette, sub-family D (ALD), member 3","intracellular membrane-bounded organelle | mitochondrion | peroxisome | cytosol | integral to membrane | peroxisomal membrane | membrane | integral to peroxisomal membrane","ATPase activity, coupled to transmembrane movement of substances | protein binding | protein self-association | ATPase activity | nucleotide binding | ATP binding","peroxisomal long-chain fatty acid import | response to drug | peroxisome organization | transmembrane transport","ABCD3","ATP-binding cassette, sub-family D (ALD), member 3","MitoCarta3.0","Small molecule transport > ABC transporters","Membrane",3,"ABC_tran | ABC_membrane_2","Peroxisome","ATP BINDING CASSETTE SUB FAMILY D MEMBER","ENSFM00250000000815",3,null,null,"ABCD3",1,0,0,0,0,0,1,1,1,1,1,1,0,0,0,0,0,0,0,0,0,0,0,1,1,1,0,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,0,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,117,1,0,"Small molecule transport > ABC transporters","Small molecule transport "," ABC transporters"]
["hsa:2109",2109,"ETFB","MADD",19,"electron-transfer-flavoprotein, beta polypeptide","intracellular membrane-bounded organelle | mitochondrion | mitochondrial matrix","electron carrier activity","transport | respiratory electron transport chain","ETFB","electron-transfer-flavoprotein, beta polypeptide","MitoCarta3.0","Metabolism > Lipid metabolism > Fatty acid oxidation | Metabolism > Amino acid metabolism > Branched-chain amino acid metabolism | Metabolism > Amino acid metabolism > Lysine metabolism | Metabolism > Amino acid metabolism > Glycine metabolism | Metabolism > Vitamin metabolism > Choline and betaine metabolism | Metabolism > Electron carriers > Q-linked reactions, other","Matrix",0,"ETF",null,null,null,null,"FirstInEnsemblFamily","Glutaric acidemia IIA","ETFB",1,0,0,0,0,0,1,1,1,1,1,1,0,0,0,0,0,0,0,0,0,0,0,1,1,1,1,1,1,1,0,0,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,0,1,1,1,1,1,1,1,117,1,1,"Metabolism > Lipid metabolism > Fatty acid oxidation | Metabolism > Amino acid metabolism > Branched-chain amino acid metabolism | Metabolism > Amino acid metabolism > Lysine metabolism | Metabolism > Amino acid metabolism > Glycine metabolism | Metabolism > Vitamin metabolism > Choline and betaine metabolism | Metabolism > Electron carriers > Q-linked reactions, other","Metabolism "," Lipid metabolism "," Fatty acid oxidation "," Metabolism "," Amino acid metabolism "," Branched-chain amino acid metabolism "," Metabolism "," Amino acid metabolism "," Lysine metabolism "," Metabolism "," Amino acid metabolism "," Glycine metabolism "," Metabolism "," Vitamin metabolism "," Choline and betaine metabolism "]
["hsa:4358",4358,"MPV17","MTDPS6|SYM1",2,"MpV17 mitochondrial inner membrane protein","mitochondrion | integral to membrane | mitochondrial inner membrane | membrane | integral to peroxisomal membrane","molecular_function","glomerular basement membrane development | inner ear development | homeostatic process | cellular response to reactive oxygen species | mitochondrial genome maintenance | regulation of reactive oxygen species metabolic process","MPV17","MpV17 mitochondrial inner membrane protein","MitoCarta3.0","Small molecule transport","MIM",2,"Mpv17_PMP22","Peroxisome",null,null,null,"FirstInEnsemblFamily","Mitochondrial DNA depletion syndrome 6 (hepatocerebral type)","MPV17",0,0,0,0,0,0,0,0,0,0,0,1,0,0,0,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,0,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,117,1,1,"Small molecule transport","Small molecule transport"]
["hsa:51004",51004,"COQ6","FLJ51173|FLJ53037|FLJ58904|FLJ79255",14,"coenzyme Q6 homolog, monooxygenase (S. cerevisiae)","mitochondrion","oxidoreductase activity, acting on paired donors, with incorporation or reduction of molecular oxygen, NADH or NADPH as one donor, and incorporation of one atom of oxygen | flavin adenine dinucleotide binding","ubiquinone biosynthetic process","COQ6","coenzyme Q6 homolog, monooxygenase (S. cerevisiae)","MitoCarta3.0","Metabolism > Metals and cofactors > Coenzyme Q metabolism","MIM",0,"FAD_binding_3","Ubiquinone and other terpenoid-quinone biosynthesis",null,null,null,"FirstInEnsemblFamily","Coenzyme Q10 deficiency, primary, 6","COQ6",1,0,0,0,0,0,1,1,1,1,1,0,1,1,0,0,0,0,0,0,0,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,0,0,1,0,1,1,1,1,1,1,0,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,116,1,1,"Metabolism > Metals and cofactors > Coenzyme Q metabolism","Metabolism "," Metals and cofactors "," Coenzyme Q metabolism"]
["hsa:5498",5498,"PPOX","MGC8485|PPO|V290M|VP",1,"protoporphyrinogen oxidase","intrinsic to mitochondrial inner membrane | mitochondrion | mitochondrial membrane | membrane | integral to mitochondrial inner membrane | mitochondrial intermembrane space","oxygen-dependent protoporphyrinogen oxidase activity | oxidoreductase activity | flavin adenine dinucleotide binding | nucleotide binding","oxidation-reduction process | protoporphyrinogen IX metabolic process | porphyrin-containing compound metabolic process | heme biosynthetic process | porphyrin-containing compound biosynthetic process | response to drug","PPOX","protoporphyrinogen oxidase","MitoCarta3.0","Metabolism > Metals and cofactors > Heme synthesis and processing","MIM",0,"DAO | Amino_oxidase","Porphyrin and chlorophyll metabolism",null,null,null,"FirstInEnsemblFamily","Porphyria variegata","PPOX",1,0,0,0,0,0,0,0,0,0,0,1,0,0,0,0,0,0,0,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,0,1,1,1,1,1,1,1,116,1,1,"Metabolism > Metals and cofactors > Heme synthesis and processing","Metabolism "," Metals and cofactors "," Heme synthesis and processing"]
["hsa:26519",26519,"TIMM10","TIM10|TIM10A",11,"translocase of inner mitochondrial membrane 10 homolog (yeast)","mitochondrion | mitochondrial inner membrane | mitochondrial intermembrane space protein transporter complex | membrane | mitochondrial inner membrane presequence translocase complex | mitochondrial intermembrane space","protein binding | transporter activity | chaperone binding | protein homodimerization activity | zinc ion binding | metal ion binding","chaperone-mediated protein transport | protein import into mitochondrial inner membrane | protein targeting to mitochondrion | sensory perception of sound","TIMM10","translocase of inner mitochondrial membrane 10 homolog (yeast)","MitoCarta3.0","Protein import, sorting and homeostasis > Protein homeostasis > Chaperones | Protein import, sorting and homeostasis > Protein import and sorting","MIM",0,"zf-Tim10_DDP",null,null,null,null,"FirstInEnsemblFamily",null,"TIMM10",0,0,0,0,0,0,0,0,0,0,0,1,0,0,1,1,1,1,1,1,1,1,1,0,0,1,1,1,1,0,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,116,1,1,"Protein import, sorting and homeostasis > Protein homeostasis > Chaperones | Protein import, sorting and homeostasis > Protein import and sorting","Protein import, sorting and homeostasis "," Protein homeostasis "," Chaperones "," Protein import, sorting and homeostasis "," Protein import and sorting"]
["hsa:55149",55149,"MTPAP","FLJ10486|PAPD1|SPAX4",10,"mitochondrial poly(A) polymerase","mitochondrion | cytoplasm","UTP binding | magnesium ion binding | transferase activity | nucleotide binding | ATP binding | manganese ion binding | polynucleotide adenylyltransferase activity | RNA binding | protein homodimerization activity | identical protein binding | nucleotidyltransferase activity","cell death | mRNA processing | mRNA polyadenylation | transcription, DNA-dependent | histone mRNA catabolic process","PAPD1","PAP associated domain containing 1","MitoCarta3.0","Mitochondrial central dogma > mtRNA metabolism > mtRNA granules | Mitochondrial central dogma > mtRNA metabolism > mt-rRNA modifications | Mitochondrial central dogma > mtRNA metabolism > mt-mRNA modifications | Mitochondrial central dogma > mtRNA metabolism > mtRNA stability and decay","Matrix",0,"PAP_assoc",null,null,null,null,"FirstInEnsemblFamily","Ataxia, spastic, 4","MTPAP",0,0,1,1,1,0,1,1,1,1,1,1,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,0,0,0,0,0,0,0,0,0,0,0,0,1,0,0,0,0,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,115,1,0,"Mitochondrial central dogma > mtRNA metabolism > mtRNA granules | Mitochondrial central dogma > mtRNA metabolism > mt-rRNA modifications | Mitochondrial central dogma > mtRNA metabolism > mt-mRNA modifications | Mitochondrial central dogma > mtRNA metabolism > mtRNA stability and decay","Mitochondrial central dogma "," mtRNA metabolism "," mtRNA granules "," Mitochondrial central dogma "," mtRNA metabolism "," mt-rRNA modifications "," Mitochondrial central dogma "," mtRNA metabolism "," mt-mRNA modifications "," Mitochondrial central dogma "," mtRNA metabolism "," mtRNA stability and decay"]
["hsa:26521",26521,"TIMM8B","DDP2|FLJ21744|MGC102866|MGC117373|TIM8B",11,"translocase of inner mitochondrial membrane 8 homolog B (yeast)","mitochondrion | mitochondrial inner membrane | mitochondrial intermembrane space protein transporter complex | membrane","zinc ion binding | metal ion binding","chaperone-mediated protein transport | protein import into mitochondrial inner membrane | protein targeting to mitochondrion | sensory perception of sound","TIMM8B","translocase of inner mitochondrial membrane 8 homolog B (yeast)","MitoCarta3.0","Protein import, sorting and homeostasis > Protein homeostasis > Chaperones | Protein import, sorting and homeostasis > Protein import and sorting","MIM",0,"zf-Tim10_DDP",null,null,null,null,"FirstInEnsemblFamily",null,"TIMM8B",0,0,0,0,0,0,0,0,0,0,0,1,0,0,1,1,1,0,0,1,1,1,1,0,0,1,1,0,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,0,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,115,1,1,"Protein import, sorting and homeostasis > Protein homeostasis > Chaperones | Protein import, sorting and homeostasis > Protein import and sorting","Protein import, sorting and homeostasis "," Protein homeostasis "," Chaperones "," Protein import, sorting and homeostasis "," Protein import and sorting"]
["hsa:37",37,"ACADVL","ACAD6|LCACD|VLCAD",17,"acyl-CoA dehydrogenase, very long chain","mitochondrial nucleoid | mitochondrion | mitochondrial inner membrane | membrane | mitochondrial matrix","acyl-CoA dehydrogenase activity | long-chain-acyl-CoA dehydrogenase activity | fatty-acyl-CoA binding | flavin adenine dinucleotide binding | very long-chain-acyl-CoA dehydrogenase activity","regulation of cholesterol metabolic process | fatty acid beta-oxidation using acyl-CoA dehydrogenase | fatty acid beta-oxidation | cellular lipid metabolic process | very long-chain fatty acid catabolic process | negative regulation of fatty acid oxidation | fatty acid metabolic process | energy derivation by oxidation of organic compounds | negative regulation of fatty acid biosynthetic process | temperature homeostasis","ACADVL","acyl-Coenzyme A dehydrogenase, very long chain","MitoCarta3.0","Metabolism > Lipid metabolism > Fatty acid oxidation","MIM",0,"Acyl-CoA_dh_1 | Acyl-CoA_dh_N | Acyl-CoA_dh_M | Acyl-CoA_dh_2","Fatty acid metabolism","VERY LONG CHAIN SPECIFIC ACYL COA DEHYDROGENASE MITOCHONDRIAL PRECURSOR EC_1.3.99.-","ENSFM00500000270065",2,"FirstInEnsemblFamily","VLCAD deficiency","ACADVL",1,0,0,0,0,0,1,1,1,1,1,1,0,0,0,0,0,0,0,0,0,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,0,0,1,0,0,0,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,114,1,0,"Metabolism > Lipid metabolism > Fatty acid oxidation","Metabolism "," Lipid metabolism "," Fatty acid oxidation"]
["hsa:51409",51409,"HEMK1","FLJ22320|HEMK|MTQ1",3,"HemK methyltransferase family member 1","mitochondrion","protein methyltransferase activity | N-methyltransferase activity | transferase activity | DNA binding","DNA methylation","HEMK1","HemK methyltransferase family member 1","MitoCarta3.0","Mitochondrial central dogma > Translation > Translation factors","Matrix",0,"MTS","Histidine metabolism | Tyrosine metabolism",null,null,null,"FirstInEnsemblFamily",null,"HEMK1",1,0,0,0,0,0,1,1,1,1,1,0,1,1,1,0,0,1,0,1,0,1,1,0,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,0,1,0,1,1,1,1,0,0,1,0,0,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,0,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,0,1,1,1,1,1,1,1,1,1,1,1,113,1,1,"Mitochondrial central dogma > Translation > Translation factors","Mitochondrial central dogma "," Translation "," Translation factors"]
["hsa:387787",387787,"LIPT2","-",11,"lipoyl(octanoyl) transferase 2 (putative)","mitochondrion | cytoplasm","octanoyltransferase activity | transferase activity, transferring acyl groups | lipoyl(octanoyl) transferase activity | ligase activity","protein modification process | lipoate biosynthetic process","LOC387787","similar to CG9804-PA","MitoCarta3.0","Metabolism > Lipid metabolism > Lipoate insertion","Matrix",0,"BPL_LipA_LipB","Lipoic acid metabolism",null,null,null,"FirstInEnsemblFamily",null,"LIPT2",1,0,0,0,0,0,1,1,1,1,1,1,0,0,0,0,0,1,1,1,0,0,1,0,0,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,0,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,0,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,0,0,1,1,1,1,1,1,1,113,1,1,"Metabolism > Lipid metabolism > Lipoate insertion","Metabolism "," Lipid metabolism "," Lipoate insertion"]
["hsa:80724",80724,"ACAD10","MGC5601",12,"acyl-CoA dehydrogenase family, member 10",null,"hydrolase activity | acyl-CoA dehydrogenase activity | flavin adenine dinucleotide binding | transferase activity, transferring phosphorus-containing groups",null,"ACAD10","acyl-Coenzyme A dehydrogenase family, member 10","MitoCarta3.0","Metabolism > Lipid metabolism > Fatty acid oxidation","Matrix",0,"Acyl-CoA_dh_1 | APH | Hydrolase | Acyl-CoA_dh_M | Acyl-CoA_dh_2",null,"ACYL COA DEHYDROGENASE FAMILY MEMBER ACAD EC_1.3.99.-","ENSFM00250000002241",2,null,null,"ACAD10",1,0,0,0,1,0,1,1,1,1,1,1,0,0,0,0,0,0,0,0,0,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,0,1,1,1,1,1,1,1,1,1,0,0,0,0,0,0,0,0,0,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,111,1,0,"Metabolism > Lipid metabolism > Fatty acid oxidation","Metabolism "," Lipid metabolism "," Fatty acid oxidation"]
["hsa:4729",4729,"NDUFV2","CI-24k",18,"NADH dehydrogenase (ubiquinone) flavoprotein 2, 24kDa","mitochondrion | respiratory chain | mitochondrial inner membrane | mitochondrial respiratory chain complex I | membrane","electron carrier activity | NADH dehydrogenase activity | oxidoreductase activity | NAD binding | 2 iron, 2 sulfur cluster binding | NADH dehydrogenase (ubiquinone) activity | metal ion binding","transport | mitochondrial electron transport, NADH to ubiquinone | cardiac muscle tissue development | respiratory electron transport chain | nervous system development","NDUFV2","NADH dehydrogenase (ubiquinone) flavoprotein 2, 24kDa","MitoCarta3.0","OXPHOS > Complex I > CI subunits | Metabolism > Metals and cofactors > Fe-S-containing proteins | OXPHOS > OXPHOS subunits","MIM",0,"Complex1_24kDa","Oxidative phosphorylation",null,null,null,"FirstInEnsemblFamily",null,"NDUFV2",1,0,0,0,0,1,1,1,1,1,1,0,0,0,0,0,0,0,0,0,0,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,0,0,0,0,0,0,0,0,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,111,1,1,"OXPHOS > Complex I > CI subunits | Metabolism > Metals and cofactors > Fe-S-containing proteins | OXPHOS > OXPHOS subunits","OXPHOS "," Complex I "," CI subunits "," Metabolism "," Metals and cofactors "," Fe-S-containing proteins "," OXPHOS "," OXPHOS subunits"]
["hsa:84129",84129,"ACAD11","ACAD-11|FLJ12592|MGC150619",3,"acyl-CoA dehydrogenase family, member 11","mitochondrion | peroxisome","acyl-CoA dehydrogenase activity | flavin adenine dinucleotide binding | transferase activity, transferring phosphorus-containing groups",null,"ACAD11","acyl-Coenzyme A dehydrogenase family, member 11","MitoCarta3.0","Metabolism > Lipid metabolism > Fatty acid oxidation","MIM",0,"Acyl-CoA_dh_1 | APH | Acyl-CoA_dh_M | Acyl-CoA_dh_2",null,"ACYL COA DEHYDROGENASE FAMILY MEMBER ACAD EC_1.3.99.-","ENSFM00250000002241",2,"FirstInEnsemblFamily",null,"ACAD11",1,0,0,0,0,0,1,1,1,1,1,1,0,0,0,0,0,0,0,0,0,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,0,1,1,1,1,1,1,1,1,1,0,0,0,0,0,0,0,0,0,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,110,1,0,"Metabolism > Lipid metabolism > Fatty acid oxidation","Metabolism "," Lipid metabolism "," Fatty acid oxidation"]
["hsa:3712",3712,"IVD","ACAD2|FLJ12715|FLJ34849",15,"isovaleryl-CoA dehydrogenase","mitochondrion | mitochondrial membrane | mitochondrial matrix","isovaleryl-CoA dehydrogenase activity | flavin adenine dinucleotide binding","branched chain family amino acid catabolic process | cellular nitrogen compound metabolic process | leucine catabolic process","IVD","isovaleryl Coenzyme A dehydrogenase","MitoCarta3.0","Metabolism > Amino acid metabolism > Branched-chain amino acid metabolism","Matrix",0,"Acyl-CoA_dh_1 | Acyl-CoA_dh_N | Acyl-CoA_dh_M | Acyl-CoA_dh_2","Valine, leucine and isoleucine degradation",null,null,null,"FirstInEnsemblFamily","Isovaleric acidemia","IVD",1,0,0,0,0,0,1,1,1,1,1,1,0,0,0,0,0,0,0,0,0,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,0,1,1,1,1,1,1,1,1,0,0,0,0,0,0,0,0,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,110,1,1,"Metabolism > Amino acid metabolism > Branched-chain amino acid metabolism","Metabolism "," Amino acid metabolism "," Branched-chain amino acid metabolism"]
["hsa:8564",8564,"KMO","dJ317G22.1",1,"kynurenine 3-monooxygenase (kynurenine 3-hydroxylase)","cytosol | mitochondrial outer membrane | mitochondrion | integral to membrane | mitochondrial inner membrane | membrane","NAD(P)H oxidase activity | kynurenine 3-monooxygenase activity | electron carrier activity | monooxygenase activity | flavin adenine dinucleotide binding","tryptophan catabolic process to kynurenine | response to salt stress | kynurenine metabolic process | NAD metabolic process | pyridine nucleotide biosynthetic process | tryptophan catabolic process | cellular nitrogen compound metabolic process","KMO","kynurenine 3-monooxygenase (kynurenine 3-hydroxylase)","MitoCarta3.0","Metabolism > Amino acid metabolism > Kynurenine metabolism | Metabolism > Metals and cofactors > NAD biosynthesis and metabolism","MOM",2,"FAD_binding_3 | DAO","Tryptophan metabolism",null,null,null,"FirstInEnsemblFamily",null,"KMO",1,0,0,0,0,0,1,1,1,1,0,0,1,1,0,0,0,0,0,0,0,0,0,0,1,0,1,1,1,1,1,1,1,1,1,1,1,0,1,0,1,1,0,1,0,1,1,1,1,1,1,0,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,0,1,1,1,1,1,1,1,1,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,110,1,1,"Metabolism > Amino acid metabolism > Kynurenine metabolism | Metabolism > Metals and cofactors > NAD biosynthesis and metabolism","Metabolism "," Amino acid metabolism "," Kynurenine metabolism "," Metabolism "," Metals and cofactors "," NAD biosynthesis and metabolism"]
["hsa:847",847,"CAT","MGC138422|MGC138424",11,"catalase","Golgi apparatus | endoplasmic reticulum | peroxisome | mitochondrial intermembrane space | cytosol | peroxisomal matrix | peroxisomal membrane | plasma membrane | lysosome","catalase activity | NADP binding | protein homodimerization activity | oxidoreductase activity, acting on peroxide as acceptor | heme binding | metal ion binding","positive regulation of cell division | response to reactive oxygen species | nucleobase-containing small molecule metabolic process | UV protection | aging | purine base metabolic process | purine nucleotide catabolic process | response to hypoxia | response to hyperoxia | hydrogen peroxide catabolic process | menopause | protein tetramerization | negative regulation of apoptosis | cellular response to growth factor stimulus | response to vitamin E","CAT","catalase","MitoCarta3.0","Metabolism > Detoxification > ROS and glutathione metabolism","IMS",0,"Catalase","Tryptophan metabolism | Peroxisome",null,null,null,"FirstInEnsemblFamily","Acatalasemia","CAT",1,0,0,0,0,0,0,0,0,0,0,1,0,0,0,0,0,0,0,0,0,0,0,1,1,1,0,1,1,1,0,0,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,0,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,109,1,1,"Metabolism > Detoxification > ROS and glutathione metabolism","Metabolism "," Detoxification "," ROS and glutathione metabolism"]
["hsa:28992",28992,"MACROD1","LRP16",11,"MACRO domain containing 1","mitochondrion","deacetylase activity","purine nucleoside metabolic process","LRP16","LRP16 protein","MitoCarta3.0","Signaling","Matrix",0,"A1pp",null,"MACRO DOMAIN CONTAINING","ENSFM00500000270740",2,"FirstInEnsemblFamily",null,"MACROD1",1,0,1,1,1,1,1,1,0,0,0,1,0,0,1,0,0,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,0,1,1,1,0,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,0,0,0,0,0,1,0,1,1,1,0,0,0,0,0,0,0,0,0,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,109,1,1,"Signaling","Signaling"]
["hsa:27429",27429,"HTRA2","OMI|PARK13|PRSS25",2,"HtrA serine peptidase 2","CD40 receptor complex | mitochondrion | endoplasmic reticulum | internal side of plasma membrane | mitochondrial intermembrane space | cytosol | integral to membrane | nucleus | mitochondrial membrane | endoplasmic reticulum membrane | membrane","unfolded protein binding | peptidase activity | protein binding | serine-type endopeptidase activity","neuron development | positive regulation of apoptosis | mitochondrion organization | adult walking behavior | response to stress | proteolysis | cell death | induction of apoptosis by intracellular signals | forebrain development | regulation of multicellular organism growth","HTRA2","HtrA serine peptidase 2","MitoCarta3.0","Protein import, sorting and homeostasis > Protein homeostasis > Proteases | Mitochondrial dynamics and surveillance > Apoptosis","MIM",1,"Trypsin | PDZ",null,"SERINE PROTEASE HTRA2 MITOCHONDRIAL PRECURSOR EC_3.4.21.108 HIGH TEMPERATURE REQUIREMENT A2","ENSFM00250000001030",4,"FirstInEnsemblFamily","Parkinson disease 13","HTRA2",1,0,0,0,0,0,0,0,0,0,0,1,0,0,1,1,1,1,1,1,0,1,1,0,0,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,0,0,0,0,0,0,1,0,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,0,1,1,1,0,0,0,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,108,1,0,"Protein import, sorting and homeostasis > Protein homeostasis > Proteases | Mitochondrial dynamics and surveillance > Apoptosis","Protein import, sorting and homeostasis "," Protein homeostasis "," Proteases "," Mitochondrial dynamics and surveillance "," Apoptosis"]
["hsa:1962",1962,"EHHADH","ECHD|L-PBE|LBFP|LBP|MGC120586|PBFE",3,"enoyl-CoA, hydratase/3-hydroxyacyl CoA dehydrogenase","mitochondrion | peroxisome","isomerase activity | coenzyme binding | lyase activity | oxidoreductase activity | enoyl-CoA hydratase activity | dodecenoyl-CoA delta-isomerase activity | 3-hydroxyacyl-CoA dehydrogenase activity | nucleotide binding","internal protein amino acid acetylation | fatty acid metabolic process | lipid metabolic process","EHHADH","enoyl-Coenzyme A, hydratase/3-hydroxyacyl Coenzyme A dehydrogenase","MitoCarta3.0","Metabolism > Lipid metabolism","unknown",0,"ECH | 3HCDH_N | 3HCDH",null,"PEROXISOMAL BIFUNCTIONAL ENZYME PBE PBFE [INCLUDES ENOYL COA HYDRATASE/3 2 TRANS ENOYL COA ISOMERASE EC_4.2.1.17 EC_5.3.3.- 8 ; 3 HYDROXYACYL COA DEHYDROGENASE EC_1.1.-.- 1 35 ]","ENSFM00640001103061",2,"FirstInEnsemblFamily",null,"EHHADH",1,0,0,0,0,0,1,1,1,1,1,1,0,0,0,0,0,0,1,0,0,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,0,0,0,0,0,0,0,0,0,0,0,0,1,0,0,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,107,1,1,"Metabolism > Lipid metabolism","Metabolism "," Lipid metabolism"]
["hsa:7417",7417,"VDAC2","FLJ23841|POR",10,"voltage-dependent anion channel 2","pore complex | mitochondrial nucleoid | mitochondrial outer membrane | mitochondrion | integral to membrane | membrane","voltage-gated anion channel activity | porin activity | nucleotide binding","regulation of anion transport | anion transport | transmembrane transport","VDAC2","voltage-dependent anion channel 2","MitoCarta3.0","Small molecule transport","MOM",0,"Porin_3",null,"VOLTAGE DEPENDENT ANION SELECTIVE CHANNEL VDAC OUTER MITOCHONDRIAL MEMBRANE PORIN","ENSFM00250000001189",3,"FirstInEnsemblFamily",null,"VDAC2",0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,1,0,1,0,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,107,1,1,"Small molecule transport","Small molecule transport"]
["hsa:79922",79922,"MRM1","FLJ22578",17,"mitochondrial rRNA methyltransferase 1 homolog (S. cerevisiae)","mitochondrion","RNA binding | transferase activity | RNA methyltransferase activity","RNA processing","MRM1","mitochondrial rRNA methyltransferase 1 homolog (S. cerevisiae)","MitoCarta3.0","Mitochondrial central dogma > mtRNA metabolism > mtRNA granules | Mitochondrial central dogma > mtRNA metabolism > mt-rRNA modifications","Matrix",0,"SpoU_methylase | SpoU_sub_bind",null,null,null,null,"FirstInEnsemblFamily",null,"MRM1",1,0,0,0,0,0,1,1,1,1,1,0,1,1,0,0,0,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,0,0,0,0,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,0,0,1,1,1,0,0,0,0,0,0,0,0,1,0,1,1,0,1,1,1,1,0,1,1,0,1,0,1,1,1,1,1,1,1,106,1,1,"Mitochondrial central dogma > mtRNA metabolism > mtRNA granules | Mitochondrial central dogma > mtRNA metabolism > mt-rRNA modifications","Mitochondrial central dogma "," mtRNA metabolism "," mtRNA granules "," Mitochondrial central dogma "," mtRNA metabolism "," mt-rRNA modifications"]
["hsa:55163",55163,"PNPO","FLJ10535|PDXPO",17,"pyridoxamine 5'-phosphate oxidase","cytosol","oxidoreductase activity, acting on the CH-NH2 group of donors | FMN binding | pyridoxamine-phosphate oxidase activity","pyridoxine biosynthetic process | pyridoxal phosphate biosynthetic process | vitamin B6 metabolic process | water-soluble vitamin metabolic process | vitamin metabolic process","PNPO","pyridoxamine 5'-phosphate oxidase","MitoCarta3.0","Metabolism > Vitamin metabolism > Vitamin B6 metabolism","Matrix",0,"Pyridox_oxidase","Vitamin B6 metabolism",null,null,null,"FirstInEnsemblFamily","Pyridoxamine 5'-phosphate oxidase deficiency","PNPO",1,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,1,0,0,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,106,1,1,"Metabolism > Vitamin metabolism > Vitamin B6 metabolism","Metabolism "," Vitamin metabolism "," Vitamin B6 metabolism"]
["hsa:25996",25996,"REXO2","DKFZp566E144|MGC111570|REX2|RFN|SFN",11,"REX2, RNA exonuclease 2 homolog (S. cerevisiae)","mitochondrion | nucleus | intracellular","3'-5' exonuclease activity | nucleic acid binding","nucleobase-containing compound metabolic process | nucleotide metabolic process","REXO2","REX2, RNA exonuclease 2 homolog (S. cerevisiae)","MitoCarta3.0","Mitochondrial central dogma > mtRNA metabolism > mtRNA stability and decay","Matrix",0,"Exonuc_X-T","Ribosome biogenesis in eukaryotes",null,null,null,"FirstInEnsemblFamily",null,"REXO2",1,0,0,0,0,0,0,0,0,0,0,1,0,0,0,0,0,0,0,0,0,0,0,1,1,0,0,0,1,1,0,0,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,0,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,106,1,1,"Mitochondrial central dogma > mtRNA metabolism > mtRNA stability and decay","Mitochondrial central dogma "," mtRNA metabolism "," mtRNA stability and decay"]
["hsa:4513",4513,"COX2","COII|MTCO2","MT","cytochrome c oxidase subunit II","mitochondrion | respiratory chain | integral to membrane | mitochondrial inner membrane | membrane","electron carrier activity | oxidoreductase activity | cytochrome-c oxidase activity | copper ion binding | heme binding | metal ion binding","mitochondrial electron transport, cytochrome c to oxygen | transport | response to cold | lactation | respiratory electron transport chain","COX2","cytochrome c oxidase subunit II","MitoCarta3.0","OXPHOS > Complex IV > CIV subunits | OXPHOS > OXPHOS subunits","MIM",2,"COX2_TM | COX2","Oxidative phosphorylation",null,null,null,"FirstInEnsemblFamily","Mitochondrial complex IV deficiency","COX2",1,0,0,0,0,0,1,1,0,1,0,1,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,0,1,1,1,0,0,1,1,1,1,1,1,0,1,1,1,0,1,1,0,1,1,0,1,1,1,1,0,1,1,1,0,0,0,0,1,1,1,1,1,1,1,1,1,1,0,1,0,0,1,1,1,0,1,1,0,1,0,1,1,0,0,1,0,0,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,105,1,1,"OXPHOS > Complex IV > CIV subunits | OXPHOS > OXPHOS subunits","OXPHOS "," Complex IV "," CIV subunits "," OXPHOS "," OXPHOS subunits"]
["hsa:100287932",100287932,"TIMM23","FLJ40725|FLJ56773|FLJ57459|FLJ79448|MGC71995|MGC87383|TIM23",10,"translocase of inner mitochondrial membrane 23 homolog (yeast)","mitochondrion | integral to membrane | mitochondrial inner membrane | membrane | mitochondrial inner membrane presequence translocase complex | integral to mitochondrial inner membrane | mitochondrial intermembrane space","protein binding | P-P-bond-hydrolysis-driven protein transmembrane transporter activity","intracellular protein transport | protein targeting to mitochondrion",null,null,"MitoCarta3.0","Protein import, sorting and homeostasis > Protein import and sorting > TIM23 presequence pathway","MIM",null,null,null,null,null,null,"FirstInEnsemblFamily",null,"TIMM23",0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,1,0,0,0,0,0,0,0,0,0,0,1,1,0,1,1,0,0,1,1,1,0,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,0,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,105,1,0,"Protein import, sorting and homeostasis > Protein import and sorting > TIM23 presequence pathway","Protein import, sorting and homeostasis "," Protein import and sorting "," TIM23 presequence pathway"]
["hsa:6901",6901,"TAZ","BTHS|CMD3A|EFE|EFE2|FLJ27390|G4.5|LVNCX|Taz1|XAP-2","X","tafazzin","mitochondrion | integral to membrane | membrane | cytoplasm","1-acylglycerophosphocholine O-acyltransferase activity | transferase activity, transferring acyl groups","cardiac muscle contraction | mitochondrial respiratory chain complex I assembly | cardiolipin biosynthetic process | cristae formation | mitochondrial ATP synthesis coupled electron transport | heart development | skeletal muscle tissue development | hemopoiesis | muscle contraction | metabolic process | cardiac muscle tissue development","TAZ","tafazzin (cardiomyopathy, dilated 3A (X-linked); endocardial fibroelastosis 2; Barth syndrome)","MitoCarta3.0","Metabolism > Lipid metabolism > Cardiolipin synthesis","IMS",1,"Acyltransferase","Glycerophospholipid metabolism",null,null,null,"FirstInEnsemblFamily","Barth syndrome","TAZ",1,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,0,1,1,1,0,1,1,1,1,0,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,0,0,1,1,0,1,1,1,1,104,1,1,"Metabolism > Lipid metabolism > Cardiolipin synthesis","Metabolism "," Lipid metabolism "," Cardiolipin synthesis"]
["hsa:125965",125965,"COX6B2","COXVIB2|CT59|FLJ46422|MGC119094",19,"cytochrome c oxidase subunit VIb polypeptide 2 (testis)","mitochondrion | mitochondrial crista | mitochondrial intermembrane space","cytochrome-c oxidase activity",null,"COX6B2","cytochrome c oxidase subunit VIb polypeptide 2 (testis)","MitoCarta3.0","OXPHOS > Complex IV > CIV subunits | OXPHOS > OXPHOS subunits","MIM",0,"COX6B","Oxidative phosphorylation",null,null,null,"FirstInEnsemblFamily",null,"COX6B2",0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,0,1,0,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,0,1,1,1,1,1,0,0,1,1,1,1,1,1,1,103,1,0,"OXPHOS > Complex IV > CIV subunits | OXPHOS > OXPHOS subunits","OXPHOS "," Complex IV "," CIV subunits "," OXPHOS "," OXPHOS subunits"]
["hsa:25961",25961,"NUDT13","-",10,"nudix (nucleoside diphosphate linked moiety X)-type motif 13","mitochondrion","hydrolase activity | metal ion binding",null,"NUDT13","nudix (nucleoside diphosphate linked moiety X)-type motif 13","MitoCarta3.0","Metabolism > Metals and cofactors > NAD biosynthesis and metabolism","Matrix",0,"NUDIX-like | zf-NADH-PPase | NUDIX",null,null,null,null,"FirstInEnsemblFamily",null,"NUDT13",1,0,1,1,0,1,0,0,1,1,1,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,0,0,1,1,1,0,1,0,0,0,0,0,0,0,0,0,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,103,1,0,"Metabolism > Metals and cofactors > NAD biosynthesis and metabolism","Metabolism "," Metals and cofactors "," NAD biosynthesis and metabolism"]
["hsa:5423",5423,"POLB","MGC125976",8,"polymerase (DNA directed), beta","spindle microtubule | nucleoplasm | microtubule | nucleus | cytoplasm","microtubule binding | damaged DNA binding | lyase activity | enzyme binding | transferase activity | protein binding | DNA-directed DNA polymerase activity | metal ion binding | nucleotidyltransferase activity","base-excision repair | response to DNA damage stimulus | pyrimidine dimer repair | DNA repair | base-excision repair, gap-filling | cell death | response to ethanol | DNA-dependent DNA replication | nucleotide-excision repair, DNA gap filling | anti-apoptosis","POLB","polymerase (DNA directed), beta","MitoCarta3.0","Mitochondrial central dogma > mtDNA maintenance > mtDNA repair","Matrix",0,null,"Base excision repair",null,null,null,"FirstInEnsemblFamily",null,"POLB",1,0,0,0,0,0,1,1,1,1,1,0,0,0,0,0,0,0,0,1,0,0,0,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,0,0,0,0,0,0,0,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,0,0,0,1,1,1,0,1,0,1,0,0,0,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,102,1,0,"Mitochondrial central dogma > mtDNA maintenance > mtDNA repair","Mitochondrial central dogma "," mtDNA maintenance "," mtDNA repair"]
["hsa:4700",4700,"NDUFA6","B14|CI-B14|LYRM6|NADHB14",22,"NADH dehydrogenase (ubiquinone) 1 alpha subcomplex, 6, 14kDa","mitochondrion | respiratory chain | mitochondrial inner membrane | mitochondrial membrane | mitochondrial respiratory chain complex I | membrane","NADH dehydrogenase (ubiquinone) activity","response to oxidative stress | transport | mitochondrial electron transport, NADH to ubiquinone | respiratory electron transport chain","NDUFA6","NADH dehydrogenase (ubiquinone) 1 alpha subcomplex, 6, 14kDa","MitoCarta3.0","OXPHOS > Complex I > CI subunits | OXPHOS > OXPHOS subunits","MIM",0,"Complex1_LYR","Oxidative phosphorylation",null,null,null,"FirstInEnsemblFamily",null,"NDUFA6",0,0,0,0,0,0,1,1,1,1,1,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,0,1,1,1,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,0,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,0,0,0,0,0,0,0,0,0,0,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,101,1,1,"OXPHOS > Complex I > CI subunits | OXPHOS > OXPHOS subunits","OXPHOS "," Complex I "," CI subunits "," OXPHOS "," OXPHOS subunits"]
["hsa:57380",57380,"MRS2","HPT|MGC78523|MRS2L",6,"MRS2 magnesium homeostasis factor homolog (S. cerevisiae)","mitochondrion | integral to membrane | mitochondrial inner membrane | membrane",null,"ion transport","MRS2L","MRS2-like, magnesium homeostasis factor (S. cerevisiae)","MitoCarta3.0","Small molecule transport | Signaling","MIM",2,"CorA",null,null,null,null,"FirstInEnsemblFamily",null,"MRS2",0,0,0,0,0,0,0,0,0,0,0,1,1,1,0,0,0,1,1,1,1,1,1,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,0,0,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,100,1,1,"Small molecule transport | Signaling","Small molecule transport ",null,null," Signaling"]
["hsa:55218",55218,"EXD2","C14orf114|DKFZp781A0133|DKFZp781L15100|EXDL2|FLJ10738",14,"exonuclease 3'-5' domain containing 2","intracellular","hydrolase activity | 3'-5' exonuclease activity | nucleic acid binding","nucleobase-containing compound metabolic process","EXDL2","exonuclease 3'-5' domain-like 2","MitoCarta3.0","Mitochondrial central dogma > Translation","Matrix",0,"3_5_exonuc",null,null,null,null,"FirstInEnsemblFamily",null,"EXD2",1,0,0,0,0,1,0,1,1,1,1,1,0,0,1,1,0,1,1,1,0,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,0,0,1,1,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,1,1,1,0,0,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,99,1,0,"Mitochondrial central dogma > Translation","Mitochondrial central dogma "," Translation"]
["hsa:10573",10573,"MRPL28","MAAT1|MGC8499|p15",16,"mitochondrial ribosomal protein L28","mitochondrion | ribosome | mitochondrial ribosome | cytoplasm","protein binding | structural constituent of ribosome","translation","MRPL28","mitochondrial ribosomal protein L28","MitoCarta3.0","Mitochondrial central dogma > Translation > Mitochondrial ribosome","Matrix",0,null,null,null,null,null,"FirstInEnsemblFamily",null,"MRPL28",0,0,0,0,0,0,0,0,0,0,0,1,0,0,1,1,1,1,1,1,1,1,0,1,1,1,0,1,1,1,1,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,0,1,0,1,0,1,1,1,1,1,0,0,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,0,0,0,0,0,0,0,1,1,1,0,0,1,1,1,1,1,1,0,0,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,99,1,1,"Mitochondrial central dogma > Translation > Mitochondrial ribosome","Mitochondrial central dogma "," Translation "," Mitochondrial ribosome"]
["hsa:8192",8192,"CLPP","-",19,"ClpP caseinolytic peptidase, ATP-dependent, proteolytic subunit homolog (E. coli)","mitochondrion | mitochondrial matrix","peptidase activity | protein binding | nucleotide binding | ATP binding | serine-type endopeptidase activity","proteolysis","CLPP","ClpP caseinolytic peptidase, ATP-dependent, proteolytic subunit homolog (E. coli)","MitoCarta3.0","Protein import, sorting and homeostasis > Protein homeostasis > Proteases","Matrix",0,"CLP_protease",null,null,null,null,"FirstInEnsemblFamily",null,"CLPP",1,0,0,0,0,0,0,0,0,0,0,1,0,0,1,1,1,1,1,1,1,1,1,0,0,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,0,0,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,0,1,0,1,1,1,1,1,98,1,1,"Protein import, sorting and homeostasis > Protein homeostasis > Proteases","Protein import, sorting and homeostasis "," Protein homeostasis "," Proteases"]
["hsa:516",516,"ATP5G1","ATP5A|ATP5G",17,"ATP synthase, H+ transporting, mitochondrial Fo complex, subunit C1 (subunit 9)","proton-transporting ATP synthase complex, coupling factor F(o) | mitochondrion | integral to membrane | mitochondrial inner membrane | membrane | mitochondrial proton-transporting ATP synthase complex","hydrogen ion transmembrane transporter activity | lipid binding | transporter activity","transport | ATP hydrolysis coupled proton transport | ion transport | mitochondrial ATP synthesis coupled proton transport | respiratory electron transport chain","ATP5G1","ATP synthase, H+ transporting, mitochondrial F0 complex, subunit C1 (subunit 9)","MitoCarta3.0","OXPHOS > Complex V > CV subunits | OXPHOS > OXPHOS subunits","MIM",2,"ATP-synt_C","Oxidative phosphorylation",null,null,null,"FirstInEnsemblFamily",null,"ATP5G1",1,0,0,0,0,0,1,1,1,1,1,1,0,0,1,1,1,1,1,1,1,1,1,0,0,0,0,0,1,0,0,1,1,1,1,0,1,1,1,0,1,1,1,0,1,1,1,0,1,1,0,0,1,0,0,0,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,0,0,1,1,1,0,0,1,0,0,0,0,1,0,1,1,1,0,0,1,1,0,1,1,0,0,0,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,97,1,0,"OXPHOS > Complex V > CV subunits | OXPHOS > OXPHOS subunits","OXPHOS "," Complex V "," CV subunits "," OXPHOS "," OXPHOS subunits"]
["hsa:57128",57128,"LYRM4","C6orf149|CGI-203|ISD11",6,"LYR motif containing 4","mitochondrion | nucleus",null,null,"LYRM4","LYR motif containing 4","MitoCarta3.0","Metabolism > Metals and cofactors > Fe-S cluster biosynthesis","Matrix",0,"Complex1_LYR",null,null,null,null,"FirstInEnsemblFamily",null,"LYRM4",0,0,0,0,0,0,1,1,1,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,0,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,0,1,1,0,0,1,1,1,1,1,1,1,1,1,0,1,1,0,1,1,1,1,1,0,1,1,1,1,1,0,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,0,1,0,1,0,1,0,0,1,1,1,0,1,0,1,1,1,1,1,1,0,1,1,1,1,0,1,0,1,1,1,1,1,1,1,1,1,0,1,1,97,1,1,"Metabolism > Metals and cofactors > Fe-S cluster biosynthesis","Metabolism "," Metals and cofactors "," Fe-S cluster biosynthesis"]
["hsa:254552",254552,"NUDT8","FLJ41567",11,"nudix (nucleoside diphosphate linked moiety X)-type motif 8","mitochondrion","hydrolase activity | metal ion binding",null,"NUDT8","nudix (nucleoside diphosphate linked moiety X)-type motif 8","MitoCarta3.0","Metabolism > Metals and cofactors > Coenzyme A metabolism","Matrix",0,"NUDIX",null,null,null,null,"FirstInEnsemblFamily",null,"NUDT8",1,0,0,0,0,0,0,0,1,1,1,0,0,0,0,0,0,0,0,0,0,0,0,1,0,1,1,1,1,1,1,1,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,0,1,1,1,1,1,1,1,1,0,0,0,1,1,1,1,0,0,1,0,1,0,0,0,1,0,0,0,1,0,1,1,0,1,1,1,1,1,1,0,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,97,1,0,"Metabolism > Metals and cofactors > Coenzyme A metabolism","Metabolism "," Metals and cofactors "," Coenzyme A metabolism"]
["hsa:51079",51079,"NDUFA13","B16.6|CDA016|FLJ58045|FLJ59191|GRIM-19|GRIM19",19,"NADH dehydrogenase (ubiquinone) 1 alpha subcomplex, 13","nucleoplasm | mitochondrion | mitochondrial inner membrane | cytoplasm | mitochondrial respiratory chain | integral to membrane | nucleus | mitochondrial membrane | mitochondrial respiratory chain complex I | membrane","protein binding | NADH dehydrogenase activity | NADH dehydrogenase (ubiquinone) activity | ATP binding","transport | negative regulation of translation | reactive oxygen species metabolic process | negative regulation of transcription, DNA-dependent | apoptosis | apoptotic nuclear change | respiratory electron transport chain | induction of apoptosis | negative regulation of cell growth | protein import into nucleus | induction of apoptosis by extracellular signals","NDUFA13","NADH dehydrogenase (ubiquinone) 1 alpha subcomplex, 13","MitoCarta3.0","OXPHOS > Complex I > CI subunits | OXPHOS > OXPHOS subunits","MIM",1,"GRIM-19","Oxidative phosphorylation",null,null,null,"FirstInEnsemblFamily",null,"NDUFA13",0,0,0,0,0,0,1,1,1,1,1,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,0,1,1,1,1,1,1,1,1,1,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,0,1,0,1,0,1,0,1,0,0,0,0,0,0,0,0,0,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,0,0,1,1,1,1,1,1,1,96,1,1,"OXPHOS > Complex I > CI subunits | OXPHOS > OXPHOS subunits","OXPHOS "," Complex I "," CI subunits "," OXPHOS "," OXPHOS subunits"]
["hsa:51205",51205,"ACP6","ACPL1|LPAP|PACPL1",1,"acid phosphatase 6, lysophosphatidic","mitochondrion | extracellular region","hydrolase activity | acid phosphatase activity","lipid metabolic process","ACP6","acid phosphatase 6, lysophosphatidic","MitoCarta3.0","Metabolism > Lipid metabolism > Phospholipid metabolism","IMS",0,"Acid_phosphat_A","Riboflavin metabolism",null,null,null,"FirstInEnsemblFamily",null,"ACP6",0,0,1,1,1,1,1,1,1,1,1,1,1,1,0,0,0,0,0,0,0,0,0,1,1,1,1,1,1,1,1,0,0,1,1,1,0,0,0,0,0,0,0,0,1,0,0,1,1,1,1,0,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,0,1,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,94,1,0,"Metabolism > Lipid metabolism > Phospholipid metabolism","Metabolism "," Lipid metabolism "," Phospholipid metabolism"]
["hsa:90639",90639,"COX19","MGC104475",7,"COX19 cytochrome c oxidase assembly homolog (S. cerevisiae)","cytosol | cytoplasm",null,null,"COX19","COX19 cytochrome c oxidase assembly homolog (S. cerevisiae)","MitoCarta3.0","OXPHOS > Complex IV > CIV assembly factors | Metabolism > Metals and cofactors > Copper metabolism | OXPHOS > OXPHOS assembly factors","IMS",0,"CHCH",null,null,null,null,"FirstInEnsemblFamily",null,"COX19",0,0,0,0,0,0,1,1,1,1,1,1,0,0,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,0,1,1,1,0,1,1,1,1,0,1,1,1,1,0,1,1,0,0,1,1,1,1,1,1,1,1,1,0,0,0,1,1,1,1,0,1,1,1,0,0,1,0,1,0,1,1,0,1,0,1,1,1,1,1,1,1,0,1,1,0,1,1,0,1,1,0,1,0,1,0,1,1,1,0,0,1,0,1,0,0,1,1,0,1,1,0,1,0,0,1,0,1,0,1,1,1,1,0,1,0,1,1,0,1,1,94,1,1,"OXPHOS > Complex IV > CIV assembly factors | Metabolism > Metals and cofactors > Copper metabolism | OXPHOS > OXPHOS assembly factors","OXPHOS "," Complex IV "," CIV assembly factors "," Metabolism "," Metals and cofactors "," Copper metabolism "," OXPHOS "," OXPHOS assembly factors"]
["hsa:51056",51056,"LAP3","LAP|LAPEP|PEPS",4,"leucine aminopeptidase 3","mitochondrion | nucleolus | nucleus | intracellular | cytoplasm","manganese ion binding | peptidase activity | metalloexopeptidase activity | aminopeptidase activity | magnesium ion binding | zinc ion binding","proteolysis","LAP3","leucine aminopeptidase 3","MitoCarta3.0","Protein import, sorting and homeostasis > Protein homeostasis > Proteases","Matrix",0,"Peptidase_M17_N | Peptidase_M17","Glutathione metabolism | Arginine and proline metabolism",null,null,null,"FirstInEnsemblFamily",null,"LAP3",1,1,0,0,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,0,1,1,1,1,1,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,93,1,1,"Protein import, sorting and homeostasis > Protein homeostasis > Proteases","Protein import, sorting and homeostasis "," Protein homeostasis "," Proteases"]
["hsa:4720",4720,"NDUFS2","CI-49",1,"NADH dehydrogenase (ubiquinone) Fe-S protein 2, 49kDa (NADH-coenzyme Q reductase)","mitochondrion | respiratory chain | mitochondrial inner membrane | mitochondrial respiratory chain complex I | membrane","4 iron, 4 sulfur cluster binding | electron carrier activity | NADH dehydrogenase activity | protein binding | quinone binding | NAD binding | NADH dehydrogenase (ubiquinone) activity | metal ion binding","response to oxidative stress | transport | mitochondrial electron transport, NADH to ubiquinone | respiratory electron transport chain","NDUFS2","NADH dehydrogenase (ubiquinone) Fe-S protein 2, 49kDa (NADH-coenzyme Q reductase)","MitoCarta3.0","OXPHOS > Complex I > CI subunits | Metabolism > Metals and cofactors > Fe-S-containing proteins | OXPHOS > OXPHOS subunits","MIM",0,"Complex1_49kDa","Oxidative phosphorylation",null,null,null,"FirstInEnsemblFamily",null,"NDUFS2",1,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,0,0,0,1,0,0,1,1,1,1,1,1,1,0,0,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,0,0,0,0,0,0,0,0,0,0,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,0,1,1,1,1,1,1,1,93,1,1,"OXPHOS > Complex I > CI subunits | Metabolism > Metals and cofactors > Fe-S-containing proteins | OXPHOS > OXPHOS subunits","OXPHOS "," Complex I "," CI subunits "," Metabolism "," Metals and cofactors "," Fe-S-containing proteins "," OXPHOS "," OXPHOS subunits"]
["hsa:4538",4538,"ND4","MTND4","MT","NADH dehydrogenase, subunit 4 (complex I)","mitochondrion | respiratory chain | integral to membrane | mitochondrial inner membrane | mitochondrial respiratory chain complex I | membrane","oxidoreductase activity | NADH dehydrogenase (ubiquinone) activity","cerebellum development | transport | mitochondrial electron transport, NADH to ubiquinone | aging | embryo development | response to hypoxia | ATP synthesis coupled electron transport | respiratory electron transport chain","ND4","NADH dehydrogenase subunit 4","MitoCarta3.0","OXPHOS > Complex I > CI subunits | OXPHOS > OXPHOS subunits","MIM",10,"Oxidored_q1 | Oxidored_q5_N","Oxidative phosphorylation",null,null,null,"FirstInEnsemblFamily",null,"ND4",1,0,0,0,0,0,1,1,0,0,1,0,0,0,0,0,0,0,0,0,0,0,0,1,1,1,1,1,1,1,0,1,1,0,1,1,1,1,1,0,0,1,1,1,1,1,1,0,1,1,1,0,1,1,0,1,1,0,0,1,1,1,0,1,1,1,0,0,0,0,1,1,1,1,1,1,1,1,1,1,0,1,1,0,1,1,1,0,0,0,0,0,0,0,0,0,0,0,0,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,91,1,1,"OXPHOS > Complex I > CI subunits | OXPHOS > OXPHOS subunits","OXPHOS "," Complex I "," CI subunits "," OXPHOS "," OXPHOS subunits"]
["hsa:9801",9801,"MRPL19","KIAA0104|L19mt|MGC20675|MRP-L15|MRP-L19|MRPL15|RLX1|RPML15",2,"mitochondrial ribosomal protein L19","nuclear membrane | mitochondrion | ribosome | nucleus | intracellular","structural constituent of ribosome","translation","MRPL19","mitochondrial ribosomal protein L19","MitoCarta3.0","Mitochondrial central dogma > Translation > Mitochondrial ribosome","Matrix",0,null,null,null,null,null,"FirstInEnsemblFamily",null,"MRPL19",1,0,0,0,0,0,0,0,0,0,0,1,0,0,1,1,1,1,1,1,1,1,1,0,0,1,0,0,1,0,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,0,0,1,1,1,1,1,0,1,1,0,1,1,0,0,0,0,0,1,1,1,1,1,1,1,0,1,1,1,1,0,0,0,0,1,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,89,1,1,"Mitochondrial central dogma > Translation > Mitochondrial ribosome","Mitochondrial central dogma "," Translation "," Mitochondrial ribosome"]
["hsa:4535",4535,"ND1","MTND1","MT","NADH dehydrogenase, subunit 1 (complex I)","mitochondrion | respiratory chain | integral to membrane | mitochondrial inner membrane | mitochondrial membrane | mitochondrial respiratory chain complex I | membrane","protein binding | oxidoreductase activity | NADH dehydrogenase (ubiquinone) activity","transport | mitochondrial electron transport, NADH to ubiquinone | response to drug | response to organic cyclic compound | response to hydroperoxide | respiratory electron transport chain | response to stress","ND1","NADH dehydrogenase subunit 1","MitoCarta3.0","OXPHOS > Complex I > CI subunits | OXPHOS > OXPHOS subunits","MIM",8,"NADHdh","Oxidative phosphorylation",null,null,null,"FirstInEnsemblFamily",null,"ND1",1,0,0,0,0,0,1,1,0,1,0,0,0,0,0,0,0,0,0,0,1,0,0,1,1,1,1,1,1,1,0,1,1,0,1,1,1,1,1,0,0,1,1,1,1,1,1,0,1,1,1,0,1,1,0,1,1,0,0,1,1,1,0,1,1,1,0,0,0,0,1,1,1,0,1,1,1,1,1,1,0,1,0,0,1,1,1,0,0,0,0,0,0,0,0,0,0,0,0,1,1,1,0,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,88,1,1,"OXPHOS > Complex I > CI subunits | OXPHOS > OXPHOS subunits","OXPHOS "," Complex I "," CI subunits "," OXPHOS "," OXPHOS subunits"]
["hsa:4337",4337,"MOCS1","KIAA0381|MIG11|MOCOD",6,"molybdenum cofactor synthesis 1","cytosol | molybdopterin synthase complex | nucleus","4 iron, 4 sulfur cluster binding | GTP binding | catalytic activity | nucleotide binding | metal ion binding","Mo-molybdopterin cofactor biosynthetic process | water-soluble vitamin metabolic process | vitamin metabolic process | molybdopterin cofactor biosynthetic process","MOCS1","molybdenum cofactor synthesis 1","MitoCarta3.0","Metabolism > Metals and cofactors > Molybdenum cofactor synthesis and proteins","Matrix",0,"Mob_synth_C | MoaC | Radical_SAM","Folate biosynthesis | Sulfur relay system",null,null,null,"FirstInEnsemblFamily","Molybdenum cofactor deficiency, type A","MOCS1",1,0,0,0,0,0,0,0,0,0,0,1,0,0,0,0,0,0,0,0,0,0,0,0,0,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,0,1,1,1,1,1,0,1,0,1,0,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,1,1,1,1,0,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,87,1,1,"Metabolism > Metals and cofactors > Molybdenum cofactor synthesis and proteins","Metabolism "," Metals and cofactors "," Molybdenum cofactor synthesis and proteins"]
["hsa:60492",60492,"CCDC90B","MDS011|MDS025|MGC104239",11,"coiled-coil domain containing 90B","mitochondrion | integral to membrane | membrane",null,null,"CCDC90B","coiled-coil domain containing 90B","MitoCarta3.0",0,"MIM",1,"DUF1640",null,"COILED COIL DOMAIN CONTAINING MITOCHONDRIAL PRECURSOR","ENSFM00610000952921",2,"FirstInEnsemblFamily",null,"CCDC90B",0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,0,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,1,1,0,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,86,1,0,0,0]
["hsa:51654",51654,"CDK5RAP1","C20orf34|C42|CDK5RAP1.3|CDK5RAP1.4|CGI-05|HSPC167",20,"CDK5 regulatory subunit associated protein 1","cellular_component | cytoplasm","4 iron, 4 sulfur cluster binding | transferase activity | neuronal Cdc2-like kinase binding | metal ion binding","negative regulation of cyclin-dependent protein kinase activity | brain development | regulation of neuron differentiation | RNA modification | tRNA modification","CDK5RAP1","CDK5 regulatory subunit associated protein 1","MitoCarta3.0","Mitochondrial central dogma > mtRNA metabolism > mt-tRNA modifications","Matrix",0,"Radical_SAM | TRAM | UPF0004",null,null,null,null,"FirstInEnsemblFamily",null,"CDK5RAP1",1,0,1,1,1,0,1,1,0,0,0,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,85,1,1,"Mitochondrial central dogma > mtRNA metabolism > mt-tRNA modifications","Mitochondrial central dogma "," mtRNA metabolism "," mt-tRNA modifications"]
["hsa:55486",55486,"PARL","PSARL|PSARL1|PSENIP2|RHBDS1",3,"presenilin associated, rhomboid-like","mitochondrion | integral to membrane | mitochondrial inner membrane | nucleus | membrane","peptidase activity | serine-type endopeptidase activity","proteolysis","PARL","presenilin associated, rhomboid-like","MitoCarta3.0","Protein import, sorting and homeostasis > Protein homeostasis > Proteases | Mitochondrial dynamics and surveillance > Mitophagy | Mitochondrial dynamics and surveillance > Autophagy","MIM",6,"Rhomboid",null,null,null,null,"FirstInEnsemblFamily",null,"PARL",1,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,0,0,1,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,1,1,0,1,1,1,1,1,1,1,1,1,0,0,1,0,0,1,1,1,0,1,1,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,85,1,0,"Protein import, sorting and homeostasis > Protein homeostasis > Proteases | Mitochondrial dynamics and surveillance > Mitophagy | Mitochondrial dynamics and surveillance > Autophagy","Protein import, sorting and homeostasis "," Protein homeostasis "," Proteases "," Mitochondrial dynamics and surveillance "," Mitophagy ",null," Mitochondrial dynamics and surveillance "," Autophagy"]
["hsa:4713",4713,"NDUFB7","B18|CI-B18|MGC2480",19,"NADH dehydrogenase (ubiquinone) 1 beta subcomplex, 7, 18kDa","mitochondrion | respiratory chain | mitochondrial inner membrane | mitochondrial respiratory chain complex I | membrane | mitochondrial intermembrane space","NADH dehydrogenase activity | NADH dehydrogenase (ubiquinone) activity","transport | mitochondrial electron transport, NADH to ubiquinone | respiratory electron transport chain","NDUFB7","NADH dehydrogenase (ubiquinone) 1 beta subcomplex, 7, 18kDa","MitoCarta3.0","OXPHOS > Complex I > CI subunits | OXPHOS > OXPHOS subunits","MIM",0,"NDUF_B7","Oxidative phosphorylation",null,null,null,"FirstInEnsemblFamily",null,"NDUFB7",0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,1,0,1,0,1,0,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,0,0,1,0,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,0,1,1,0,1,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,1,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,0,1,1,1,1,1,1,82,1,1,"OXPHOS > Complex I > CI subunits | OXPHOS > OXPHOS subunits","OXPHOS "," Complex I "," CI subunits "," OXPHOS "," OXPHOS subunits"]
["hsa:84947",84947,"SERAC1","FLJ14917|FLJ30544",6,"serine active site containing 1","extracellular matrix | integral to membrane | membrane | intrinsic to endoplasmic reticulum membrane","hydrolase activity, acting on ester bonds | binding","intracellular protein transport | GPI anchor metabolic process | extracellular matrix organization","SERAC1","serine active site containing 1","MitoCarta3.0","Metabolism > Lipid metabolism > Phospholipid metabolism","MOM",0,null,null,null,null,null,"FirstInEnsemblFamily","3-methylglutaconic aciduria with deafness, encephalopathy, and Leigh-like syndrome","SERAC1",0,0,0,0,0,0,0,1,0,0,0,1,0,1,0,0,0,1,1,1,1,1,1,0,0,1,0,0,1,0,0,0,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,0,0,0,0,0,0,0,0,1,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,1,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,0,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,81,1,1,"Metabolism > Lipid metabolism > Phospholipid metabolism","Metabolism "," Lipid metabolism "," Phospholipid metabolism"]
["hsa:51522",51522,"TMEM14C","C6orf53|MSTP073|NET26|bA421M1.6",6,"transmembrane protein 14C","mitochondrion | integral to membrane | mitochondrial membrane | membrane",null,"heme biosynthetic process","TMEM14C","transmembrane protein 14C","MitoCarta3.0",0,"MIM",4,"TMEM14",null,null,null,null,"FirstInEnsemblFamily",null,"TMEM14C",0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,0,0,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,0,0,1,0,0,0,0,0,1,1,1,0,0,0,0,1,0,0,0,0,0,0,0,1,1,1,1,0,0,0,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,0,1,0,1,0,1,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,80,1,0,0,0]
["hsa:4537",4537,"ND3","MTND3","MT","NADH dehydrogenase, subunit 3 (complex I)","mitochondrion | respiratory chain | integral to membrane | mitochondrial inner membrane | mitochondrial respiratory chain complex I | membrane","protein binding | oxidoreductase activity | NADH dehydrogenase (ubiquinone) activity","response to oxidative stress | transport | mitochondrial electron transport, NADH to ubiquinone | response to hormone stimulus | response to light intensity | respiratory electron transport chain","ND3","NADH dehydrogenase subunit 3","MitoCarta3.0","OXPHOS > Complex I > CI subunits | OXPHOS > OXPHOS subunits","MIM",3,"Oxidored_q4","Oxidative phosphorylation",null,null,null,"FirstInEnsemblFamily",null,"ND3",1,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,1,1,1,1,1,1,0,1,1,1,1,0,1,1,1,1,0,1,1,1,1,1,1,0,1,1,1,0,1,1,0,1,1,0,0,0,0,1,0,1,1,0,0,0,0,0,0,1,1,0,0,0,0,0,1,1,0,1,0,0,1,1,1,0,0,0,0,0,0,0,0,0,0,0,0,0,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,78,1,1,"OXPHOS > Complex I > CI subunits | OXPHOS > OXPHOS subunits","OXPHOS "," Complex I "," CI subunits "," OXPHOS "," OXPHOS subunits"]
["hsa:4536",4536,"ND2","MTND2","MT","MTND2","mitochondrion | respiratory chain | integral to membrane | mitochondrial inner membrane | postsynaptic density | mitochondrial respiratory chain complex I | membrane","oxidoreductase activity | NADH dehydrogenase (ubiquinone) activity","transport | mitochondrial electron transport, NADH to ubiquinone | reactive oxygen species metabolic process | ATP synthesis coupled electron transport | respiratory electron transport chain","ND2","NADH dehydrogenase subunit 2","MitoCarta3.0","OXPHOS > Complex I > CI subunits | OXPHOS > OXPHOS subunits","MIM",9,"Oxidored_q1 | NADH_dehy_S2_C","Oxidative phosphorylation",null,null,null,"FirstInEnsemblFamily",null,"ND2",1,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,1,1,1,1,1,1,0,1,1,0,1,1,1,1,1,0,0,1,1,1,1,1,1,0,1,1,1,0,1,1,0,1,1,0,0,0,0,1,0,1,1,1,0,0,0,0,1,1,1,0,0,0,1,0,1,1,0,1,0,0,0,0,1,0,0,0,0,0,0,0,0,0,0,0,0,0,1,1,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,77,1,0,"OXPHOS > Complex I > CI subunits | OXPHOS > OXPHOS subunits","OXPHOS "," Complex I "," CI subunits "," OXPHOS "," OXPHOS subunits"]
["hsa:83875",83875,"BCO2","B-DIOX-II|BCDO2|FLJ34464",11,"beta-carotene oxygenase 2","intracellular","oxidoreductase activity, acting on single donors with incorporation of molecular oxygen, incorporation of two atoms of oxygen | oxidoreductase activity | metal ion binding","retinal metabolic process | retinoic acid metabolic process | carotene metabolic process","BCDO2","beta-carotene dioxygenase 2","MitoCarta3.0","Metabolism > Vitamin metabolism > Vitamin A metabolism","MIM",0,"RPE65",null,"RETINOID ISOMEROHYDROLASE EC_3.1.1.64 EC_5.2.1.- 7 ALL TRANS RETINYL PALMITATE HYDROLASE RETINAL PIGMENT EPITHELIUM SPECIFIC 65 KDA RETINOL ISOMERASE","ENSFM00250000000935",3,"FirstInEnsemblFamily",null,"BCO2",1,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,0,1,1,0,1,0,0,0,0,0,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,76,1,0,"Metabolism > Vitamin metabolism > Vitamin A metabolism","Metabolism "," Vitamin metabolism "," Vitamin A metabolism"]
["hsa:10989",10989,"IMMT","DKFZp779P1653|HMP|MGC111146|MINOS2|P87|P87/89|P89|PIG52",2,"inner membrane protein, mitochondrial","mitochondrion | integral to membrane | mitochondrial inner membrane | membrane | integral to mitochondrial inner membrane","protein binding","biological_process","IMMT","inner membrane protein, mitochondrial (mitofilin)","MitoCarta3.0","Mitochondrial dynamics and surveillance > Cristae formation > MICOS complex","MIM",0,null,null,null,null,null,"FirstInEnsemblFamily",null,"IMMT",0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,1,0,1,0,0,0,0,0,0,1,0,0,0,0,0,0,0,0,0,0,1,0,0,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,75,1,0,"Mitochondrial dynamics and surveillance > Cristae formation > MICOS complex","Mitochondrial dynamics and surveillance "," Cristae formation "," MICOS complex"]
["hsa:55037",55037,"PTCD3","DKFZp666K071|FLJ20758",2,"Pentatricopeptide repeat domain 3","mitochondrion",null,null,"PTCD3","Pentatricopeptide repeat domain 3","MitoCarta3.0","Mitochondrial central dogma > Translation > Mitochondrial ribosome | Mitochondrial central dogma > Translation > Mitochondrial ribosome assembly","Matrix",0,"PPR",null,null,null,null,"FirstInEnsemblFamily",null,"PTCD3",0,0,0,0,0,0,1,1,1,1,1,1,0,0,1,1,1,0,0,0,0,0,0,0,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,0,1,1,1,0,1,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,0,1,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,0,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,74,1,1,"Mitochondrial central dogma > Translation > Mitochondrial ribosome | Mitochondrial central dogma > Translation > Mitochondrial ribosome assembly","Mitochondrial central dogma "," Translation "," Mitochondrial ribosome "," Mitochondrial central dogma "," Translation "," Mitochondrial ribosome assembly"]
["hsa:57048",57048,"PLSCR3","-",17,"phospholipid scramblase 3","integral to membrane | plasma membrane","calcium-dependent protein binding | protein binding | SH3 domain binding | phospholipid scramblase activity | calcium ion binding","phospholipid scrambling","PLSCR3","phospholipid scramblase 3","MitoCarta3.0","Metabolism > Lipid metabolism > Cardiolipin synthesis","MIM",0,"Scramblase",null,"PHOSPHOLIPID SCRAMBLASE PL SCRAMBLASE CA 2+ DEPENDENT PHOSPHOLIPID SCRAMBLASE","ENSFM00500000269855",5,null,null,"PLSCR3",0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,1,0,1,1,1,1,0,0,0,1,1,0,0,1,1,1,1,1,1,1,1,1,1,0,1,1,1,0,1,1,1,0,0,1,1,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,1,1,1,1,1,0,1,0,0,0,0,0,0,0,0,1,1,1,1,0,0,1,1,0,1,0,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,73,1,0,"Metabolism > Lipid metabolism > Cardiolipin synthesis","Metabolism "," Lipid metabolism "," Cardiolipin synthesis"]
["hsa:80142",80142,"PTGES2","C9orf15|FLJ14038|GBF1|MGC11289|PGES2|mPGES-2",9,"prostaglandin E synthase 2","mitochondrion | Golgi apparatus | Golgi membrane | perinuclear region of cytoplasm | cytoplasm | cytosol | integral to membrane | nucleus | membrane","prostaglandin-E synthase activity | isomerase activity | electron carrier activity | protein disulfide oxidoreductase activity | DNA binding","positive regulation of transcription, DNA-dependent | secretion | cell redox homeostasis | fatty acid biosynthetic process | prostaglandin biosynthetic process","PTGES2","prostaglandin E synthase 2","MitoCarta3.0","Metabolism > Lipid metabolism > Eicosanoid metabolism","MIM",0,"Glutaredoxin | GST_C","Arachidonic acid metabolism",null,null,null,"FirstInEnsemblFamily",null,"PTGES2",1,0,0,0,0,0,1,1,1,1,1,1,0,0,0,0,0,0,0,0,0,0,0,0,0,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,0,0,0,0,1,0,0,1,0,0,0,0,0,0,0,0,1,0,0,1,0,0,1,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,0,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,72,1,1,"Metabolism > Lipid metabolism > Eicosanoid metabolism","Metabolism "," Lipid metabolism "," Eicosanoid metabolism"]
["hsa:55052",55052,"MRPL20","L20mt|MGC4779|MGC74465|MRP-L20",1,"mitochondrial ribosomal protein L20","mitochondrion | ribosome | mitochondrial ribosome | intracellular","protein binding | structural constituent of ribosome | rRNA binding","translation","MRPL20","mitochondrial ribosomal protein L20","MitoCarta3.0","Mitochondrial central dogma > Translation > Mitochondrial ribosome","Matrix",0,"Ribosomal_L20",null,null,null,null,"FirstInEnsemblFamily",null,"MRPL20",1,0,0,0,0,0,0,0,0,0,0,1,0,0,1,1,1,1,1,1,0,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,0,1,1,1,1,1,1,69,1,0,"Mitochondrial central dogma > Translation > Mitochondrial ribosome","Mitochondrial central dogma "," Translation "," Mitochondrial ribosome"]
["hsa:100510565",8225,"GTPBP6","FLJ20977|FLJ22809|FLJ56662|FLJ56805|FLJ90047|PGPL","X|Y","GTP binding protein 6 (putative)","intracellular","GTP binding | nucleotide binding",null,"GTPBP6","GTP binding protein 6 (putative)","MitoCarta3.0",0,"Matrix",0,"MMR_HSR1",null,null,null,null,"FirstInEnsemblFamily",null,"GTPBP6",1,0,0,0,0,0,0,0,0,0,0,1,0,0,0,0,0,1,1,1,1,1,1,1,1,0,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,0,0,0,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,68,1,0,0,0]
["hsa:115416",115416,"C7orf30","-",7,"chromosome 7 open reading frame 30","mitochondrion",null,null,null,null,"MitoCarta3.0","Mitochondrial central dogma > Translation > Mitochondrial ribosome assembly","Matrix",null,"DUF143",null,null,null,null,"FirstInEnsemblFamily",null,"C7orf30",1,0,0,0,0,0,1,1,1,1,1,0,0,0,0,1,1,0,0,0,0,0,0,0,1,1,1,1,1,1,1,1,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,1,1,1,1,0,0,0,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,66,1,1,"Mitochondrial central dogma > Translation > Mitochondrial ribosome assembly","Mitochondrial central dogma "," Translation "," Mitochondrial ribosome assembly"]
["hsa:65008",65008,"MRPL1","BM022|FLJ96680|L1MT|MRP-L1",4,"mitochondrial ribosomal protein L1","mitochondrion | intracellular | large ribosomal subunit","structural constituent of ribosome | RNA binding","translation","MRPL1","mitochondrial ribosomal protein L1","MitoCarta3.0","Mitochondrial central dogma > Translation > Mitochondrial ribosome","Matrix",0,"Ribosomal_L1",null,null,null,null,"FirstInEnsemblFamily",null,"MRPL1",1,0,0,0,0,0,0,0,0,0,0,1,0,0,1,0,0,0,0,1,0,0,0,0,0,1,1,0,1,0,0,0,1,1,1,1,1,1,1,0,0,0,0,0,1,1,1,0,0,0,1,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,1,0,0,0,0,0,0,0,0,0,0,1,1,0,0,1,1,0,0,1,1,0,1,1,0,1,1,0,0,0,0,0,0,0,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,66,1,1,"Mitochondrial central dogma > Translation > Mitochondrial ribosome","Mitochondrial central dogma "," Translation "," Mitochondrial ribosome"]
["hsa:64943",64943,"NT5DC2","FLJ12442",3,"5'-nucleotidase domain containing 2",null,"hydrolase activity | metal ion binding",null,"NT5DC2","5'-nucleotidase domain containing 2","MitoCarta3.0","Metabolism > Nucleotide metabolism > Nucleotide synthesis and processing","Matrix",0,"5_nucleotid",null,"5' NUCLEOTIDASE DOMAIN CONTAINING","ENSFM00250000001969",2,"FirstInEnsemblFamily",null,"NT5DC2",0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,64,1,0,"Metabolism > Nucleotide metabolism > Nucleotide synthesis and processing","Metabolism "," Nucleotide metabolism "," Nucleotide synthesis and processing"]
["hsa:64216",64216,"TFB2M","FLJ22661|FLJ23182|Hkp1|mtTFB2",1,"transcription factor B2, mitochondrial","mitochondrial nucleoid | mitochondrial matrix","rRNA methyltransferase activity | methyltransferase activity | rRNA (adenine-N6,N6-)-dimethyltransferase activity | transcription cofactor activity | transferase activity","positive regulation of transcription, DNA-dependent | transcription initiation from mitochondrial promoter | transcription from mitochondrial promoter","TFB2M","transcription factor B2, mitochondrial","MitoCarta3.0","Mitochondrial central dogma > mtDNA maintenance > mtDNA replication | Mitochondrial central dogma > mtRNA metabolism > Transcription","Matrix",0,"RrnaAD",null,null,null,null,"FirstInEnsemblFamily",null,"TFB2M",1,0,0,0,1,0,1,1,1,1,1,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,1,0,0,0,0,1,0,1,1,0,1,0,1,1,1,1,1,1,1,1,1,0,0,0,1,0,0,0,0,0,0,0,1,0,0,0,0,1,1,1,1,1,0,1,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,0,0,1,1,1,1,0,1,1,1,1,0,0,0,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,62,1,0,"Mitochondrial central dogma > mtDNA maintenance > mtDNA replication | Mitochondrial central dogma > mtRNA metabolism > Transcription","Mitochondrial central dogma "," mtDNA maintenance "," mtDNA replication "," Mitochondrial central dogma "," mtRNA metabolism "," Transcription"]
["hsa:51031",51031,"GLOD4","C17orf25|HC71",17,"glyoxalase domain containing 4","mitochondrion",null,null,"C17orf25","chromosome 17 open reading frame 25","MitoCarta3.0",0,"Matrix",0,"Glyoxalase",null,null,null,null,"FirstInEnsemblFamily",null,"GLOD4",1,0,0,0,0,0,0,1,1,1,1,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,1,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,1,0,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,61,1,1,0,0]
["hsa:2495",2495,"FTH1","FHC|FTH|FTHL6|MGC104426|PIG15|PLIF",11,"ferritin, heavy polypeptide 1","cytosol | mitochondrion | intracellular ferritin complex","protein binding | oxidoreductase activity | ferric iron binding | iron ion binding | ferroxidase activity","post-Golgi vesicle-mediated transport | negative regulation of cell proliferation | cellular iron ion homeostasis | immune response | intracellular sequestering of iron ion | iron ion transport | cell proliferation | cellular membrane organization | negative regulation of necrotic cell death","FTH1","ferritin, heavy polypeptide 1","MitoCarta3.0","Metabolism > Metals and cofactors > Iron homeostasis","unknown",0,"Ferritin","Porphyrin and chlorophyll metabolism","FERRITIN HEAVY CHAIN FERRITIN H SUBUNIT EC_1.16.3.1","ENSFM00500000269681",3,"FirstInEnsemblFamily",null,"FTH1",1,1,0,0,0,1,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,0,0,0,0,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,0,0,1,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,0,0,0,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,60,1,1,"Metabolism > Metals and cofactors > Iron homeostasis","Metabolism "," Metals and cofactors "," Iron homeostasis"]
["hsa:514",514,"ATP5E","ATPE|MC5DN3|MGC104243",20,"ATP synthase, H+ transporting, mitochondrial F1 complex, epsilon subunit","mitochondrion | membrane | mitochondrial matrix | mitochondrial proton-transporting ATP synthase complex | mitochondrial proton-transporting ATP synthase complex, catalytic core F(1)","hydrolase activity | hydrogen ion transporting ATP synthase activity, rotational mechanism | transmembrane transporter activity | ATPase activity | proton-transporting ATPase activity, rotational mechanism","proton transport | ion transport | mitochondrial ATP synthesis coupled proton transport | ATP catabolic process | respiratory electron transport chain","ATP5E","ATP synthase, H+ transporting, mitochondrial F1 complex, epsilon subunit","MitoCarta3.0","OXPHOS > Complex V > CV subunits | OXPHOS > OXPHOS subunits","MIM",0,"ATP-synt_Eps","Oxidative phosphorylation",null,null,null,"FirstInEnsemblFamily","Mitochondrial complex V (ATP synthase) deficiency, nuclear type 3","ATP5E",0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,0,1,1,0,0,1,0,1,1,1,1,1,1,0,1,1,1,1,0,1,1,1,0,0,0,0,0,0,0,0,0,0,1,0,0,0,0,0,1,0,1,1,1,0,0,1,1,0,1,0,0,0,1,0,0,0,0,0,0,0,0,0,0,0,1,0,0,1,1,1,0,1,0,1,1,1,0,0,1,1,1,1,1,0,1,1,1,1,1,1,0,0,1,1,0,1,1,0,0,1,0,1,1,1,1,1,1,0,1,1,58,1,1,"OXPHOS > Complex V > CV subunits | OXPHOS > OXPHOS subunits","OXPHOS "," Complex V "," CV subunits "," OXPHOS "," OXPHOS subunits"]
["hsa:134145",134145,"FAM173B","FLJ20667",5,"family with sequence similarity 173, member B","integral to membrane | membrane",null,null,"LOC134145","hypothetical protein LOC134145","MitoCarta3.0","OXPHOS > Complex V > CV assembly factors | OXPHOS > OXPHOS assembly factors","MIM",1,null,null,null,null,null,"FirstInEnsemblFamily",null,"FAM173B",1,0,1,1,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,1,1,1,0,1,0,1,1,1,0,0,0,0,0,0,0,0,0,0,0,1,1,1,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,52,1,1,"OXPHOS > Complex V > CV assembly factors | OXPHOS > OXPHOS assembly factors","OXPHOS "," Complex V "," CV assembly factors "," OXPHOS "," OXPHOS assembly factors"]
["hsa:65990",65990,"FAM173A","C16orf24|MGC2494",16,"family with sequence similarity 173, member A","integral to membrane | membrane",null,null,"C16orf24","chromosome 16 open reading frame 24","MitoCarta3.0","Metabolism > Nucleotide metabolism","MIM",1,null,null,null,null,null,"FirstInEnsemblFamily",null,"FAM173A",1,0,1,1,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,1,1,1,0,0,0,1,1,1,0,0,0,0,0,0,0,0,0,0,0,0,1,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,50,1,0,"Metabolism > Nucleotide metabolism","Metabolism "," Nucleotide metabolism"]
["hsa:55347",55347,"ABHD10","FLJ11342",3,"abhydrolase domain containing 10","mitochondrion","serine-type peptidase activity | peptidase activity","proteolysis","ABHD10","abhydrolase domain containing 10","MitoCarta3.0","Metabolism > Detoxification > Xenobiotic metabolism","Matrix",0,null,null,null,null,null,"FirstInEnsemblFamily",null,"ABHD10",1,0,0,0,0,0,0,1,1,1,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,1,0,0,1,0,1,1,0,0,1,1,1,1,1,0,1,1,1,1,0,1,1,0,1,1,1,0,0,0,0,0,0,1,0,0,0,1,0,0,0,1,0,0,1,0,0,0,0,0,0,0,1,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,1,1,0,0,0,0,0,0,0,0,0,0,0,0,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,49,1,1,"Metabolism > Detoxification > Xenobiotic metabolism","Metabolism "," Detoxification "," Xenobiotic metabolism"]
["hsa:133383",133383,"C5orf35","MGC33648",5,"chromosome 5 open reading frame 35",null,null,null,null,null,"MitoCarta3.0",0,"Matrix",null,null,null,null,null,null,"FirstInEnsemblFamily",null,"C5orf35",0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,1,0,0,0,0,1,1,0,0,0,0,1,1,0,1,0,0,0,0,0,1,1,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,0,0,0,0,0,0,0,0,0,0,0,1,1,1,0,1,1,1,1,1,1,1,1,0,1,1,1,0,1,1,1,27,1,1,0,0]
["hsa:1355",1355,"COX15","-",10,"COX15 homolog, cytochrome c oxidase assembly protein (yeast)","mitochondrial respiratory chain | integral to membrane | mitochondrial part | membrane","oxidoreductase activity, acting on the CH-CH group of donors | cytochrome-c oxidase activity | catalytic activity","oxidation-reduction process | respiratory chain complex IV assembly | respiratory gaseous exchange | mitochondrial electron transport, cytochrome c to oxygen | cellular respiration | heme a biosynthetic process | protein complex assembly","COX15","COX15 homolog, cytochrome c oxidase assembly protein (yeast)","MitoCarta3.0","OXPHOS > Complex IV > CIV assembly factors | Metabolism > Metals and cofactors > Heme synthesis and processing | OXPHOS > OXPHOS assembly factors","MIM",8,"COX15-CtaA","Porphyrin and chlorophyll metabolism | Oxidative phosphorylation",null,null,null,"FirstInEnsemblFamily",null,"COX15",1,0,0,0,0,0,1,1,1,1,1,1,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,129,0,0,"OXPHOS > Complex IV > CIV assembly factors | Metabolism > Metals and cofactors > Heme synthesis and processing | OXPHOS > OXPHOS assembly factors","OXPHOS "," Complex IV "," CIV assembly factors "," Metabolism "," Metals and cofactors "," Heme synthesis and processing "," OXPHOS "," OXPHOS assembly factors"]
["hsa:6697",6697,"SPR","SDR38C1",2,"sepiapterin reductase (7,8-dihydrobiopterin:NADP+ oxidoreductase)","cytoplasm","oxidoreductase activity | aldo-keto reductase (NADP) activity | NADP binding | sepiapterin reductase activity","oxidation-reduction process | nitric oxide biosynthetic process | tetrahydrobiopterin biosynthetic process","SPR","sepiapterin reductase (7,8-dihydrobiopterin:NADP+ oxidoreductase)","MitoCarta3.0","Metabolism > Metals and cofactors > Tetrahydobiopterin synthesis","unknown",0,"adh_short","Folate biosynthesis",null,null,null,"FirstInEnsemblFamily","Dystonia, dopa-responsive, due to sepiapterin reductase deficiency","SPR",1,0,1,1,1,1,1,0,0,0,0,1,0,0,0,0,0,1,1,1,0,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,127,0,0,"Metabolism > Metals and cofactors > Tetrahydobiopterin synthesis","Metabolism "," Metals and cofactors "," Tetrahydobiopterin synthesis"]
["hsa:55750",55750,"AGK","FLJ10842|MULK",7,"acylglycerol kinase","mitochondrion | mitochondrial membrane | membrane","diacylglycerol kinase activity | NAD+ kinase activity | ceramide kinase activity | acylglycerol kinase activity | transferase activity | nucleotide binding | ATP binding","activation of protein kinase C activity by G-protein coupled receptor protein signaling pathway | ceramide biosynthetic process | metabolic process","MULK","multiple substrate lipid kinase","MitoCarta3.0","Protein import, sorting and homeostasis > Protein import and sorting > TIM22 carrier pathway | Metabolism > Lipid metabolism > Phospholipid metabolism","MIM",0,"DAGK_cat","Glycerolipid metabolism",null,null,null,"FirstInEnsemblFamily","Cataract, autosomal recessive congenital 5|Sengers syndrome","AGK",1,0,1,1,0,1,1,1,1,1,1,0,0,0,0,0,0,0,0,0,0,0,0,1,1,1,1,1,0,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,122,0,0,"Protein import, sorting and homeostasis > Protein import and sorting > TIM22 carrier pathway | Metabolism > Lipid metabolism > Phospholipid metabolism","Protein import, sorting and homeostasis "," Protein import and sorting "," TIM22 carrier pathway "," Metabolism "," Lipid metabolism "," Phospholipid metabolism"]
["hsa:79814",79814,"AGMAT","FLJ23384",1,"agmatine ureohydrolase (agmatinase)","mitochondrion","hydrolase activity | agmatinase activity | metal ion binding","spermidine biosynthetic process | polyamine metabolic process | putrescine biosynthetic process | cellular nitrogen compound metabolic process","AGMAT","agmatine ureohydrolase (agmatinase)","MitoCarta3.0","Metabolism > Amino acid metabolism","Matrix",0,"Arginase","Arginine and proline metabolism",null,null,null,"FirstInEnsemblFamily",null,"AGMAT",1,0,1,1,0,1,1,1,1,1,1,0,0,0,0,0,0,1,1,1,0,1,1,1,1,1,1,1,0,1,0,0,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,0,1,1,1,0,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,120,0,0,"Metabolism > Amino acid metabolism","Metabolism "," Amino acid metabolism"]
["hsa:9489",9489,"PGS1","DKFZp762M186|MGC131960",17,"phosphatidylglycerophosphate synthase 1","mitochondrion | endoplasmic reticulum","phosphotransferase activity, for other substituted phosphate groups | transferase activity | nucleotide binding | CDP-diacylglycerol-glycerol-3-phosphate 3-phosphatidyltransferase activity | ATP binding","phospholipid biosynthetic process","PGS1","phosphatidylglycerophosphate synthase 1","MitoCarta3.0","Metabolism > Lipid metabolism > Cardiolipin synthesis | Metabolism > Lipid metabolism > Phospholipid metabolism","MIM",0,"PLDc","Glycerophospholipid metabolism",null,null,null,"FirstInEnsemblFamily",null,"PGS1",1,1,0,0,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,0,0,0,0,0,1,0,0,0,0,0,0,0,0,0,0,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,0,1,1,1,1,114,0,0,"Metabolism > Lipid metabolism > Cardiolipin synthesis | Metabolism > Lipid metabolism > Phospholipid metabolism","Metabolism "," Lipid metabolism "," Cardiolipin synthesis "," Metabolism "," Lipid metabolism "," Phospholipid metabolism"]
["hsa:3052",3052,"HCCS","CCHL|DKFZp779I1858|MCOPS7","X","holocytochrome c synthase","mitochondrion | mitochondrial inner membrane | membrane","lyase activity | holocytochrome-c synthase activity | metal ion binding","oxidation-reduction process | organ morphogenesis","HCCS","holocytochrome c synthase (cytochrome c heme-lyase)","MitoCarta3.0","Metabolism > Metals and cofactors > Heme synthesis and processing | OXPHOS > OXPHOS subunits | OXPHOS > Cytochrome C","IMS",0,"Cyto_heme_lyase","Porphyrin and chlorophyll metabolism",null,null,null,"FirstInEnsemblFamily","Microphthalmia, syndromic 7","HCCS",0,0,0,0,0,0,0,0,0,0,0,1,0,0,1,1,1,1,1,1,1,1,1,0,0,1,1,1,0,0,1,1,1,1,0,1,0,0,0,0,0,0,0,0,0,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,109,0,0,"Metabolism > Metals and cofactors > Heme synthesis and processing | OXPHOS > OXPHOS subunits | OXPHOS > Cytochrome C","Metabolism "," Metals and cofactors "," Heme synthesis and processing "," OXPHOS "," OXPHOS subunits ",null," OXPHOS "," Cytochrome C"]
["hsa:51012",51012,"SLMO2","C20orf45|PRELID3B|dJ543J19.5",20,"slowmo homolog 2 (Drosophila)","mitochondrion",null,null,"C20orf45","chromosome 20 open reading frame 45","MitoCarta3.0","Metabolism > Lipid metabolism > Phospholipid metabolism","IMS",0,"MSF1",null,"SLOWMO HOMOLOG","ENSFM00500000270508",2,"FirstInEnsemblFamily",null,"SLMO2",0,0,0,0,0,0,0,0,0,0,0,1,1,1,0,0,0,1,0,1,0,0,1,0,0,0,0,0,0,0,0,0,0,0,1,1,1,1,1,1,1,1,0,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,109,0,0,"Metabolism > Lipid metabolism > Phospholipid metabolism","Metabolism "," Lipid metabolism "," Phospholipid metabolism"]
["hsa:10157",10157,"AASS","LKR/SDH|LKRSDH|LORSDH",7,"aminoadipate-semialdehyde synthase","intracellular membrane-bounded organelle | mitochondrion | mitochondrial matrix | cytoplasm","saccharopine dehydrogenase (NAD+, L-glutamate-forming) activity | oxidoreductase activity | saccharopine dehydrogenase (NADP+, L-lysine-forming) activity | nucleotide binding","protein tetramerization | L-lysine catabolic process | cellular nitrogen compound metabolic process | lysine catabolic process","AASS","aminoadipate-semialdehyde synthase","MitoCarta3.0","Metabolism > Amino acid metabolism > Lysine metabolism","Matrix",0,"AlaDh_PNT_N | AlaDh_PNT_C | Saccharop_dh","Lysine degradation | Lysine biosynthesis",null,null,null,"FirstInEnsemblFamily","Hyperlysinemia","AASS",1,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,1,1,1,1,0,1,0,0,0,0,1,1,1,1,0,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,107,0,0,"Metabolism > Amino acid metabolism > Lysine metabolism","Metabolism "," Amino acid metabolism "," Lysine metabolism"]
["hsa:100131187",100131187,"TSTD1","KAT",1,"thiosulfate sulfurtransferase (rhodanese)-like domain containing 1","mitochondrion | perinuclear region of cytoplasm | cytoplasm",null,null,null,null,"MitoCarta3.0","Metabolism > Sulfur metabolism","unknown",null,null,null,null,null,null,"FirstInEnsemblFamily",null,"TSTD1",1,0,0,0,0,0,1,1,1,1,1,1,0,0,0,0,0,0,0,0,0,0,0,0,1,0,1,1,0,1,0,0,1,1,1,1,0,1,1,0,1,1,0,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,0,1,0,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,106,0,0,"Metabolism > Sulfur metabolism","Metabolism "," Sulfur metabolism"]
["hsa:118980",118980,"SFXN2","-",10,"sideroflexin 2","mitochondrion | integral to membrane | mitochondrial inner membrane | membrane","cation transmembrane transporter activity","iron ion homeostasis | transmembrane transport","SFXN2","sideroflexin 2","MitoCarta3.0","Small molecule transport > Sideroflexins","MIM",5,"Mtc",null,"SIDEROFLEXIN","ENSFM00500000269857",3,"FirstInEnsemblFamily",null,"SFXN2",0,0,0,0,0,0,1,1,1,1,1,1,0,0,0,0,0,0,0,0,0,0,0,1,1,1,1,1,0,1,0,0,0,0,1,1,0,0,0,0,0,0,0,0,0,0,0,1,1,1,1,0,1,1,0,0,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,101,0,0,"Small molecule transport > Sideroflexins","Small molecule transport "," Sideroflexins"]
["hsa:94097",94097,"SFXN5","BBG-TCC|MGC120413|MGC120415",2,"sideroflexin 5","mitochondrion | integral to membrane | mitochondrial inner membrane | membrane","cation transmembrane transporter activity","iron ion homeostasis | transmembrane transport","SFXN5","sideroflexin 5","MitoCarta3.0","Metabolism > Carbohydrate metabolism > TCA-associated | Small molecule transport > Sideroflexins","MIM",5,"Mtc",null,null,null,null,"FirstInEnsemblFamily",null,"SFXN5",0,0,0,0,0,0,1,1,1,1,1,1,0,0,0,0,0,0,0,0,0,0,0,1,1,1,1,1,0,1,0,0,0,0,1,1,0,0,0,0,0,0,0,0,0,0,0,1,1,1,1,0,1,1,0,0,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,101,0,0,"Metabolism > Carbohydrate metabolism > TCA-associated | Small molecule transport > Sideroflexins","Metabolism "," Carbohydrate metabolism "," TCA-associated "," Small molecule transport "," Sideroflexins"]
["hsa:119559",119559,"SFXN4","BCRM1",10,"sideroflexin 4","mitochondrion | integral to membrane | mitochondrial membrane | membrane","cation transmembrane transporter activity","iron ion homeostasis | transmembrane transport","SFXN4","sideroflexin 4","MitoCarta3.0","Small molecule transport > Sideroflexins","MIM",4,"Mtc",null,null,null,null,"FirstInEnsemblFamily",null,"SFXN4",0,0,0,0,0,0,1,1,1,1,1,1,0,0,0,0,0,0,0,0,0,0,0,1,1,1,1,1,0,1,0,0,0,0,1,1,0,0,0,0,0,0,0,0,0,0,0,1,1,1,1,0,1,1,0,0,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,99,0,0,"Small molecule transport > Sideroflexins","Small molecule transport "," Sideroflexins"]
["hsa:126129",126129,"CPT1C","CATL1|CPT1-B|CPT1P|CPTI-B|CPTIC|FLJ23809",19,"carnitine palmitoyltransferase 1C","mitochondrial outer membrane | mitochondrion | integral to membrane | endoplasmic reticulum membrane | membrane","carnitine O-palmitoyltransferase activity | transferase activity, transferring acyl groups","carnitine metabolic process | fatty acid metabolic process | lipid metabolic process","CPT1C","carnitine palmitoyltransferase 1C","MitoCarta3.0","Metabolism > Lipid metabolism > Fatty acid oxidation | Metabolism > Metals and cofactors > Carnitine synthesis and transport | Metabolism > Metals and cofactors > Carnitine shuttle","MOM",2,"Carn_acyltransf","Fatty acid metabolism","CARNITINE O PALMITOYLTRANSFERASE 1 MUSCLE CPT1 M EC_2.3.1.21 CARNITINE O PALMITOYLTRANSFERASE I MUSCLE CPT I CPTI M CARNITINE PALMITOYLTRANSFERASE 1B","ENSFM00500000269758",3,null,null,"CPT1C",0,0,0,0,0,0,1,1,1,1,1,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,1,1,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,0,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,98,0,0,"Metabolism > Lipid metabolism > Fatty acid oxidation | Metabolism > Metals and cofactors > Carnitine synthesis and transport | Metabolism > Metals and cofactors > Carnitine shuttle","Metabolism "," Lipid metabolism "," Fatty acid oxidation "," Metabolism "," Metals and cofactors "," Carnitine synthesis and transport "," Metabolism "," Metals and cofactors "," Carnitine shuttle"]
["hsa:246243",246243,"RNASEH1","H1RNA|RNH1",2,"ribonuclease H1","cytoplasm","hydrolase activity | ribonuclease activity | ribonuclease H activity | RNA binding | magnesium ion binding | endonuclease activity | nucleic acid binding","RNA catabolic process","RNASEH1","ribonuclease H1","MitoCarta3.0","Mitochondrial central dogma > mtDNA maintenance > mtDNA replication | Mitochondrial central dogma > mtRNA metabolism > mtRNA stability and decay","Matrix",0,"RnaseH","DNA replication",null,null,null,"FirstInEnsemblFamily",null,"RNASEH1",1,0,0,0,1,1,1,1,1,1,1,1,0,0,0,0,0,0,0,0,0,0,0,0,0,1,0,1,0,0,0,1,0,0,1,0,0,0,0,0,0,0,1,0,0,0,0,1,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,0,0,0,1,1,1,1,1,1,1,1,1,1,1,1,0,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,97,0,0,"Mitochondrial central dogma > mtDNA maintenance > mtDNA replication | Mitochondrial central dogma > mtRNA metabolism > mtRNA stability and decay","Mitochondrial central dogma "," mtDNA maintenance "," mtDNA replication "," Mitochondrial central dogma "," mtRNA metabolism "," mtRNA stability and decay"]
["hsa:4580",4580,"MTX1","MTX|MTXN",1,"metaxin 1","mitochondrial outer membrane | mitochondrion | integral to membrane | mitochondrial inner membrane | membrane",null,"protein transport | protein targeting to mitochondrion","MTX1","metaxin 1","MitoCarta3.0","Protein import, sorting and homeostasis > Protein import and sorting > SAM | Mitochondrial dynamics and surveillance > Intramitochondrial membrane interactions","MOM",1,null,null,"METAXIN 1 MITOCHONDRIAL OUTER MEMBRANE IMPORT COMPLEX 1","ENSFM00250000002187",2,"FirstInEnsemblFamily",null,"MTX1",0,0,0,0,0,0,1,1,0,0,0,1,0,0,0,0,0,0,0,0,0,0,0,0,0,1,0,0,0,0,0,0,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,0,1,1,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,0,0,1,0,0,0,0,0,0,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,96,0,0,"Protein import, sorting and homeostasis > Protein import and sorting > SAM | Mitochondrial dynamics and surveillance > Intramitochondrial membrane interactions","Protein import, sorting and homeostasis "," Protein import and sorting "," SAM "," Mitochondrial dynamics and surveillance "," Intramitochondrial membrane interactions"]
["hsa:81932",81932,"HDHD3","2810435D12Rik|C9orf158|MGC12904",9,"haloacid dehalogenase-like hydrolase domain containing 3","mitochondrion","hydrolase activity | protein binding | phosphoglycolate phosphatase activity",null,"HDHD3","haloacid dehalogenase-like hydrolase domain containing 3","MitoCarta3.0",0,"Matrix",0,"Hydrolase",null,null,null,null,"FirstInEnsemblFamily",null,"HDHD3",1,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,0,0,0,0,0,0,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,0,0,1,0,0,1,1,1,1,0,0,1,1,1,0,1,1,0,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,0,1,1,1,1,1,1,0,0,1,1,1,1,1,1,1,0,0,0,0,0,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,0,1,1,1,1,94,0,0,0,0]
["hsa:388753",388753,"C1orf31","-",1,"chromosome 1 open reading frame 31","mitochondrion","cytochrome-c oxidase activity",null,"C1orf31","chromosome 1 open reading frame 31","MitoCarta3.0","OXPHOS > Complex IV > CIV assembly factors | OXPHOS > OXPHOS assembly factors","IMS",0,null,null,null,null,null,"FirstInEnsemblFamily",null,"C1orf31",0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,0,1,0,0,1,1,0,1,1,1,1,1,1,1,0,1,0,1,1,1,1,0,1,1,0,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,0,0,1,1,0,0,1,0,0,0,0,1,0,1,1,1,0,1,1,0,1,1,1,1,1,1,1,91,0,0,"OXPHOS > Complex IV > CIV assembly factors | OXPHOS > OXPHOS assembly factors","OXPHOS "," Complex IV "," CIV assembly factors "," OXPHOS "," OXPHOS assembly factors"]
["hsa:1337",1337,"COX6A1","COX6A|COX6AL|MGC104500",12,"cytochrome c oxidase subunit VIa polypeptide 1","mitochondrion | mitochondrial inner membrane | membrane | mitochondrial respiratory chain complex IV","cytochrome-c oxidase activity","generation of precursor metabolites and energy | respiratory electron transport chain","COX6A1","cytochrome c oxidase subunit VIa polypeptide 1","MitoCarta3.0","OXPHOS > Complex IV > CIV subunits | OXPHOS > OXPHOS subunits","MIM",1,"COX6A","Oxidative phosphorylation",null,null,null,"FirstInEnsemblFamily",null,"COX6A1",0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,0,0,0,0,0,0,0,0,0,1,1,0,1,0,0,1,1,0,1,0,0,1,1,0,1,1,1,1,1,0,1,1,0,1,1,1,1,1,1,0,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,90,0,0,"OXPHOS > Complex IV > CIV subunits | OXPHOS > OXPHOS subunits","OXPHOS "," Complex IV "," CIV subunits "," OXPHOS "," OXPHOS subunits"]
["hsa:58472",58472,"SQRDL","-",15,"sulfide quinone reductase-like (yeast)","mitochondrion | mitochondrial inner membrane | cytoplasm","oxidoreductase activity",null,"SQRDL","sulfide quinone reductase-like (yeast)","MitoCarta3.0","Metabolism > Electron carriers > Q-linked reactions, other | Metabolism > Sulfur metabolism","MIM",0,"Pyr_redox_2",null,null,null,null,"FirstInEnsemblFamily",null,"SQRDL",1,0,0,0,0,0,1,1,1,1,1,0,0,0,0,0,0,0,0,0,0,0,0,1,1,1,1,0,0,0,0,0,0,0,0,0,1,0,0,0,1,1,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,0,0,1,0,0,0,1,1,0,0,0,0,0,0,1,0,0,0,0,0,1,1,1,1,1,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,88,0,0,"Metabolism > Electron carriers > Q-linked reactions, other | Metabolism > Sulfur metabolism","Metabolism "," Electron carriers "," Q-linked reactions, other "," Metabolism "," Sulfur metabolism"]
["hsa:51499",51499,"TRIAP1","P53CSV|WF-1",12,"TP53 regulated inhibitor of apoptosis 1","mitochondrion | perinuclear region of cytoplasm | cytoplasm","protein binding | caspase inhibitor activity","DNA damage response, signal transduction by p53 class mediator | apoptosis | negative regulation of caspase activity | DNA damage response, signal transduction by p53 class mediator resulting in cell cycle arrest | anti-apoptosis","TRIAP1","TP53 regulated inhibitor of apoptosis 1","MitoCarta3.0","Metabolism > Lipid metabolism > Cardiolipin synthesis | Metabolism > Lipid metabolism > Phospholipid metabolism","IMS",0,"UPF0203",null,null,null,null,"FirstInEnsemblFamily",null,"TRIAP1",0,0,0,0,0,0,0,0,0,0,0,1,1,0,1,0,0,1,1,1,1,1,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,1,1,1,1,1,0,1,1,1,0,0,1,1,1,0,0,0,1,1,1,1,1,1,1,0,1,1,0,0,0,0,0,0,1,1,0,0,1,0,0,1,1,1,0,1,0,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,0,1,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,86,0,0,"Metabolism > Lipid metabolism > Cardiolipin synthesis | Metabolism > Lipid metabolism > Phospholipid metabolism","Metabolism "," Lipid metabolism "," Cardiolipin synthesis "," Metabolism "," Lipid metabolism "," Phospholipid metabolism"]
["hsa:64064",64064,"OXCT2","FLJ00030|SCOTT",1,"3-oxoacid CoA transferase 2","mitochondrion | microtubule-based flagellum","transferase activity | 3-oxoacid CoA-transferase activity","ketone body catabolic process | metabolic process",null,null,"MitoCarta3.0","Metabolism > Carbohydrate metabolism > Ketone metabolism","Matrix",null,"CoA_trans","Valine, leucine and isoleucine degradation | Synthesis and degradation of ketone bodies | Butanoate metabolism","SUCCINYL COA:3 KETOACID COENZYME A TRANSFERASE 1 MITOCHONDRIAL PRECURSOR EC_2.8.3.5 3 OXOACID COA TRANSFERASE 1 SOMATIC TYPE SUCCINYL COA:3 OXOACID COA TRANSFERASE SCOT S","ENSFM00250000002519",2,null,null,"OXCT2",1,0,0,0,0,0,1,1,1,1,1,0,0,0,0,0,0,0,0,0,0,0,0,1,1,1,1,1,0,1,0,0,0,1,0,0,0,0,0,0,0,0,0,0,0,0,0,1,1,0,0,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,0,1,0,1,1,1,1,1,0,0,0,0,0,0,0,0,0,0,0,1,1,1,1,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,85,0,0,"Metabolism > Carbohydrate metabolism > Ketone metabolism","Metabolism "," Carbohydrate metabolism "," Ketone metabolism"]
["hsa:9804",9804,"TOMM20","KIAA0016|MAS20|MGC117367|MOM19|TOM20",1,"translocase of outer mitochondrial membrane 20 homolog (yeast)","mitochondrial envelope | mitochondrion | integral to membrane | mitochondrial outer membrane translocase complex | membrane","unfolded protein binding | protein binding | P-P-bond-hydrolysis-driven protein transmembrane transporter activity","protein targeting to mitochondrion","TOMM20","translocase of outer mitochondrial membrane 20 homolog (yeast)","MitoCarta3.0","Protein import, sorting and homeostasis > Protein import and sorting > TOM | Mitochondrial dynamics and surveillance","MOM",1,"MAS20",null,null,null,null,"FirstInEnsemblFamily",null,"TOMM20",0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,1,0,1,1,1,1,1,0,1,1,1,0,0,1,1,1,1,1,1,1,1,1,1,0,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,83,0,0,"Protein import, sorting and homeostasis > Protein import and sorting > TOM | Mitochondrial dynamics and surveillance","Protein import, sorting and homeostasis "," Protein import and sorting "," TOM "," Mitochondrial dynamics and surveillance"]
["hsa:79783",79783,"C7orf10","DERP13|FLJ11808|FLJ53872|ORF19",7,"chromosome 7 open reading frame 10",null,"transferase activity | catalytic activity",null,"C7orf10","chromosome 7 open reading frame 10","MitoCarta3.0","Metabolism > Amino acid metabolism","Matrix",0,"CoA_transf_3",null,null,null,null,"FirstInEnsemblFamily",null,"C7orf10",1,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,1,1,1,0,0,1,0,0,1,1,0,0,0,0,0,0,0,0,0,0,0,0,1,1,1,1,1,0,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,0,1,0,1,0,1,1,1,0,0,0,0,0,0,0,0,0,0,0,1,1,1,1,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,82,0,0,"Metabolism > Amino acid metabolism","Metabolism "," Amino acid metabolism"]
["hsa:56910",56910,"STARD7","GTT1",2,"StAR-related lipid transfer (START) domain containing 7","mitochondrion",null,null,"STARD7","START domain containing 7","MitoCarta3.0","Metabolism > Lipid metabolism > Phospholipid metabolism | Small molecule transport","IMS",0,"START",null,null,null,null,"FirstInEnsemblFamily",null,"STARD7",0,0,1,1,0,0,1,1,1,1,1,1,1,1,1,1,1,0,1,0,0,0,0,1,1,1,0,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,1,1,0,0,1,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,82,0,0,"Metabolism > Lipid metabolism > Phospholipid metabolism | Small molecule transport","Metabolism "," Lipid metabolism "," Phospholipid metabolism "," Small molecule transport"]
["hsa:55013",55013,"CCDC109B","FLJ20647",4,"coiled-coil domain containing 109B","integral to membrane | membrane",null,null,"CCDC109B","coiled-coil domain containing 109B","MitoCarta3.0","Signaling > Calcium homeostasis > Calcium cycle | Small molecule transport > Calcium uniporter","MIM",2,"DUF607",null,"CALCIUM UNIPORTER PROTEIN MITOCHONDRIAL COILED COIL DOMAIN CONTAINING 109A","ENSFM00250000002939",2,null,null,"CCDC109B",0,0,0,0,0,0,1,1,1,1,1,0,0,0,0,0,0,0,0,0,0,0,0,1,1,1,0,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,0,1,1,1,0,0,1,1,1,0,1,1,1,0,0,0,1,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,79,0,0,"Signaling > Calcium homeostasis > Calcium cycle | Small molecule transport > Calcium uniporter","Signaling "," Calcium homeostasis "," Calcium cycle "," Small molecule transport "," Calcium uniporter"]
["hsa:1339",1339,"COX6A2","COX6AH|COXVIAH",16,"cytochrome c oxidase subunit VIa polypeptide 2","mitochondrion | mitochondrial inner membrane | membrane | mitochondrial respiratory chain complex IV","cytochrome-c oxidase activity","generation of precursor metabolites and energy","COX6A2","cytochrome c oxidase subunit VIa polypeptide 2","MitoCarta3.0","OXPHOS > Complex IV > CIV subunits | OXPHOS > OXPHOS subunits","MIM",0,"COX6A","Oxidative phosphorylation",null,null,null,"FirstInEnsemblFamily",null,"COX6A2",0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,0,0,1,1,0,1,0,0,0,0,0,1,1,0,1,1,1,0,1,1,0,1,1,1,1,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,78,0,0,"OXPHOS > Complex IV > CIV subunits | OXPHOS > OXPHOS subunits","OXPHOS "," Complex IV "," CIV subunits "," OXPHOS "," OXPHOS subunits"]
["hsa:345778",345778,"MTX3","DKFZp686O1788",5,"metaxin 3","mitochondrial outer membrane | mitochondrion | membrane",null,"protein transport | protein targeting to mitochondrion",null,null,"MitoCarta3.0","Mitochondrial dynamics and surveillance > Intramitochondrial membrane interactions","MOM",null,null,null,"METAXIN 1 MITOCHONDRIAL OUTER MEMBRANE IMPORT COMPLEX 1","ENSFM00250000002187",2,null,null,"MTX3",0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,0,0,0,0,0,0,1,1,1,0,1,0,1,1,1,1,1,1,0,1,1,1,0,1,1,0,0,0,0,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,0,1,1,0,1,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,1,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,77,0,0,"Mitochondrial dynamics and surveillance > Intramitochondrial membrane interactions","Mitochondrial dynamics and surveillance "," Intramitochondrial membrane interactions"]
["hsa:23530",23530,"NNT","MGC126502|MGC126503",5,"nicotinamide nucleotide transhydrogenase","mitochondrial respiratory chain | mitochondrion | integral to membrane | mitochondrial inner membrane | membrane","NAD(P)+ transhydrogenase (AB-specific) activity | NAD(P)+ transhydrogenase activity | NAD(P)+ transhydrogenase (B-specific) activity | oxidoreductase activity | NAD binding | NADP binding","oxidation-reduction process | proton transport | tricarboxylic acid cycle","NNT","nicotinamide nucleotide transhydrogenase","MitoCarta3.0","Metabolism > Metals and cofactors > NAD biosynthesis and metabolism","MIM",12,"AlaDh_PNT_N | PNTB | AlaDh_PNT_C","Nicotinate and nicotinamide metabolism",null,null,null,"FirstInEnsemblFamily","Glucocorticoid deficiency 4","NNT",1,0,1,1,0,1,0,0,0,0,0,1,1,1,0,0,0,1,1,1,1,1,1,1,1,1,1,1,0,0,0,0,1,1,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,0,0,0,0,0,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,1,1,1,1,0,1,1,1,1,1,0,0,1,1,0,0,1,1,1,1,0,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,76,0,0,"Metabolism > Metals and cofactors > NAD biosynthesis and metabolism","Metabolism "," Metals and cofactors "," NAD biosynthesis and metabolism"]
["hsa:5264",5264,"PHYH","LN1|LNAP1|PAHX|PHYH1|RD",10,"phytanoyl-CoA 2-hydroxylase","mitochondrion | peroxisomal matrix | peroxisome","electron carrier activity | oxidoreductase activity, acting on single donors with incorporation of molecular oxygen, incorporation of two atoms of oxygen | oxidoreductase activity | phytanoyl-CoA dioxygenase activity | L-ascorbic acid binding | catalytic activity | metal ion binding","cellular lipid metabolic process | fatty acid alpha-oxidation | nervous system development | lipid metabolic process","PHYH","phytanoyl-CoA 2-hydroxylase","MitoCarta3.0","Metabolism > Lipid metabolism","Matrix",0,"PhyH","Peroxisome",null,null,null,"FirstInEnsemblFamily","Refsum disease","PHYH",1,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,1,1,0,0,0,1,0,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,0,0,1,0,0,1,0,0,1,1,1,0,1,1,0,0,0,0,0,0,0,0,0,0,0,0,0,1,1,1,1,1,1,1,1,1,1,0,0,0,0,0,0,0,0,0,0,0,1,1,0,1,0,0,1,1,1,1,1,1,1,1,1,0,1,1,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,75,0,0,"Metabolism > Lipid metabolism","Metabolism "," Lipid metabolism"]
["hsa:25934",25934,"NIPSNAP3A","DKFZp564D177|FLJ13953|MGC14553|NIPSNAP4|TASSC",9,"nipsnap homolog 3A (C. elegans)","cytosol | cytoplasm",null,null,"NIPSNAP3A","nipsnap homolog 3A (C. elegans)","MitoCarta3.0",0,"Matrix",0,"NIPSNAP",null,"NIPSNAP HOMOLOG 3A NIPSNAP3A","ENSFM00250000007263",2,"FirstInEnsemblFamily",null,"NIPSNAP3A",1,0,0,0,0,0,0,1,1,1,1,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,1,0,0,1,1,0,1,0,0,0,0,0,0,0,0,0,0,0,0,0,1,1,1,1,0,1,1,1,1,1,0,0,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,0,0,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,73,0,0,0,0]
["hsa:55335",55335,"NIPSNAP3B","FLJ11275|NIPSNAP3",9,"nipsnap homolog 3B (C. elegans)",null,null,null,"NIPSNAP3B","nipsnap homolog 3B (C. elegans)","MitoCarta3.0",0,"Matrix",0,"NIPSNAP",null,"NIPSNAP HOMOLOG 3A NIPSNAP3A","ENSFM00250000007263",2,null,null,"NIPSNAP3B",1,0,0,0,0,0,0,1,1,1,1,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,1,0,0,1,1,0,1,0,0,0,0,0,0,0,0,0,0,0,0,0,1,1,1,1,0,1,1,1,1,1,0,0,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,0,0,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,73,0,0,0,0]
["hsa:140823",140823,"ROMO1","C20orf52|MGC111180|MTGMP|bA353C18.2",20,"reactive oxygen species modulator 1","mitochondrion | integral to membrane | mitochondrial membrane | membrane","molecular_function","positive regulation of reactive oxygen species metabolic process | positive regulation of cell proliferation | replicative cell aging | cellular response to reactive oxygen species","C20orf52","chromosome 20 open reading frame 52","MitoCarta3.0","Protein import, sorting and homeostasis > Protein import and sorting > TIM23 presequence pathway | Small molecule transport","MIM",1,null,null,null,null,null,"FirstInEnsemblFamily",null,"ROMO1",0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,1,1,1,0,1,1,1,1,0,1,1,0,0,1,1,1,1,1,1,1,1,1,1,1,1,0,1,0,0,0,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,0,1,0,1,0,1,1,0,0,1,1,0,0,0,1,1,1,1,1,0,1,1,0,1,1,1,1,1,1,1,1,0,0,1,1,1,1,1,1,71,0,0,"Protein import, sorting and homeostasis > Protein import and sorting > TIM23 presequence pathway | Small molecule transport","Protein import, sorting and homeostasis "," Protein import and sorting "," TIM23 presequence pathway "," Small molecule transport"]
["hsa:26027",26027,"ACOT11","BFIT|DKFZp667O1916|KIAA0707|STARD14|THEA|THEM1",1,"acyl-CoA thioesterase 11","intracellular | cytoplasm","hydrolase activity | acyl-CoA thioesterase activity | carboxylesterase activity","response to cold | response to temperature stimulus | fatty acid metabolic process | intracellular signal transduction","ACOT11","acyl-CoA thioesterase 11","MitoCarta3.0","Metabolism > Lipid metabolism > Fatty acid oxidation","Matrix",0,"4HBT | START",null,"ACYL COENZYME A THIOESTERASE 12 ACYL COA THIOESTERASE 12 EC_3.1.2.1 ACYL COA THIOESTER HYDROLASE 12 CYTOPLASMIC ACETYL COA HYDROLASE 1 CACH 1 1","ENSFM00250000003357",2,"FirstInEnsemblFamily",null,"ACOT11",1,0,0,0,0,0,1,0,1,1,1,0,0,0,0,0,0,0,0,0,0,0,0,1,0,1,1,1,0,0,0,0,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,0,0,0,0,0,0,0,1,1,0,0,0,0,0,0,0,0,0,0,0,1,0,0,0,1,1,1,0,1,1,0,0,0,0,0,1,1,1,1,0,0,0,0,0,0,0,1,1,1,1,1,0,1,1,1,0,0,1,0,0,0,1,1,0,0,0,1,1,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,69,0,0,"Metabolism > Lipid metabolism > Fatty acid oxidation","Metabolism "," Lipid metabolism "," Fatty acid oxidation"]
["hsa:64968",64968,"MRPS6","C21orf101|MRP-S6|RPMS6|S6mt",21,"mitochondrial ribosomal protein S6","mitochondrion | small ribosomal subunit","structural constituent of ribosome | rRNA binding","translation","MRPS6","mitochondrial ribosomal protein S6","MitoCarta3.0","Mitochondrial central dogma > Translation > Mitochondrial ribosome","Matrix",0,null,null,null,null,null,"FirstInEnsemblFamily",null,"MRPS6",1,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,1,0,1,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,1,0,0,1,0,1,0,1,0,0,1,1,0,1,1,0,0,1,1,0,0,0,0,0,1,1,0,0,0,0,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,0,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,0,1,1,1,1,1,0,0,1,1,1,1,0,1,1,1,1,1,68,0,0,"Mitochondrial central dogma > Translation > Mitochondrial ribosome","Mitochondrial central dogma "," Translation "," Mitochondrial ribosome"]
["hsa:64981",64981,"MRPL34","L34mt|MGC24974|MGC2633",19,"mitochondrial ribosomal protein L34","mitochondrion | ribosome | mitochondrial ribosome | intracellular","structural constituent of ribosome","translation","MRPL34","mitochondrial ribosomal protein L34","MitoCarta3.0","Mitochondrial central dogma > Translation > Mitochondrial ribosome","Matrix",0,"Ribosomal_L34",null,null,null,null,"FirstInEnsemblFamily",null,"MRPL34",1,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,0,1,1,1,1,1,1,0,0,1,1,0,0,1,1,0,1,0,1,0,1,1,1,1,0,0,0,0,0,0,0,1,0,0,1,1,1,0,0,0,0,1,0,1,1,0,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,0,0,0,0,1,0,0,1,1,0,0,1,1,1,1,1,1,0,0,0,0,1,1,1,0,1,1,1,1,1,1,0,1,1,1,1,67,0,0,"Mitochondrial central dogma > Translation > Mitochondrial ribosome","Mitochondrial central dogma "," Translation "," Mitochondrial ribosome"]
["hsa:2647",2647,"BLOC1S1","BLOS1|FLJ39337|FLJ97089|GCN5L1|MGC87455|MICoA|RT14",12,"biogenesis of lysosomal organelles complex-1, subunit 1","lysosomal membrane | BLOC-1 complex","protein binding","post-Golgi vesicle-mediated transport | cellular membrane organization | platelet dense granule organization | melanosome organization","BLOC1S1","biogenesis of lysosome-related organelles complex-1, subunit 1","MitoCarta3.0","Signaling","unknown",0,"GCN5L1",null,null,null,null,"FirstInEnsemblFamily",null,"BLOC1S1",0,0,0,0,0,1,0,1,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,0,0,0,0,1,1,1,0,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,0,1,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,1,0,1,0,0,1,1,0,0,0,1,1,1,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,0,0,1,0,1,1,1,1,1,65,0,0,"Signaling","Signaling"]
["hsa:192111",192111,"PGAM5","BXLBV68|MGC5352",12,"phosphoglycerate mutase family member 5","mitochondrial outer membrane | mitochondrion | integral to membrane | membrane","hydrolase activity | phosphoprotein phosphatase activity",null,"PGAM5","phosphoglycerate mutase family member 5","MitoCarta3.0","Mitochondrial dynamics and surveillance > Mitophagy | Mitochondrial dynamics and surveillance > Autophagy","MOM",1,"PGAM",null,null,null,null,"FirstInEnsemblFamily",null,"PGAM5",1,0,0,0,0,0,1,1,1,1,1,1,1,1,0,0,0,1,1,1,1,1,1,0,0,1,1,1,0,0,1,1,0,0,0,0,1,0,1,0,0,0,0,0,1,0,1,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,0,0,0,0,0,1,1,1,1,1,0,0,0,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,63,0,0,"Mitochondrial dynamics and surveillance > Mitophagy | Mitochondrial dynamics and surveillance > Autophagy","Mitochondrial dynamics and surveillance "," Mitophagy ",null," Mitochondrial dynamics and surveillance "," Autophagy"]
["hsa:114294",114294,"LACTB","FLJ14902|G24|MRPL56",15,"lactamase, beta","mitochondrion","hydrolase activity",null,"LACTB","lactamase, beta","MitoCarta3.0","Protein import, sorting and homeostasis > Protein homeostasis > Proteases | Metabolism > Lipid metabolism > Phospholipid metabolism","IMS",0,"Beta-lactamase",null,null,null,null,"FirstInEnsemblFamily",null,"LACTB",1,0,0,0,0,1,0,0,0,0,0,0,0,0,0,0,1,0,0,0,0,0,0,1,1,0,0,0,0,1,0,0,0,0,1,0,0,0,0,0,0,0,0,0,0,0,1,0,1,1,0,0,1,0,0,0,1,0,0,0,0,1,0,0,0,1,1,1,1,1,1,1,1,1,1,1,1,0,1,0,1,1,0,1,1,1,1,1,0,0,0,0,0,0,0,0,0,0,0,1,0,1,1,1,1,1,1,1,0,1,0,0,0,0,0,0,0,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,62,0,0,"Protein import, sorting and homeostasis > Protein homeostasis > Proteases | Metabolism > Lipid metabolism > Phospholipid metabolism","Protein import, sorting and homeostasis "," Protein homeostasis "," Proteases "," Metabolism "," Lipid metabolism "," Phospholipid metabolism"]
["hsa:84908",84908,"FAM136A","FLJ14668",2,"family with sequence similarity 136, member A","mitochondrion | cytoplasm",null,null,"FLJ14668","hypothetical protein FLJ14668","MitoCarta3.0",0,"IMS",0,"DUF842",null,null,null,null,"FirstInEnsemblFamily",null,"FAM136A",0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,1,1,1,1,1,0,1,1,0,0,1,0,1,0,0,1,1,1,0,1,1,1,1,1,1,1,1,0,1,1,1,1,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,61,0,0,0,0]
["hsa:150763",150763,"GPAT2","CT123",2,"glycerol-3-phosphate acyltransferase 2, mitochondrial","mitochondrial outer membrane | mitochondrion | integral to membrane | membrane","glycerol-3-phosphate O-acyltransferase activity | transferase activity, transferring acyl groups","triglyceride biosynthetic process | glycerol-3-phosphate metabolic process | metabolic process | phospholipid biosynthetic process","LOC150763","hypothetical protein LOC150763","MitoCarta3.0","Metabolism > Lipid metabolism > Cardiolipin synthesis | Metabolism > Lipid metabolism > Phospholipid metabolism","MOM",0,"Acyltransferase","Glycerophospholipid metabolism | Glycerolipid metabolism","GLYCEROL 3 PHOSPHATE ACYLTRANSFERASE MITOCHONDRIAL GPAT EC_2.3.1.15","ENSFM00600000921257",2,null,null,"GPAT2",0,0,0,0,0,0,0,1,0,0,0,1,0,0,0,0,0,0,0,0,0,0,0,1,1,1,0,0,0,1,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,1,1,1,0,1,0,0,0,0,0,0,0,0,1,1,0,0,1,1,1,1,1,1,1,1,0,1,1,1,1,1,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,1,0,0,0,0,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,60,0,0,"Metabolism > Lipid metabolism > Cardiolipin synthesis | Metabolism > Lipid metabolism > Phospholipid metabolism","Metabolism "," Lipid metabolism "," Cardiolipin synthesis "," Metabolism "," Lipid metabolism "," Phospholipid metabolism"]
["hsa:284486",284486,"THEM5","FLJ37964|MGC126659|MGC138444",1,"thioesterase superfamily member 5","mitochondrion","hydrolase activity",null,"THEM5","thioesterase superfamily member 5","MitoCarta3.0","Metabolism > Lipid metabolism","Matrix",0,"4HBT",null,null,null,null,"FirstInEnsemblFamily",null,"THEM5",1,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,1,0,1,1,0,1,0,0,1,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,1,0,1,1,1,1,1,0,1,1,1,1,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,0,0,0,0,0,0,0,1,1,1,0,0,0,0,1,1,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,1,1,1,1,0,1,1,1,1,0,0,0,1,1,1,1,1,1,1,58,0,0,"Metabolism > Lipid metabolism","Metabolism "," Lipid metabolism"]
["hsa:129607",129607,"CMPK2","TMPK2|TYKi|UMP-CMPK2",2,"cytidine monophosphate (UMP-CMP) kinase 2, mitochondrial","mitochondrion","cytidylate kinase activity | thymidylate kinase activity | transferase activity | UMP kinase activity | kinase activity | nucleotide binding | ATP binding","dTDP biosynthetic process | cellular response to lipopolysaccharide | pyrimidine nucleotide biosynthetic process","LOC129607","hypothetical protein LOC129607","MitoCarta3.0","Metabolism > Nucleotide metabolism > Nucleotide synthesis and processing","Matrix",0,null,"Pyrimidine metabolism",null,null,null,"FirstInEnsemblFamily",null,"CMPK2",1,1,1,0,0,1,0,1,1,1,1,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,0,1,0,0,0,0,0,0,0,0,0,0,1,1,1,0,0,1,0,0,1,1,1,1,1,1,1,1,1,1,1,0,0,0,1,1,1,1,0,0,0,0,0,1,0,0,1,1,1,1,0,1,1,0,1,0,1,0,0,0,0,0,1,1,1,0,0,0,0,0,0,0,0,0,1,0,1,0,0,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,57,0,0,"Metabolism > Nucleotide metabolism > Nucleotide synthesis and processing","Metabolism "," Nucleotide metabolism "," Nucleotide synthesis and processing"]
["hsa:9927",9927,"MFN2","CMT2A|CMT2A2|CPRP1|HSG|KIAA0214|MARF",1,"mitofusin 2","microtubule cytoskeleton | cytosol | mitochondrial outer membrane | mitochondrion | integral to membrane | intrinsic to mitochondrial outer membrane | membrane","hydrolase activity | GTP binding | GTPase activity | nucleotide binding | ubiquitin protein ligase binding","camera-type eye morphogenesis | negative regulation of smooth muscle cell proliferation | blood coagulation | blastocyst formation | cell cycle arrest | protein targeting to mitochondrion | negative regulation of Ras protein signal transduction | mitochondrial fusion | mitochondrion localization | mitochondrial membrane organization","MFN2","mitofusin 2","MitoCarta3.0","Mitochondrial dynamics and surveillance > Fusion | Mitochondrial dynamics and surveillance > Organelle contact sites","MOM",1,"Dynamin_N | Fzo_mitofusin",null,"TRANSMEMBRANE GTPASE","ENSFM00250000001660",2,null,"Charcot-Marie-Tooth disease, type 2A1|\"Charcot-Marie-Tooth disease, type 2A2\"|Hereditary motor and sensory neuropathy VI","MFN2",1,0,0,1,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,1,0,0,1,0,1,1,0,0,0,0,0,1,0,0,0,0,0,0,0,1,0,0,0,0,0,0,0,0,0,0,0,0,1,0,0,0,0,0,0,0,0,0,1,1,1,1,0,0,0,0,0,0,0,1,0,1,0,0,0,0,0,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,56,0,0,"Mitochondrial dynamics and surveillance > Fusion | Mitochondrial dynamics and surveillance > Organelle contact sites","Mitochondrial dynamics and surveillance "," Fusion ",null," Mitochondrial dynamics and surveillance "," Organelle contact sites"]
["hsa:9692",9692,"KIAA0391","MRPP3",14,"KIAA0391","mitochondrion",null,"tRNA processing","KIAA0391","KIAA0391","MitoCarta3.0","Mitochondrial central dogma > mtRNA metabolism > mtRNA granules | Mitochondrial central dogma > mtRNA metabolism > Polycistronic mtRNA processing","Matrix",0,null,null,null,null,null,"FirstInEnsemblFamily",null,"KIAA0391",0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,0,0,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,1,0,1,1,1,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,54,0,0,"Mitochondrial central dogma > mtRNA metabolism > mtRNA granules | Mitochondrial central dogma > mtRNA metabolism > Polycistronic mtRNA processing","Mitochondrial central dogma "," mtRNA metabolism "," mtRNA granules "," Mitochondrial central dogma "," mtRNA metabolism "," Polycistronic mtRNA processing"]
["hsa:4705",4705,"NDUFA10","CI-42KD|CI-42k|FLJ21923|MGC5103",2,"NADH dehydrogenase (ubiquinone) 1 alpha subcomplex, 10, 42kDa","mitochondrion | respiratory chain | mitochondrial inner membrane | mitochondrial respiratory chain complex I | mitochondrial matrix","NADH dehydrogenase (ubiquinone) activity | phosphotransferase activity, alcohol group as acceptor | ATP binding","transport | mitochondrial electron transport, NADH to ubiquinone | response to drug | nucleobase-containing compound metabolic process | respiratory electron transport chain","NDUFA10","NADH dehydrogenase (ubiquinone) 1 alpha subcomplex, 10, 42kDa","MitoCarta3.0","OXPHOS > Complex I > CI subunits | OXPHOS > OXPHOS subunits","MIM",0,"dNK","Oxidative phosphorylation",null,null,null,"FirstInEnsemblFamily",null,"NDUFA10",1,0,0,0,1,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,0,0,0,0,0,1,1,1,0,0,1,1,1,1,1,1,1,1,0,1,0,1,1,1,1,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,54,0,0,"OXPHOS > Complex I > CI subunits | OXPHOS > OXPHOS subunits","OXPHOS "," Complex I "," CI subunits "," OXPHOS "," OXPHOS subunits"]
["hsa:65991",65991,"FUNDC2","DC44|FLJ33773|HCBP6|HCC3|MGC131676|MGC2495|PD03104","X","FUN14 domain containing 2","mitochondrion",null,null,"FUNDC2","FUN14 domain containing 2","MitoCarta3.0","Mitochondrial dynamics and surveillance > Organelle contact sites | Signaling","MOM",0,"FUN14",null,null,null,null,"FirstInEnsemblFamily",null,"FUNDC2",0,0,0,0,0,0,1,0,0,0,0,1,0,0,0,0,0,0,0,0,0,0,0,0,1,1,1,1,0,0,0,0,1,1,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,0,1,1,1,0,1,1,1,1,1,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,0,1,1,0,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,52,0,0,"Mitochondrial dynamics and surveillance > Organelle contact sites | Signaling","Mitochondrial dynamics and surveillance "," Organelle contact sites ",null," Signaling"]
["hsa:57226",57226,"LYRM2","DJ122O8.2|FLJ18193|FLJ18317|FLJ21130|MGC104253",6,"LYR motif containing 2","mitochondrion",null,null,"LYRM2","LYR motif containing 2","MitoCarta3.0","OXPHOS > Complex I > CI assembly factors | OXPHOS > OXPHOS assembly factors","Matrix",0,"Complex1_LYR",null,null,null,null,"FirstInEnsemblFamily",null,"LYRM2",0,0,0,0,0,0,1,1,0,1,1,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,0,0,0,0,0,0,0,0,1,0,0,0,0,0,0,0,0,1,0,0,0,1,1,0,0,0,0,1,0,1,0,0,1,1,1,0,1,0,0,0,0,1,1,1,0,0,1,0,1,0,0,0,0,0,0,0,0,0,0,1,0,0,0,0,0,0,1,0,0,0,0,1,1,0,1,0,0,0,0,0,1,0,0,1,0,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,52,0,0,"OXPHOS > Complex I > CI assembly factors | OXPHOS > OXPHOS assembly factors","OXPHOS "," Complex I "," CI assembly factors "," OXPHOS "," OXPHOS assembly factors"]
["hsa:339983",339983,"NAT8L","CML3|DKFZp434B0650|DKFZp566H184|FLJ37478|MGC117272|NACED|NAT8-LIKE",4,"N-acetyltransferase 8-like (GCN5-related, putative)","mitochondrion | integral to membrane | endoplasmic reticulum | mitochondrial membrane | microsome | membrane | rough endoplasmic reticulum membrane | cytoplasm","aspartate N-acetyltransferase activity | transferase activity, transferring acyl groups","positive regulation of dopamine uptake | metabolic process","NAT8L","N-acetyltransferase 8-like","MitoCarta3.0","Metabolism > Amino acid metabolism > Glutamate metabolism","Membrane",0,"Acetyltransf_1",null,null,null,null,"FirstInEnsemblFamily","N-acetylaspartate deficiency","NAT8L",1,0,1,1,0,0,0,0,0,0,0,1,1,1,0,0,0,0,0,0,0,0,0,0,1,1,1,0,0,1,0,0,1,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,0,0,1,1,0,0,1,1,1,0,1,0,1,1,1,1,0,1,1,1,0,1,1,0,0,1,1,0,1,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,0,0,0,0,0,0,0,0,0,0,0,1,1,0,0,1,0,1,1,0,0,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,51,0,0,"Metabolism > Amino acid metabolism > Glutamate metabolism","Metabolism "," Amino acid metabolism "," Glutamate metabolism"]
["hsa:51537",51537,"MTFP1","MTP18",22,"mitochondrial fission process 1","mitochondrion | integral to membrane | mitochondrial inner membrane | membrane","carbonate dehydratase activity | zinc ion binding","mitochondrial fission | apoptosis","MTP18","mitochondrial protein 18 kDa","MitoCarta3.0","Mitochondrial dynamics and surveillance > Fission","Membrane",0,null,null,null,null,null,"FirstInEnsemblFamily",null,"MTFP1",0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,0,0,0,1,1,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,0,0,1,0,1,1,0,1,1,1,0,0,0,0,0,0,0,0,0,0,1,0,0,0,0,1,0,1,0,1,1,0,0,0,0,1,0,1,0,0,1,0,1,0,0,0,0,0,0,0,0,1,1,0,0,0,1,1,1,0,0,1,1,1,1,1,1,1,1,1,1,1,1,0,0,1,1,1,1,1,1,1,0,1,0,1,1,1,1,1,1,50,0,0,"Mitochondrial dynamics and surveillance > Fission","Mitochondrial dynamics and surveillance "," Fission"]
["hsa:100303755",100303755,"PET117","-",20,"cytochrome c oxidase assembly factor-like",null,"histone acetyltransferase activity","histone acetylation",null,null,"MitoCarta3.0","OXPHOS > Complex IV > CIV assembly factors | OXPHOS > OXPHOS assembly factors","MIM",null,null,null,null,null,null,"FirstInEnsemblFamily",null,"PET117",0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,0,0,0,0,0,1,0,0,0,1,1,1,1,1,1,0,1,1,1,0,0,0,0,0,0,1,0,0,1,1,1,1,1,0,0,1,0,1,1,1,1,1,1,1,1,1,1,1,1,0,1,0,1,0,0,0,0,1,0,1,1,1,0,1,1,1,0,1,1,0,0,0,1,0,1,1,0,0,0,0,1,0,0,0,1,1,0,1,1,49,0,0,"OXPHOS > Complex IV > CIV assembly factors | OXPHOS > OXPHOS assembly factors","OXPHOS "," Complex IV "," CIV assembly factors "," OXPHOS "," OXPHOS assembly factors"]
["hsa:51300",51300,"TIMMDC1","C3orf1|FLJ22597",3,"translocase of inner mitochondrial membrane domain containing 1","mitochondrion | integral to membrane | membrane",null,null,"C3orf1","chromosome 3 open reading frame 1","MitoCarta3.0","OXPHOS > Complex I > CI assembly factors | OXPHOS > OXPHOS assembly factors","MIM",1,"Tim17",null,null,null,null,"FirstInEnsemblFamily",null,"TIMMDC1",0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,0,0,0,0,0,0,1,1,1,1,0,1,1,1,0,0,0,1,1,0,1,0,0,0,0,0,0,0,0,0,0,1,0,0,0,0,0,1,0,0,0,0,1,1,0,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,0,1,0,1,1,1,1,48,0,0,"OXPHOS > Complex I > CI assembly factors | OXPHOS > OXPHOS assembly factors","OXPHOS "," Complex I "," CI assembly factors "," OXPHOS "," OXPHOS assembly factors"]
["hsa:54543",54543,"TOMM7","TOM7",7,"translocase of outer mitochondrial membrane 7 homolog (yeast)","mitochondrion | integral to membrane | mitochondrial outer membrane translocase complex | membrane","protein binding | protein transmembrane transporter activity | P-P-bond-hydrolysis-driven protein transmembrane transporter activity","intracellular protein transport | protein targeting to mitochondrion","TOMM7","translocase of outer mitochondrial membrane 7 homolog (yeast)","MitoCarta3.0","Protein import, sorting and homeostasis > Protein import and sorting > TOM | Mitochondrial dynamics and surveillance > Mitophagy | Mitochondrial dynamics and surveillance > Autophagy","MOM",1,"Tom7",null,null,null,null,"FirstInEnsemblFamily",null,"TOMM7",0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,0,0,0,0,0,1,0,0,0,0,1,1,1,1,0,1,1,1,0,1,1,1,0,0,0,1,0,0,0,1,0,0,0,1,1,1,0,0,0,1,0,1,0,0,0,0,0,0,0,1,0,0,0,1,1,1,0,1,1,1,1,0,1,1,1,1,1,1,0,0,1,0,1,1,1,0,1,1,1,1,0,1,0,1,1,1,1,1,47,0,0,"Protein import, sorting and homeostasis > Protein import and sorting > TOM | Mitochondrial dynamics and surveillance > Mitophagy | Mitochondrial dynamics and surveillance > Autophagy","Protein import, sorting and homeostasis "," Protein import and sorting "," TOM "," Mitochondrial dynamics and surveillance "," Mitophagy ",null," Mitochondrial dynamics and surveillance "," Autophagy"]
["hsa:93974",93974,"ATPIF1","ATPI|ATPIP|IP|MGC1167|MGC8898",1,"ATPase inhibitory factor 1","mitochondrion | cell surface | mitochondrial proton-transporting ATP synthase complex","enzyme inhibitor activity | angiostatin binding | ATPase binding | calmodulin binding | protein homodimerization activity | ATPase inhibitor activity","protein homotetramerization | angiogenesis | generation of precursor metabolites and energy | negative regulation of endothelial cell proliferation | protein homooligomerization | negative regulation of catalytic activity | negative regulation of hydrolase activity | negative regulation of nucleotide metabolic process","ATPIF1","ATPase inhibitory factor 1","MitoCarta3.0","OXPHOS > Complex V > CV subunits | OXPHOS > OXPHOS subunits","Matrix",0,"IATP",null,null,null,null,"FirstInEnsemblFamily",null,"ATPIF1",0,0,0,0,0,0,1,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,0,1,0,0,0,1,0,0,1,0,0,1,0,0,0,0,1,1,1,1,1,0,1,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,0,0,1,1,1,1,1,1,1,45,0,0,"OXPHOS > Complex V > CV subunits | OXPHOS > OXPHOS subunits","OXPHOS "," Complex V "," CV subunits "," OXPHOS "," OXPHOS subunits"]
["hsa:8799",8799,"PEX11B","PEX11-BETA",1,"peroxisomal biogenesis factor 11 beta","mitochondrion | integral to membrane | peroxisomal membrane | peroxisome | membrane | integral to peroxisomal membrane","protein binding","signal transduction | peroxisome fission | peroxisome organization","PEX11B","peroxisomal biogenesis factor 11B","MitoCarta3.0",0,"Membrane",1,"PEX11","Peroxisome",null,null,null,"FirstInEnsemblFamily",null,"PEX11B",0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,1,0,0,1,1,1,0,1,0,0,1,0,1,1,0,1,0,0,0,0,0,0,0,0,0,0,1,0,0,0,1,1,1,0,1,1,0,1,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,1,0,0,0,0,0,0,1,1,1,0,0,1,1,1,1,0,0,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,45,0,0,0,0]
["hsa:51287",51287,"CHCHD8","DKFZp762H1711|E2IG2|MGC117206",11,"coiled-coil-helix-coiled-coil-helix domain containing 8",null,null,null,"CHCHD8","coiled-coil-helix-coiled-coil-helix domain containing 8","MitoCarta3.0","OXPHOS > Complex IV > CIV assembly factors | OXPHOS > OXPHOS assembly factors","IMS",0,"CHCH",null,null,null,null,"FirstInEnsemblFamily",null,"CHCHD8",0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,0,0,0,0,0,0,0,0,0,0,1,0,1,1,0,0,0,0,0,0,0,0,0,1,0,0,0,0,0,0,0,0,1,0,0,0,0,0,1,0,0,0,0,0,1,1,0,0,1,0,1,0,0,0,1,1,0,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,0,0,0,1,1,1,0,0,0,0,1,1,0,0,0,0,0,0,1,0,0,0,0,1,0,0,1,1,0,0,0,1,0,1,1,1,1,0,1,1,44,0,0,"OXPHOS > Complex IV > CIV assembly factors | OXPHOS > OXPHOS assembly factors","OXPHOS "," Complex IV "," CIV assembly factors "," OXPHOS "," OXPHOS assembly factors"]
["hsa:4539",4539,"ND4L","MTND4L","MT","NADH dehydrogenase, subunit 4L (complex I)","mitochondrion | respiratory chain | integral to membrane | mitochondrial inner membrane | mitochondrial respiratory chain complex I | membrane","NADH dehydrogenase (ubiquinone) activity | oxidoreductase activity, acting on NADH or NADPH","transport | mitochondrial electron transport, NADH to ubiquinone | ATP synthesis coupled electron transport | respiratory electron transport chain","ND4L","NADH dehydrogenase subunit 4L","MitoCarta3.0","OXPHOS > Complex I > CI subunits | OXPHOS > OXPHOS subunits","MIM",3,"Oxidored_q2","Oxidative phosphorylation",null,null,null,"FirstInEnsemblFamily",null,"ND4L",0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,0,0,0,0,1,1,0,0,1,0,1,0,0,1,0,0,0,0,0,0,0,0,0,0,0,0,0,1,0,0,0,1,0,0,0,0,0,0,0,0,0,0,1,1,1,0,0,0,0,0,1,1,0,0,0,1,0,1,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,0,0,0,0,0,0,0,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,0,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,44,0,0,"OXPHOS > Complex I > CI subunits | OXPHOS > OXPHOS subunits","OXPHOS "," Complex I "," CI subunits "," OXPHOS "," OXPHOS subunits"]
["hsa:29796",29796,"UQCR10","HSPC051|HSPC151|QCR9|UCCR7.2|UCRC",22,"ubiquinol-cytochrome c reductase, complex III subunit X","mitochondrial envelope | mitochondrion | respiratory chain | mitochondrial inner membrane | membrane","ubiquinol-cytochrome-c reductase activity","transport | mitochondrial electron transport, ubiquinol to cytochrome c | respiratory electron transport chain","UCRC","ubiquinol-cytochrome c reductase complex (7.2 kD)","MitoCarta3.0","OXPHOS > Complex III > CIII subunits | OXPHOS > OXPHOS subunits","MIM",1,"UCR_UQCRX_QCR9","Oxidative phosphorylation",null,null,null,"FirstInEnsemblFamily",null,"UQCR10",0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,1,0,0,0,1,1,0,0,0,0,0,1,0,1,0,0,0,0,1,0,0,1,0,1,0,0,0,0,1,1,1,0,1,0,0,0,0,0,0,0,0,0,0,1,1,0,0,0,0,1,0,0,0,0,0,1,0,0,0,0,0,0,1,1,1,1,1,1,1,1,1,0,1,1,0,0,1,0,1,1,1,0,1,1,1,1,0,1,1,1,1,1,1,1,43,0,0,"OXPHOS > Complex III > CIII subunits | OXPHOS > OXPHOS subunits","OXPHOS "," Complex III "," CIII subunits "," OXPHOS "," OXPHOS subunits"]
["hsa:25994",25994,"HIGD1A","DKFZp564K247|HIG1",3,"HIG1 hypoxia inducible domain family, member 1A","mitochondrion | integral to membrane | protein complex | membrane",null,"response to stress","HIGD1A","HIG1 domain family, member 1A","MitoCarta3.0","OXPHOS > Complex IV > CIV assembly factors | OXPHOS > OXPHOS assembly factors | OXPHOS > Respirasome assembly","MIM",2,"HIG_1_N",null,null,null,null,"FirstInEnsemblFamily",null,"HIGD1A",0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,0,0,0,0,0,0,0,0,0,1,0,1,1,1,1,0,0,0,0,0,0,0,0,0,1,1,0,0,1,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,0,0,0,0,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,42,0,0,"OXPHOS > Complex IV > CIV assembly factors | OXPHOS > OXPHOS assembly factors | OXPHOS > Respirasome assembly","OXPHOS "," Complex IV "," CIV assembly factors "," OXPHOS "," OXPHOS assembly factors ",null," OXPHOS "," Respirasome assembly"]
["hsa:114971",114971,"PTPMT1","DUSP23|FLJ46081|MOSP|PLIP",11,"protein tyrosine phosphatase, mitochondrial 1","mitochondrion | mitochondrial inner membrane | membrane","hydrolase activity | protein tyrosine phosphatase activity | phosphatidylinositol-4,5-bisphosphate 5-phosphatase activity | protein tyrosine/serine/threonine phosphatase activity","inositol phosphate dephosphorylation | protein dephosphorylation",null,null,"MitoCarta3.0","Metabolism > Lipid metabolism > Cardiolipin synthesis | Metabolism > Lipid metabolism > Phospholipid metabolism","MIM",null,null,null,null,null,null,"FirstInEnsemblFamily",null,"PTPMT1",0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,1,1,1,1,1,1,1,1,1,1,1,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,0,1,0,1,0,0,1,1,1,1,1,1,42,0,0,"Metabolism > Lipid metabolism > Cardiolipin synthesis | Metabolism > Lipid metabolism > Phospholipid metabolism","Metabolism "," Lipid metabolism "," Cardiolipin synthesis "," Metabolism "," Lipid metabolism "," Phospholipid metabolism"]
["hsa:205327",205327,"C2orf69","FLJ38973",2,"chromosome 2 open reading frame 69","extracellular region",null,null,"FLJ38973","hypothetical protein FLJ38973","MitoCarta3.0",0,"Matrix",0,null,null,null,null,null,"FirstInEnsemblFamily",null,"C2orf69",0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,1,1,0,0,1,0,0,1,0,0,0,0,0,0,0,0,1,0,1,0,1,0,0,0,0,0,1,1,1,1,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,0,0,0,1,0,1,1,0,1,0,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,41,0,0,0,0]
["hsa:27109",27109,"ATP5S","ATPW|HSU79253",14,"ATP synthase, H+ transporting, mitochondrial Fo complex, subunit s (factor B)","proton-transporting ATP synthase complex, coupling factor F(o) | mitochondrion | mitochondrial inner membrane | membrane","hydrogen ion transmembrane transporter activity","proton transport | ion transport | ATP biosynthetic process","ATP5S","ATP synthase, H+ transporting, mitochondrial F0 complex, subunit s (factor B)","MitoCarta3.0","OXPHOS > Complex V > CV subunits | OXPHOS > OXPHOS subunits","MIM",0,null,null,null,null,null,"FirstInEnsemblFamily",null,"ATP5S",0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,40,0,0,"OXPHOS > Complex V > CV subunits | OXPHOS > OXPHOS subunits","OXPHOS "," Complex V "," CV subunits "," OXPHOS "," OXPHOS subunits"]
["hsa:414919",414919,"C8orf82","MGC70857",8,"chromosome 8 open reading frame 82",null,null,null,"MGC70857","similar to RIKEN cDNA C030006K11 gene","MitoCarta3.0",0,"Matrix",0,null,null,null,null,null,"FirstInEnsemblFamily",null,"C8orf82",0,0,0,0,0,0,1,1,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,0,1,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,0,0,1,0,1,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,1,1,1,0,0,0,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,0,0,1,1,1,1,1,1,1,40,0,0,0,0]
["hsa:51295",51295,"ECSIT","SITPEC",19,"ECSIT homolog (Drosophila)","mitochondrion | transcription factor complex | nucleus | cytoplasm","protein binding | sequence-specific DNA binding transcription factor activity | oxidoreductase activity, acting on NADH or NADPH | signal transducer activity","regulation of oxidoreductase activity | BMP signaling pathway | regulation of transcription from RNA polymerase II promoter | transcription, DNA-dependent | mesoderm formation | innate immune response","ECSIT","ECSIT homolog (Drosophila)","MitoCarta3.0","OXPHOS > Complex I > CI assembly factors | OXPHOS > OXPHOS assembly factors","MIM",0,"ECSIT",null,null,null,null,"FirstInEnsemblFamily",null,"ECSIT",0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,0,1,1,1,1,1,1,1,39,0,0,"OXPHOS > Complex I > CI assembly factors | OXPHOS > OXPHOS assembly factors","OXPHOS "," Complex I "," CI assembly factors "," OXPHOS "," OXPHOS assembly factors"]
["hsa:4594",4594,"MUT","MCM",6,"methylmalonyl CoA mutase","mitochondrion | mitochondrial matrix","cobalamin binding | methylmalonyl-CoA mutase activity | metal ion binding","post-embryonic development | fatty acid beta-oxidation | cellular lipid metabolic process","MUT","methylmalonyl Coenzyme A mutase","MitoCarta3.0","Metabolism > Carbohydrate metabolism > Propanoate metabolism | Metabolism > Lipid metabolism > Fatty acid oxidation | Metabolism > Vitamin metabolism > Vitamin B12 metabolism","Matrix",0,"B12-binding | MM_CoA_mutase","Glyoxylate and dicarboxylate metabolism | Propanoate metabolism | \"Valine, leucine and isoleucine degradation\"",null,null,null,"FirstInEnsemblFamily","Methylmalonic aciduria, mut(0) type|\"Methylmalonic aciduria, vitamin B12-responsive\"|\"Methylmalonic aciduria, vitamin B12-responsive, due to defect in synthesis of adenosylcobalamin, cblB complementation type\"","MUT",1,0,0,0,0,0,0,0,1,1,1,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,1,1,0,1,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,1,1,1,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,1,1,1,0,0,1,1,1,0,0,0,0,0,0,0,0,0,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,39,0,0,"Metabolism > Carbohydrate metabolism > Propanoate metabolism | Metabolism > Lipid metabolism > Fatty acid oxidation | Metabolism > Vitamin metabolism > Vitamin B12 metabolism","Metabolism "," Carbohydrate metabolism "," Propanoate metabolism "," Metabolism "," Lipid metabolism "," Fatty acid oxidation "," Metabolism "," Vitamin metabolism "," Vitamin B12 metabolism"]
["hsa:132158",132158,"GLYCTK","HBEBP2|HBEBP4|HBeAgBP4A",3,"glycerate kinase","mitochondrion | Golgi apparatus | cytoplasm","protein binding | glycerate kinase activity | transferase activity | nucleotide binding | ATP binding","protein phosphorylation","GLYCTK","glycerate kinase","MitoCarta3.0","Metabolism > Carbohydrate metabolism","Matrix",0,"MOFRL","Glyoxylate and dicarboxylate metabolism | Glycerolipid metabolism | \"Glycine, serine and threonine metabolism\"",null,null,null,"FirstInEnsemblFamily","D-glyceric aciduria","GLYCTK",1,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,38,0,0,"Metabolism > Carbohydrate metabolism","Metabolism "," Carbohydrate metabolism"]
["hsa:54332",54332,"GDAP1","CMT4|CMT4A|CMTRIA",8,"ganglioside-induced differentiation-associated protein 1","cytoplasm",null,"response to retinoic acid","GDAP1","ganglioside-induced differentiation-associated protein 1","MitoCarta3.0",0,"MOM",2,"GST_C | GST_N",null,"GANGLIOSIDE INDUCED DIFFERENTIATION ASSOCIATED 1 GDAP1","ENSFM00250000004188",2,"FirstInEnsemblFamily","Charcot-Marie-Tooth disease, axonal, type 2K|\"Charcot-Marie-Tooth disease, axonal, with vocal cord paresis\"|\"Charcot-Marie-Tooth disease, recessive intermediate, A\"|\"Charcot-Marie-Tooth disease, type 4A\"","GDAP1",1,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,1,0,1,1,0,1,1,0,1,1,1,1,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,0,0,0,0,0,0,0,0,0,1,0,0,1,1,1,1,1,1,1,1,1,1,0,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,37,0,0,0,0]
["hsa:56945",56945,"MRPS22","C3orf5|COXPD5|GIBT|MRP-S22|RPMS22",3,"mitochondrial ribosomal protein S22","mitochondrion | ribosome | mitochondrial ribosome | mitochondrial small ribosomal subunit","structural constituent of ribosome","biological_process","MRPS22","mitochondrial ribosomal protein S22","MitoCarta3.0","Mitochondrial central dogma > Translation > Mitochondrial ribosome","Matrix",0,null,null,null,null,null,"FirstInEnsemblFamily","Combined oxidative phosphorylation deficiency 5","MRPS22",0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,37,0,0,"Mitochondrial central dogma > Translation > Mitochondrial ribosome","Mitochondrial central dogma "," Translation "," Mitochondrial ribosome"]
["hsa:841",841,"CASP8","ALPS2B|CAP4|Casp-8|FLICE|FLJ17672|MACH|MCH5|MGC78473",2,"caspase 8, apoptosis-related cysteine peptidase","mitochondrion | membrane raft | centrosome | death-inducing signaling complex | cell body | cytoplasm | neuron projection | CD95 death-inducing signaling complex | mitochondrial outer membrane | cytosol | nucleolus | nucleus | cytoskeleton | Noc1p-Noc2p complex","tumor necrosis factor receptor binding | cysteine-type peptidase activity | peptidase activity | protein binding | cysteine-type endopeptidase activity | identical protein binding","response to antibiotic | response to cold | macrophage differentiation | cellular component disassembly involved in apoptosis | apoptosis | positive regulation of I-kappaB kinase/NF-kappaB cascade | cellular response to mechanical stimulus | proteolysis | protein heterooligomerization | neural tube formation | nucleotide-binding oligomerization domain containing signaling pathway | response to ethanol | response to lipopolysaccharide | induction of apoptosis by extracellular signals | activation of caspase activity | angiogenesis | regulation of apoptosis | innate immune response | response to estradiol stimulus | activation of pro-apoptotic gene products | induction of apoptosis by intracellular signals | proteolysis involved in cellular protein catabolic process | response to tumor necrosis factor | heart development | activation of innate immune response | response to cobalt ion","CASP8","caspase 8, apoptosis-related cysteine peptidase","MitoCarta3.0","Protein import, sorting and homeostasis > Protein homeostasis | Mitochondrial dynamics and surveillance > Apoptosis","MOM",0,"DED | Peptidase_C14","p53 signaling pathway | Apoptosis","CASPASE PRECURSOR CASP APOPTOTIC PROTEASE MCH ICE  APOPTOTIC PROTEASE [CONTAINS CASPASE SUBUNIT CASPASE SUBUNIT","ENSFM00500000269928",2,"FirstInEnsemblFamily","Immunodeficiency due to CASP8 deficiency|Lung cancer","CASP8",0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,36,0,0,"Protein import, sorting and homeostasis > Protein homeostasis | Mitochondrial dynamics and surveillance > Apoptosis","Protein import, sorting and homeostasis "," Protein homeostasis ",null," Mitochondrial dynamics and surveillance "," Apoptosis"]
["hsa:64976",64976,"MRPL40","FLJ41774|MGC9400|MRP-L22|NLVCF|URIM",22,"mitochondrial ribosomal protein L40","mitochondrion | ribosome | mitochondrial ribosome | nucleus","molecular_function","anatomical structure morphogenesis","MRPL40","mitochondrial ribosomal protein L40","MitoCarta3.0","Mitochondrial central dogma > Translation > Mitochondrial ribosome","Matrix",0,null,null,null,null,null,"FirstInEnsemblFamily",null,"MRPL40",0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,0,0,1,0,0,0,0,0,0,0,0,1,0,0,0,0,0,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,0,1,1,1,0,1,1,0,0,0,1,1,1,1,1,1,36,0,0,"Mitochondrial central dogma > Translation > Mitochondrial ribosome","Mitochondrial central dogma "," Translation "," Mitochondrial ribosome"]
["hsa:64432",64432,"MRPS25","DKFZp313H0817|FLJ00023|MRP-S25|RPMS25",3,"mitochondrial ribosomal protein S25","mitochondrion | ribosome | mitochondrial small ribosomal subunit","structural constituent of ribosome","translation","MRPS25","mitochondrial ribosomal protein S25","MitoCarta3.0","Mitochondrial central dogma > Translation > Mitochondrial ribosome","Matrix",0,"L51_S25_CI-B8",null,null,null,null,"FirstInEnsemblFamily",null,"MRPS25",0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,36,0,0,"Mitochondrial central dogma > Translation > Mitochondrial ribosome","Mitochondrial central dogma "," Translation "," Mitochondrial ribosome"]
["hsa:8834",8834,"TMEM11","C17orf35|PM1|PMI",17,"transmembrane protein 11","mitochondrion | mitochondrial inner membrane | integral to plasma membrane | membrane | integral to mitochondrial inner membrane","protein binding | molecular_function","mitochondrion organization","TMEM11","transmembrane protein 11","MitoCarta3.0","Mitochondrial dynamics and surveillance","MIM",0,null,null,null,null,null,"FirstInEnsemblFamily",null,"TMEM11",0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,1,1,1,1,0,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,36,0,0,"Mitochondrial dynamics and surveillance","Mitochondrial dynamics and surveillance"]
["hsa:51263",51263,"MRPL30","FLJ44438|L28MT|L30MT|MGC24095|MGC3314|MRP-L28|MRP-L30|MRPL28|MRPL28M|RPML28",2,"mitochondrial ribosomal protein L30","mitochondrion | ribosome","structural constituent of ribosome","translation","MRPL30","mitochondrial ribosomal protein L30","MitoCarta3.0","Mitochondrial central dogma > Translation > Mitochondrial ribosome","Matrix",0,null,null,"39S RIBOSOMAL L30 MITOCHONDRIAL PRECURSOR L30MT MRP L30","ENSFM00500000273719",2,"FirstInEnsemblFamily",null,"MRPL30",0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,1,1,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,35,0,0,"Mitochondrial central dogma > Translation > Mitochondrial ribosome","Mitochondrial central dogma "," Translation "," Mitochondrial ribosome"]
["hsa:4697",4697,"NDUFA4","CI-9k|CI-MLRQ|FLJ27440|MGC104422|MGC126843|MGC126845|MLRQ",7,"NADH dehydrogenase (ubiquinone) 1 alpha subcomplex, 4, 9kDa","mitochondrion | respiratory chain | mitochondrial inner membrane | mitochondrial respiratory chain complex I | membrane","NADH dehydrogenase (ubiquinone) activity","transport | mitochondrial electron transport, NADH to ubiquinone | respiratory electron transport chain","NDUFA4","NADH dehydrogenase (ubiquinone) 1 alpha subcomplex, 4, 9kDa","MitoCarta3.0","OXPHOS > Complex IV > CIV subunits | OXPHOS > OXPHOS subunits","MIM",1,null,"Oxidative phosphorylation",null,null,null,"FirstInEnsemblFamily",null,"NDUFA4",0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,0,0,1,1,0,1,1,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,0,1,0,0,0,0,1,1,1,0,1,1,1,1,1,1,1,1,0,0,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,35,0,0,"OXPHOS > Complex IV > CIV subunits | OXPHOS > OXPHOS subunits","OXPHOS "," Complex IV "," CIV subunits "," OXPHOS "," OXPHOS subunits"]
["hsa:10240",10240,"MRPS31","IMOGN38|MRP-S31|S31mt",13,"mitochondrial ribosomal protein S31","mitochondrion | ribosome","protein domain specific binding",null,"MRPS31","mitochondrial ribosomal protein S31","MitoCarta3.0","Mitochondrial central dogma > Translation > Mitochondrial ribosome","Matrix",0,null,null,null,null,null,"FirstInEnsemblFamily",null,"MRPS31",0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,34,0,0,"Mitochondrial central dogma > Translation > Mitochondrial ribosome","Mitochondrial central dogma "," Translation "," Mitochondrial ribosome"]
["hsa:9551",9551,"ATP5J2","ATP5JL",7,"ATP synthase, H+ transporting, mitochondrial Fo complex, subunit F2","proton-transporting ATP synthase complex, coupling factor F(o) | mitochondrion | mitochondrial inner membrane | membrane | mitochondrial proton-transporting ATP synthase complex","transmembrane transporter activity | ATPase activity","proton transport | ion transport | mitochondrial ATP synthesis coupled proton transport | ATP catabolic process | respiratory electron transport chain | ATP biosynthetic process","ATP5J2","ATP synthase, H+ transporting, mitochondrial F0 complex, subunit F2","MitoCarta3.0","OXPHOS > Complex V > CV subunits | OXPHOS > OXPHOS subunits","MIM",1,null,"Oxidative phosphorylation",null,null,null,"FirstInEnsemblFamily",null,"ATP5J2",0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,0,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,33,0,0,"OXPHOS > Complex V > CV subunits | OXPHOS > OXPHOS subunits","OXPHOS "," Complex V "," CV subunits "," OXPHOS "," OXPHOS subunits"]
["hsa:596",596,"BCL2","Bcl-2|PPP1R50",18,"B-cell CLL/lymphoma 2","mitochondrion | endoplasmic reticulum | myelin sheath | intracellular | cytoplasm | pore complex | nuclear membrane | cytosol | mitochondrial outer membrane | nucleus | endoplasmic reticulum membrane | microsome | membrane | membrane fraction","sequence-specific DNA binding | protein phosphatase 2A binding | protein heterodimerization activity | protease binding | channel activity | protein binding | protein homodimerization activity | BH3 domain binding | transcription factor binding | identical protein binding | ubiquitin protein ligase binding","mesenchymal cell development | cell-cell adhesion | glomerulus development | peptidyl-serine phosphorylation | response to toxin | negative regulation of osteoblast proliferation | T cell differentiation in thymus | apoptosis | positive regulation of developmental pigmentation | negative regulation of cell migration | response to estrogen stimulus | cell death | actin filament organization | positive regulation of cell growth | response to inorganic substance | response to gamma radiation | spleen development | response to L-ascorbic acid | regulation of protein heterodimerization activity | CD8-positive, alpha-beta T cell lineage commitment | post-embryonic development | immune system development | B cell proliferation | positive regulation of catalytic activity | cochlear nucleus development | positive regulation of melanocyte differentiation | cell aging | branching involved in ureteric bud morphogenesis | activation of pro-apoptotic gene products | positive regulation of B cell proliferation | humoral immune response | homeostasis of number of cells within a tissue | oligodendrocyte differentiation | negative regulation of cellular pH reduction | hemopoiesis | T cell lineage commitment | response to cytokine stimulus | response to iron ion | negative regulation of mitotic cell cycle | male gonad development | negative regulation of ossification | response to drug | axon regeneration | protein dephosphorylation | apoptosis in response to endoplasmic reticulum stress | negative regulation of cell growth | oocyte development | organ morphogenesis | regulation of mitochondrial membrane permeability | response to ethanol | B cell lineage commitment | regulation of nitrogen utilization | regulation of cell cycle | regulation of protein localization | regulation of cell-matrix adhesion | response to DNA damage stimulus | response to corticosterone stimulus | thymus development | innate immune response | regulation of transmembrane transporter activity | regulation of mitochondrial membrane potential | T cell homeostasis | protein polyubiquitination | activation of innate immune response | defense response to virus | response to oxidative stress | positive regulation of neuron maturation | pigment granule organization | response to caffeine | renal system process | reactive oxygen species metabolic process | negative regulation of myeloid cell apoptosis | regulation of protein stability | metanephros development | response to external stimulus | focal adhesion assembly | ovarian follicle development | negative regulation of neuron apoptosis | digestive tract morphogenesis | negative regulation of retinal cell programmed cell death | positive regulation of multicellular organism growth | behavioral fear response | response to UV-B | cell morphogenesis | organ growth | response to nicotine | response to radiation | negative regulation of mitochondrial depolarization | response to folic acid | negative regulation of apoptosis | positive regulation of smooth muscle cell migration | B cell receptor signaling pathway | response to hydrogen peroxide | response to insulin stimulus | melanin metabolic process | ear development | regulation of calcium ion transport | neuron apoptosis | cellular calcium ion homeostasis | response to aluminum ion | ureteric bud development | regulation of protein homodimerization activity | release of cytochrome c from mitochondria | G1/S transition of mitotic cell cycle | glial cell apoptosis | B cell homeostasis | response to nutrient | lymphoid progenitor cell differentiation | anti-apoptosis | positive regulation of skeletal muscle fiber development | regulation of gene expression | response to hypoxia | developmental growth | regulation of glycoprotein biosynthetic process | brain development | female pregnancy | induction of apoptosis by intracellular signals | response to heat | response to copper ion | peptidyl-threonine phosphorylation | gland morphogenesis","BCL2","B-cell CLL/lymphoma 2","MitoCarta3.0","Mitochondrial dynamics and surveillance > Apoptosis","MOM",1,"Bcl-2 | BH4","Focal adhesion | Apoptosis | Protein processing in endoplasmic reticulum",null,null,null,"FirstInEnsemblFamily",null,"BCL2",0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,0,1,0,1,0,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,32,0,0,"Mitochondrial dynamics and surveillance > Apoptosis","Mitochondrial dynamics and surveillance "," Apoptosis"]
["hsa:283459",283459,"GATC","15E1.2|FLJ37000|MGC129938",12,"glutamyl-tRNA(Gln) amidotransferase, subunit C homolog (bacterial)","mitochondrion | glutamyl-tRNA(Gln) amidotransferase complex","glutaminyl-tRNA synthase (glutamine-hydrolyzing) activity | nucleotide binding | ligase activity | ATP binding","glutaminyl-tRNAGln biosynthesis via transamidation | regulation of translational fidelity | mitochondrial translation","15E1.2","hypothetical protein LOC283459","MitoCarta3.0","Mitochondrial central dogma > Translation > mt-tRNA synthetases","Matrix",0,null,null,null,null,null,"FirstInEnsemblFamily",null,"GATC",1,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,0,1,1,1,1,0,1,0,1,1,1,1,1,0,1,1,1,1,1,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,32,0,0,"Mitochondrial central dogma > Translation > mt-tRNA synthetases","Mitochondrial central dogma "," Translation "," mt-tRNA synthetases"]
["hsa:28957",28957,"MRPS28","FLJ22853|MRP-S28|MRP-S35|MRPS35",8,"mitochondrial ribosomal protein S28","mitochondrion | ribosome | mitochondrial small ribosomal subunit","molecular_function","biological_process","MRPS28","mitochondrial ribosomal protein S28","MitoCarta3.0","Mitochondrial central dogma > Translation > Mitochondrial ribosome","Matrix",0,null,null,"TUMOR D52","ENSFM00500000271184",2,"FirstInEnsemblFamily",null,"MRPS28",0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,1,0,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,0,1,0,1,0,0,0,1,1,1,1,1,1,1,1,1,1,32,0,0,"Mitochondrial central dogma > Translation > Mitochondrial ribosome","Mitochondrial central dogma "," Translation "," Mitochondrial ribosome"]
["hsa:54940",54940,"OCIAD1","ASRIJ|FLJ20455|MGC111072|OCIA|TPA018",4,"OCIA domain containing 1","endosome | mitochondrion","protein binding",null,"OCIAD1","OCIA domain containing 1","MitoCarta3.0",0,"MOM",0,"OCIA",null,null,null,null,"FirstInEnsemblFamily",null,"OCIAD1",0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,0,1,0,0,0,1,1,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,32,0,0,0,0]
["hsa:4257",4257,"MGST1","GST12|MGC14525|MGST|MGST-I",12,"microsomal glutathione S-transferase 1","mitochondrion | endoplasmic reticulum | mitochondrial inner membrane | apical part of cell | mitochondrial outer membrane | integral to membrane | peroxisomal membrane | nucleus | endoplasmic reticulum membrane | microsome | membrane","protein binding | glutathione transferase activity | glutathione binding | protein homodimerization activity | transferase activity","xenobiotic metabolic process | response to organic nitrogen | glutathione metabolic process | response to drug | protein homotrimerization | Leydig cell differentiation | response to lipopolysaccharide","MGST1","microsomal glutathione S-transferase 1","MitoCarta3.0","Metabolism > Detoxification > Xenobiotic metabolism | Metabolism > Detoxification > ROS and glutathione metabolism","Membrane",1,"MAPEG","Glutathione metabolism | Metabolism of xenobiotics by cytochrome P450 | Drug metabolism - cytochrome P450",null,null,null,"FirstInEnsemblFamily",null,"MGST1",0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,0,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,31,0,0,"Metabolism > Detoxification > Xenobiotic metabolism | Metabolism > Detoxification > ROS and glutathione metabolism","Metabolism "," Detoxification "," Xenobiotic metabolism "," Metabolism "," Detoxification "," ROS and glutathione metabolism"]
["hsa:139322",139322,"APOOL","CXorf33|FAM121A|MGC129748|UNQ8193","X","apolipoprotein O-like","mitochondrion | extracellular region | mitochondrial inner membrane",null,null,"FAM121A","family with sequence similarity 121A","MitoCarta3.0","Mitochondrial dynamics and surveillance > Cristae formation > MICOS complex","MIM",0,null,null,null,null,null,"FirstInEnsemblFamily",null,"APOOL",0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,1,1,0,1,0,1,1,0,1,1,1,1,1,1,0,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,30,0,0,"Mitochondrial dynamics and surveillance > Cristae formation > MICOS complex","Mitochondrial dynamics and surveillance "," Cristae formation "," MICOS complex"]
["hsa:91689",91689,"C22orf32","DDDD|dJ186O1.1",22,"chromosome 22 open reading frame 32","mitochondrion | integral to membrane | membrane",null,null,"C22orf32","chromosome 22 open reading frame 32","MitoCarta3.0","Signaling > Calcium homeostasis > Calcium cycle | Small molecule transport > Calcium uniporter","MIM",1,null,null,null,null,null,"FirstInEnsemblFamily",null,"C22orf32",0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,1,0,1,1,0,1,1,0,0,1,1,1,1,1,1,1,1,0,1,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,30,0,0,"Signaling > Calcium homeostasis > Calcium cycle | Small molecule transport > Calcium uniporter","Signaling "," Calcium homeostasis "," Calcium cycle "," Small molecule transport "," Calcium uniporter"]
["hsa:28977",28977,"MRPL42","L31MT|L42MT|MRP-L31|MRP-L42|MRP-S32|MRPL31|MRPS32|PTD007|RPML31|S32MT",12,"mitochondrial ribosomal protein L42","mitochondrion | ribosome | mitochondrial small ribosomal subunit","structural constituent of ribosome","translation","MRPL42","mitochondrial ribosomal protein L42","MitoCarta3.0","Mitochondrial central dogma > Translation > Mitochondrial ribosome","Matrix",0,null,null,null,null,null,"FirstInEnsemblFamily",null,"MRPL42",0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,1,1,1,0,1,1,0,1,1,1,1,1,1,1,1,1,1,0,0,0,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,30,0,0,"Mitochondrial central dogma > Translation > Mitochondrial ribosome","Mitochondrial central dogma "," Translation "," Mitochondrial ribosome"]
["hsa:29078",29078,"NDUFAF4","C6orf66|HRPAP20|My013|bA22L21.1",6,"NADH dehydrogenase (ubiquinone) 1 alpha subcomplex, assembly factor 4","mitochondrion | mitochondrial membrane","protein binding | calmodulin binding","mitochondrial respiratory chain complex I assembly","C6orf66","chromosome 6 open reading frame 66","MitoCarta3.0","OXPHOS > Complex I > CI assembly factors | OXPHOS > OXPHOS assembly factors","MIM",0,"UPF0240",null,null,null,null,"FirstInEnsemblFamily",null,"NDUFAF4",0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,0,0,0,1,1,1,0,0,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,30,0,0,"OXPHOS > Complex I > CI assembly factors | OXPHOS > OXPHOS assembly factors","OXPHOS "," Complex I "," CI assembly factors "," OXPHOS "," OXPHOS assembly factors"]
["hsa:128308",128308,"MRPL55","AAVG5835|DKFZp686D1387|L55nt|MGC61802|MRP-L55|PRO19675",1,"mitochondrial ribosomal protein L55","mitochondrion | ribosome | mitochondrial large ribosomal subunit","structural constituent of ribosome","translation","MRPL55","mitochondrial ribosomal protein L55","MitoCarta3.0","Mitochondrial central dogma > Translation > Mitochondrial ribosome","Matrix",0,null,null,null,null,null,"FirstInEnsemblFamily",null,"MRPL55",0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,1,1,1,0,1,1,1,1,1,0,1,1,1,1,1,0,0,1,1,1,1,1,0,1,0,1,0,1,1,1,1,1,1,1,1,29,0,0,"Mitochondrial central dogma > Translation > Mitochondrial ribosome","Mitochondrial central dogma "," Translation "," Mitochondrial ribosome"]
["hsa:4708",4708,"NDUFB2","AGGG|CI-AGGG|MGC70788",7,"NADH dehydrogenase (ubiquinone) 1 beta subcomplex, 2, 8kDa","mitochondrion | respiratory chain | mitochondrial inner membrane | mitochondrial respiratory chain complex I | membrane","NADH dehydrogenase (ubiquinone) activity","transport | mitochondrial electron transport, NADH to ubiquinone | respiratory electron transport chain","NDUFB2","NADH dehydrogenase (ubiquinone) 1 beta subcomplex, 2, 8kDa","MitoCarta3.0","OXPHOS > Complex I > CI subunits | OXPHOS > OXPHOS subunits","MIM",0,null,"Oxidative phosphorylation",null,null,null,"FirstInEnsemblFamily",null,"NDUFB2",0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,1,1,1,1,0,0,1,1,1,1,1,1,1,1,1,0,0,1,1,1,0,1,1,1,1,1,1,0,0,1,1,1,0,1,1,29,0,0,"OXPHOS > Complex I > CI subunits | OXPHOS > OXPHOS subunits","OXPHOS "," Complex I "," CI subunits "," OXPHOS "," OXPHOS subunits"]
["hsa:57150",57150,"C6orf162","DKFZp586E1923|dJ102H19.2",6,"chromosome 6 open reading frame 162","integral to membrane | membrane",null,null,"C6orf162","chromosome 6 open reading frame 162","MitoCarta3.0",0,"MOM",1,null,null,null,null,null,"FirstInEnsemblFamily",null,"C6orf162",0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,0,0,0,0,0,0,1,1,1,1,1,1,0,1,1,1,1,1,1,0,0,1,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,28,0,0,0,0]
["hsa:285343",285343,"C3orf23","DKFZp313N0621|FLJ41686|MGC119530|MGC119531|MGC119532|MGC119533|TOAG1",3,"chromosome 3 open reading frame 23","mitochondrion",null,null,"C3orf23","chromosome 3 open reading frame 23","MitoCarta3.0",0,"Matrix",0,null,null,null,null,null,"FirstInEnsemblFamily",null,"C3orf23",0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,0,1,1,1,1,1,1,1,1,1,1,1,0,1,0,1,0,1,1,1,1,1,1,1,1,1,0,1,1,1,1,27,0,0,0,0]
["hsa:122704",122704,"MRPL52","-",14,"mitochondrial ribosomal protein L52","mitochondrion | ribosome | mitochondrial large ribosomal subunit","structural constituent of ribosome","translation","MRPL52","mitochondrial ribosomal protein L52","MitoCarta3.0","Mitochondrial central dogma > Translation > Mitochondrial ribosome","Matrix",0,null,null,null,null,null,"FirstInEnsemblFamily",null,"MRPL52",0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,1,1,1,1,0,0,0,1,1,1,1,1,0,0,1,1,1,0,1,0,0,1,0,1,0,0,1,1,1,1,0,0,0,1,1,1,1,1,1,27,0,0,"Mitochondrial central dogma > Translation > Mitochondrial ribosome","Mitochondrial central dogma "," Translation "," Mitochondrial ribosome"]
["hsa:521",521,"ATP5I","ATP5K|MGC12532",4,"ATP synthase, H+ transporting, mitochondrial Fo complex, subunit E","mitochondrion | mitochondrial inner membrane | membrane | mitochondrial proton-transporting ATP synthase complex | mitochondrial proton-transporting ATP synthase complex, coupling factor F(o)","hydrogen ion transmembrane transporter activity | transmembrane transporter activity | ATPase activity","ion transport | mitochondrial ATP synthesis coupled proton transport | ATP catabolic process | respiratory electron transport chain","ATP5I","ATP synthase, H+ transporting, mitochondrial F0 complex, subunit E","MitoCarta3.0","OXPHOS > Complex V > CV subunits | OXPHOS > OXPHOS subunits | Mitochondrial dynamics and surveillance > Cristae formation","MIM",0,"ATP-synt_E","Oxidative phosphorylation",null,null,null,"FirstInEnsemblFamily",null,"ATP5I",0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,0,0,0,1,1,1,1,1,1,1,1,0,1,1,1,1,1,0,0,0,1,0,0,0,1,1,1,1,1,1,0,1,0,1,1,0,1,1,26,0,0,"OXPHOS > Complex V > CV subunits | OXPHOS > OXPHOS subunits | Mitochondrial dynamics and surveillance > Cristae formation","OXPHOS "," Complex V "," CV subunits "," OXPHOS "," OXPHOS subunits ",null," Mitochondrial dynamics and surveillance "," Cristae formation"]
["hsa:9167",9167,"COX7A2L","COX7AR|COX7RP|EB1|SIG81",2,"cytochrome c oxidase subunit VIIa polypeptide 2 like","mitochondrial respiratory chain | mitochondrion | mitochondrial inner membrane | membrane","electron carrier activity | cytochrome-c oxidase activity","respiratory electron transport chain","COX7A2L","cytochrome c oxidase subunit VIIa polypeptide 2 like","MitoCarta3.0","OXPHOS > Complex IV > CIV subunits | OXPHOS > OXPHOS subunits | OXPHOS > OXPHOS assembly factors | OXPHOS > Respirasome assembly","MIM",1,"COX7a","Oxidative phosphorylation",null,null,null,"FirstInEnsemblFamily",null,"COX7A2L",0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,0,1,1,1,1,1,1,1,1,0,1,0,1,0,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,26,0,0,"OXPHOS > Complex IV > CIV subunits | OXPHOS > OXPHOS subunits | OXPHOS > OXPHOS assembly factors | OXPHOS > Respirasome assembly","OXPHOS "," Complex IV "," CIV subunits "," OXPHOS "," OXPHOS subunits ",null," OXPHOS "," OXPHOS assembly factors ",null," OXPHOS "," Respirasome assembly"]
["hsa:22868",22868,"FASTKD2","KIAA0971",2,"FAST kinase domains 2","mitochondrion","protein kinase activity","cellular respiration","FASTKD2","FAST kinase domains 2","MitoCarta3.0","Mitochondrial central dogma > mtRNA metabolism > mtRNA granules | Mitochondrial central dogma > mtRNA metabolism > Polycistronic mtRNA processing | Mitochondrial central dogma > mtRNA metabolism > mtRNA stability and decay | Mitochondrial central dogma > Translation > Mitochondrial ribosome assembly","Matrix",0,"FAST_1 | FAST_2 | RAP",null,null,null,null,"FirstInEnsemblFamily",null,"FASTKD2",0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,1,0,0,0,0,1,1,1,1,1,1,1,1,1,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,26,0,0,"Mitochondrial central dogma > mtRNA metabolism > mtRNA granules | Mitochondrial central dogma > mtRNA metabolism > Polycistronic mtRNA processing | Mitochondrial central dogma > mtRNA metabolism > mtRNA stability and decay | Mitochondrial central dogma > Translation > Mitochondrial ribosome assembly","Mitochondrial central dogma "," mtRNA metabolism "," mtRNA granules "," Mitochondrial central dogma "," mtRNA metabolism "," Polycistronic mtRNA processing "," Mitochondrial central dogma "," mtRNA metabolism "," mtRNA stability and decay "," Mitochondrial central dogma "," Translation "," Mitochondrial ribosome assembly"]
["hsa:25880",25880,"TMEM186","C16orf51|DKFZp564K2062",16,"transmembrane protein 186","mitochondrion | integral to membrane | membrane",null,null,"C16orf51","chromosome 16 open reading frame 51","MitoCarta3.0","OXPHOS > Complex I > CI assembly factors | OXPHOS > OXPHOS assembly factors","MIM",2,null,null,null,null,null,"FirstInEnsemblFamily",null,"TMEM186",0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,0,0,1,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,0,0,1,0,0,0,1,1,0,0,0,0,0,1,1,1,1,1,1,1,1,0,1,0,1,0,1,0,1,1,1,1,1,1,1,0,1,1,26,0,0,"OXPHOS > Complex I > CI assembly factors | OXPHOS > OXPHOS assembly factors","OXPHOS "," Complex I "," CI assembly factors "," OXPHOS "," OXPHOS assembly factors"]
["hsa:597",597,"BCL2A1","ACC-1|ACC-2|BCL2L5|BFL1|GRS|HBPA1",15,"BCL2-related protein A1","cytoplasm","protein binding","regulation of apoptosis | anti-apoptosis","BCL2A1","BCL2-related protein A1","MitoCarta3.0","Mitochondrial dynamics and surveillance > Apoptosis","MOM",0,"Bcl-2",null,null,null,null,"FirstInEnsemblFamily",null,"BCL2A1",0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,0,0,0,1,0,0,1,0,0,0,0,0,1,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,25,0,0,"Mitochondrial dynamics and surveillance > Apoptosis","Mitochondrial dynamics and surveillance "," Apoptosis"]
["hsa:2628",2628,"GATM","AGAT|AT",15,"glycine amidinotransferase (L-arginine:glycine amidinotransferase)","mitochondrion | mitochondrial inner membrane | membrane | mitochondrial intermembrane space | cytoplasm","glycine amidinotransferase activity | transferase activity","response to oxidative stress | embryo development | creatine metabolic process | creatine biosynthetic process | tissue regeneration | response to peptide hormone stimulus | response to mercury ion | response to nutrient | cellular nitrogen compound metabolic process | response to organic substance","GATM","glycine amidinotransferase (L-arginine:glycine amidinotransferase)","MitoCarta3.0","Metabolism > Nucleotide metabolism > Creatine metabolism","Matrix",0,"Amidinotransf","Glycine, serine and threonine metabolism | Arginine and proline metabolism",null,null,null,"FirstInEnsemblFamily","AGAT deficiency","GATM",1,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,0,1,0,0,0,0,0,0,0,0,0,1,1,1,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,0,1,0,1,1,1,1,1,1,1,1,1,0,1,1,1,1,1,1,25,0,0,"Metabolism > Nucleotide metabolism > Creatine metabolism","Metabolism "," Nucleotide metabolism "," Creatine metabolism"]
["hsa:100131801",100131801,"C19orf79","FLJ42727",19,"chromosome 19 open reading frame 79","integral to membrane | membrane",null,null,null,null,"MitoCarta3.0","OXPHOS > Complex IV > CIV assembly factors | OXPHOS > OXPHOS assembly factors","MIM",null,null,null,null,null,null,"FirstInEnsemblFamily",null,"C19orf79",0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,1,0,0,1,1,1,1,1,1,1,1,1,0,1,0,0,0,1,0,1,1,1,0,0,1,1,1,1,1,1,1,24,0,0,"OXPHOS > Complex IV > CIV assembly factors | OXPHOS > OXPHOS assembly factors","OXPHOS "," Complex IV "," CIV assembly factors "," OXPHOS "," OXPHOS assembly factors"]
["hsa:56947",56947,"MFF","C2orf33|DKFZp666J168|GL004|MGC110913",2,"mitochondrial fission factor","mitochondrial outer membrane | mitochondrion | integral to membrane | membrane | cytoplasm",null,null,"C2orf33","chromosome 2 open reading frame 33","MitoCarta3.0","Mitochondrial dynamics and surveillance > Fission","MOM",1,"DUF800",null,null,null,null,"FirstInEnsemblFamily",null,"MFF",0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,0,0,1,1,1,1,1,1,1,0,1,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,24,0,0,"Mitochondrial dynamics and surveillance > Fission","Mitochondrial dynamics and surveillance "," Fission"]
["hsa:51309",51309,"ARMCX1","ALEX1|DKFZp686P06199","X","armadillo repeat containing, X-linked 1","integral to membrane | membrane","binding",null,"ARMCX1","armadillo repeat containing, X-linked 1","MitoCarta3.0","Mitochondrial dynamics and surveillance > Trafficking","MOM",1,"DUF634",null,"ARMADILLO REPEAT CONTAINING X LINKED","ENSFM00250000001585",4,null,null,"ARMCX1",0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,0,0,0,0,0,0,0,1,0,0,0,0,1,1,0,0,0,0,0,0,1,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,0,0,0,0,0,0,0,0,0,0,1,0,0,0,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,23,0,0,"Mitochondrial dynamics and surveillance > Trafficking","Mitochondrial dynamics and surveillance "," Trafficking"]
["hsa:78988",78988,"MRP63","MGC3243|bMRP63",13,"mitochondrial ribosomal protein 63","mitochondrion | ribosome | mitochondrial ribosome","structural constituent of ribosome","translation","MRP63","mitochondrial ribosomal protein 63","MitoCarta3.0","Mitochondrial central dogma > Translation > Mitochondrial ribosome","Matrix",0,null,null,null,null,null,"FirstInEnsemblFamily",null,"MRP63",0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,0,0,0,0,0,0,0,1,1,0,1,1,1,0,1,1,1,1,1,0,0,1,1,0,1,0,1,1,1,1,1,0,1,1,1,1,23,0,0,"Mitochondrial central dogma > Translation > Mitochondrial ribosome","Mitochondrial central dogma "," Translation "," Mitochondrial ribosome"]
["hsa:4541",4541,"ND6","MTND6","MT","NADH dehydrogenase, subunit 6 (complex I)","mitochondrion | respiratory chain | integral to membrane | mitochondrial inner membrane | mitochondrial respiratory chain complex I | membrane","oxidoreductase activity | NADH dehydrogenase (ubiquinone) activity","transport | mitochondrial electron transport, NADH to ubiquinone | response to hydrogen peroxide | respiratory electron transport chain","ND6","NADH dehydrogenase subunit 6","MitoCarta3.0","OXPHOS > Complex I > CI subunits | OXPHOS > OXPHOS subunits","MIM",6,"Oxidored_q3","Oxidative phosphorylation",null,null,null,"FirstInEnsemblFamily",null,"ND6",0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,0,0,0,0,0,1,1,1,1,1,1,0,1,0,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,22,0,0,"OXPHOS > Complex I > CI subunits | OXPHOS > OXPHOS subunits","OXPHOS "," Complex I "," CI subunits "," OXPHOS "," OXPHOS subunits"]
["hsa:1678",1678,"TIMM8A","DDP|DDP1|DFN1|MGC12262|MTS|TIM8","X","translocase of inner mitochondrial membrane 8 homolog A (yeast)","mitochondrion | mitochondrial inner membrane | soluble fraction | mitochondrial intermembrane space protein transporter complex | membrane | mitochondrial intermembrane space","zinc ion binding | metal ion binding","chaperone-mediated protein transport | protein import into mitochondrial inner membrane | nervous system development","TIMM8A","translocase of inner mitochondrial membrane 8 homolog A (yeast)","MitoCarta3.0","Protein import, sorting and homeostasis > Protein homeostasis > Chaperones | Protein import, sorting and homeostasis > Protein import and sorting","MIM",0,"zf-Tim10_DDP",null,null,null,null,"FirstInEnsemblFamily","Jensen syndrome|Mohr-Tranebjaerg syndrome","TIMM8A",0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,0,0,0,0,0,0,0,0,0,0,1,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,0,0,0,0,0,0,1,0,1,0,0,0,0,0,0,0,1,0,0,1,1,1,0,0,1,0,1,1,1,1,0,1,1,1,1,1,1,1,22,0,0,"Protein import, sorting and homeostasis > Protein homeostasis > Chaperones | Protein import, sorting and homeostasis > Protein import and sorting","Protein import, sorting and homeostasis "," Protein homeostasis "," Chaperones "," Protein import, sorting and homeostasis "," Protein import and sorting"]
["hsa:28958",28958,"CCDC56","FLJ50764",17,"coiled-coil domain containing 56","mitochondrion | integral to membrane | membrane",null,null,"CCDC56","coiled-coil domain containing 56","MitoCarta3.0","Mitochondrial central dogma > Translation > Translation factors | OXPHOS > Complex IV > CIV assembly factors | OXPHOS > OXPHOS assembly factors","MIM",1,null,null,null,null,null,"FirstInEnsemblFamily",null,"CCDC56",0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,0,0,0,0,0,1,0,0,0,1,1,1,1,1,0,0,1,0,0,1,1,1,1,1,1,1,0,0,0,1,1,1,1,1,21,0,0,"Mitochondrial central dogma > Translation > Translation factors | OXPHOS > Complex IV > CIV assembly factors | OXPHOS > OXPHOS assembly factors","Mitochondrial central dogma "," Translation "," Translation factors "," OXPHOS "," Complex IV "," CIV assembly factors "," OXPHOS "," OXPHOS assembly factors"]
["hsa:9650",9650,"MTFR1","CHPPR|FAM54A2|KIAA0009",8,"mitochondrial fission regulator 1","mitochondrion | plasma membrane",null,null,"MTFR1","mitochondrial fission regulator 1","MitoCarta3.0","Mitochondrial dynamics and surveillance > Fission","Membrane",0,"DUF729",null,null,null,null,"FirstInEnsemblFamily",null,"MTFR1",0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,0,0,0,1,0,0,0,0,0,0,0,0,0,0,0,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,21,0,0,"Mitochondrial dynamics and surveillance > Fission","Mitochondrial dynamics and surveillance "," Fission"]
["hsa:79135",79135,"APOO","FAM121B|MGC4825","X","apolipoprotein O","low-density lipoprotein particle | integral to membrane | extracellular region | high-density lipoprotein particle | very-low-density lipoprotein particle | membrane",null,"transport | lipid transport","FAM121B","family with sequence similarity 121B","MitoCarta3.0","Mitochondrial dynamics and surveillance > Cristae formation > MICOS complex","MIM",1,null,null,null,null,null,"FirstInEnsemblFamily",null,"APOO",0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,0,0,0,0,0,1,1,1,0,1,1,0,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,20,0,0,"Mitochondrial dynamics and surveillance > Cristae formation > MICOS complex","Mitochondrial dynamics and surveillance "," Cristae formation "," MICOS complex"]
["hsa:284184",284184,"C17orf89","-",17,"chromosome 17 open reading frame 89","mitochondrion",null,null,"LOC284184","hypothetical LOC284184","MitoCarta3.0","OXPHOS > Complex I > CI assembly factors | OXPHOS > OXPHOS assembly factors","IMS",0,null,null,null,null,null,"FirstInEnsemblFamily",null,"C17orf89",0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,0,0,0,1,0,0,0,0,0,0,0,0,0,0,0,0,0,1,0,1,1,1,0,1,0,0,0,0,0,0,1,1,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,0,0,0,0,1,0,0,0,0,0,0,0,0,0,0,1,0,1,0,0,0,0,1,0,0,1,0,1,1,0,0,0,1,0,1,1,20,0,0,"OXPHOS > Complex I > CI assembly factors | OXPHOS > OXPHOS assembly factors","OXPHOS "," Complex I "," CI assembly factors "," OXPHOS "," OXPHOS assembly factors"]
["hsa:79863",79863,"RBFA","C18orf22|FLJ18281|FLJ18505|FLJ21172|FLJ42604|FLJ97160|HsT169",18,"ribosome binding factor A (putative)","mitochondrion",null,"rRNA processing","C18orf22","chromosome 18 open reading frame 22","MitoCarta3.0","Mitochondrial central dogma > Translation > Mitochondrial ribosome assembly","Matrix",0,null,null,null,null,null,"FirstInEnsemblFamily",null,"RBFA",0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,0,0,1,1,1,1,1,1,1,1,1,0,0,0,0,0,1,0,0,0,0,1,1,1,1,1,1,1,1,1,20,0,0,"Mitochondrial central dogma > Translation > Mitochondrial ribosome assembly","Mitochondrial central dogma "," Translation "," Mitochondrial ribosome assembly"]
["hsa:10975",10975,"UQCR11","0710008D09Rik|QCR10|UQCR",19,"ubiquinol-cytochrome c reductase, complex III subunit XI","mitochondrion | respiratory chain | integral to membrane | mitochondrial inner membrane | membrane","electron carrier activity | ubiquinol-cytochrome-c reductase activity","transport | generation of precursor metabolites and energy | respiratory electron transport chain","UQCR","ubiquinol-cytochrome c reductase, 6.4kDa subunit","MitoCarta3.0","OXPHOS > Complex III > CIII subunits | OXPHOS > OXPHOS subunits","MIM",1,"UCR_6-4kD","Oxidative phosphorylation",null,null,null,"FirstInEnsemblFamily",null,"UQCR11",0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,1,0,1,1,1,1,0,0,0,1,1,0,0,0,1,0,0,1,0,0,1,0,1,1,1,1,1,1,1,1,1,20,0,0,"OXPHOS > Complex III > CIII subunits | OXPHOS > OXPHOS subunits","OXPHOS "," Complex III "," CIII subunits "," OXPHOS "," OXPHOS subunits"]
["hsa:84833",84833,"USMG5","DAPIT|DKFZp566D211|HCVFTP2|MGC14697|bA792D24.4",10,"up-regulated during skeletal muscle growth 5 homolog (mouse)","mitochondrion | integral to membrane | mitochondrial inner membrane | membrane",null,null,"USMG5","upregulated during skeletal muscle growth 5 homolog (mouse)","MitoCarta3.0","OXPHOS > Complex V > CV subunits | OXPHOS > OXPHOS subunits | Mitochondrial dynamics and surveillance > Cristae formation","MIM",1,null,null,null,null,null,"FirstInEnsemblFamily",null,"USMG5",0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,0,0,0,0,0,0,1,1,0,0,1,1,1,1,1,1,1,1,0,0,0,0,0,0,1,1,0,0,0,0,1,1,1,1,1,0,1,1,20,0,0,"OXPHOS > Complex V > CV subunits | OXPHOS > OXPHOS subunits | Mitochondrial dynamics and surveillance > Cristae formation","OXPHOS "," Complex V "," CV subunits "," OXPHOS "," OXPHOS subunits ",null," Mitochondrial dynamics and surveillance "," Cristae formation"]
["hsa:84419",84419,"C15orf48","FLJ22645|FOAP-11|MGC32925|NMES1",15,"chromosome 15 open reading frame 48","mitochondrion | nucleus",null,null,"C15orf48","chromosome 15 open reading frame 48","MitoCarta3.0",0,"Membrane",1,null,null,null,null,null,"FirstInEnsemblFamily",null,"C15orf48",0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,0,0,1,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,0,0,0,0,0,0,0,0,0,0,0,1,0,0,0,0,0,0,0,0,0,1,1,0,1,1,1,1,0,1,1,1,1,1,1,1,1,1,19,0,0,0,0]
["hsa:3429",3429,"IFI27","FAM14D|ISG12|ISG12A|P27",14,"interferon, alpha-inducible protein 27","mitochondrial outer membrane | mitochondrion | integral to membrane | membrane","molecular_function","activation of pro-apoptotic gene products | activation of caspase activity | type I interferon-mediated signaling pathway | cytokine-mediated signaling pathway | induction of apoptosis by extracellular signals","IFI27","interferon, alpha-inducible protein 27","MitoCarta3.0","Signaling > Immune response | Mitochondrial dynamics and surveillance > Apoptosis","Membrane",2,"Ifi-6-16",null,null,null,null,"FirstInEnsemblFamily",null,"IFI27",0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,1,0,1,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,0,0,0,0,0,0,1,0,0,0,0,0,0,0,0,0,0,0,0,0,1,0,0,0,1,1,0,1,0,1,1,1,1,1,1,1,1,1,19,0,0,"Signaling > Immune response | Mitochondrial dynamics and surveillance > Apoptosis","Signaling "," Immune response ",null," Mitochondrial dynamics and surveillance "," Apoptosis"]
["hsa:4694",4694,"NDUFA1","CI-MWFE|MWFE|ZNF183","X","NADH dehydrogenase (ubiquinone) 1 alpha subcomplex, 1, 7.5kDa","mitochondrion | respiratory chain | integral to membrane | mitochondrial inner membrane | mitochondrial membrane | mitochondrial respiratory chain complex I | membrane","NADH dehydrogenase (ubiquinone) activity","transport | mitochondrial electron transport, NADH to ubiquinone | respiratory electron transport chain","NDUFA1","NADH dehydrogenase (ubiquinone) 1 alpha subcomplex, 1, 7.5kDa","MitoCarta3.0","OXPHOS > Complex I > CI subunits | OXPHOS > OXPHOS subunits","MIM",1,null,"Oxidative phosphorylation",null,null,null,"FirstInEnsemblFamily",null,"NDUFA1",0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,0,0,0,0,1,1,1,1,1,0,0,0,0,0,1,1,0,1,1,1,1,1,1,1,1,1,1,1,19,0,0,"OXPHOS > Complex I > CI subunits | OXPHOS > OXPHOS subunits","OXPHOS "," Complex I "," CI subunits "," OXPHOS "," OXPHOS subunits"]
["hsa:4707",4707,"NDUFB1","CI-MNLL|CI-SGDH|MNLL",14,"NADH dehydrogenase (ubiquinone) 1 beta subcomplex, 1, 7kDa","mitochondrion | respiratory chain | integral to membrane | mitochondrial inner membrane | mitochondrial respiratory chain complex I | membrane","NADH dehydrogenase activity | NADH dehydrogenase (ubiquinone) activity","transport | mitochondrial electron transport, NADH to ubiquinone | respiratory electron transport chain",null,null,"MitoCarta3.0","OXPHOS > Complex I > CI subunits | OXPHOS > OXPHOS subunits","MIM",null,"NADH_oxidored","Oxidative phosphorylation",null,null,null,"FirstInEnsemblFamily",null,"NDUFB1",0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,1,1,1,0,1,1,1,0,0,1,0,0,0,1,0,1,1,1,1,1,1,1,1,0,0,1,1,19,0,0,"OXPHOS > Complex I > CI subunits | OXPHOS > OXPHOS subunits","OXPHOS "," Complex I "," CI subunits "," OXPHOS "," OXPHOS subunits"]
["hsa:84233",84233,"TMEM126A","DKFZp586C1924|OPA7",11,"transmembrane protein 126A","mitochondrion | integral to membrane | membrane","molecular_function","optic nerve development","TMEM126A","transmembrane protein 126A","MitoCarta3.0","OXPHOS > Complex I > CI assembly factors | OXPHOS > OXPHOS assembly factors","MIM",2,"DUF1370",null,null,null,null,"FirstInEnsemblFamily","Optic atrophy-7","TMEM126A",0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,1,0,1,0,1,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,19,0,0,"OXPHOS > Complex I > CI assembly factors | OXPHOS > OXPHOS assembly factors","OXPHOS "," Complex I "," CI assembly factors "," OXPHOS "," OXPHOS assembly factors"]
["hsa:56181",56181,"FAM54B","MST116|MSTP116",1,"family with sequence similarity 54, member B",null,null,null,"FAM54B","family with sequence similarity 54, member B","MitoCarta3.0",0,"unknown",0,"DUF729",null,null,null,null,"FirstInEnsemblFamily",null,"FAM54B",0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,1,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,1,18,0,0,0,0]
["hsa:4710",4710,"NDUFB4","B15|CI-B15|MGC5105",3,"NADH dehydrogenase (ubiquinone) 1 beta subcomplex, 4, 15kDa","mitochondrion | respiratory chain | integral to membrane | mitochondrial inner membrane | mitochondrial respiratory chain complex I | membrane","NADH dehydrogenase (ubiquinone) activity","response to oxidative stress | transport | mitochondrial electron transport, NADH to ubiquinone | respiratory electron transport chain","NDUFB4","NADH dehydrogenase (ubiquinone) 1 beta subcomplex, 4, 15kDa","MitoCarta3.0","OXPHOS > Complex I > CI subunits | OXPHOS > OXPHOS subunits","MIM",1,"NDUF_B4","Oxidative phosphorylation",null,null,null,"FirstInEnsemblFamily",null,"NDUFB4",0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,1,1,0,1,0,0,0,0,0,0,0,0,0,0,1,0,0,0,0,0,1,1,1,1,1,1,1,1,1,0,1,1,1,1,18,0,0,"OXPHOS > Complex I > CI subunits | OXPHOS > OXPHOS subunits","OXPHOS "," Complex I "," CI subunits "," OXPHOS "," OXPHOS subunits"]
["hsa:389203",389203,"C4orf52","-",4,"chromosome 4 open reading frame 52","integral to membrane | membrane",null,null,"LOC389203","hypothetical gene supported by BC032431","MitoCarta3.0","OXPHOS > Complex IV > CIV assembly factors | OXPHOS > OXPHOS assembly factors","MIM",1,null,null,null,null,null,"FirstInEnsemblFamily",null,"C4orf52",0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,0,0,0,0,1,1,0,0,0,0,1,0,0,1,1,1,0,0,0,0,0,1,1,0,0,1,1,1,1,0,0,0,1,1,1,1,17,0,0,"OXPHOS > Complex IV > CIV assembly factors | OXPHOS > OXPHOS assembly factors","OXPHOS "," Complex IV "," CIV assembly factors "," OXPHOS "," OXPHOS assembly factors"]
["hsa:54471",54471,"SMCR7L","FLJ20232|HSU79252|MID51|MIEF1|dJ1104E15.3",22,"Smith-Magenis syndrome chromosome region, candidate 7-like","mitochondrial outer membrane | mitochondrion | integral to membrane | membrane","protein binding","mitochondrial fusion","SMCR7L","Smith-Magenis syndrome chromosome region, candidate 7-like","MitoCarta3.0","Mitochondrial central dogma > Translation > Mitochondrial ribosome assembly | Mitochondrial dynamics and surveillance > Fission","MOM|Matrix",1,null,null,"SMITH MAGENIS SYNDROME CHROMOSOMAL REGION CANDIDATE GENE 7","ENSFM00250000003315",2,"FirstInEnsemblFamily",null,"SMCR7L",0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,0,0,0,0,0,0,0,0,1,0,0,0,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,17,0,0,"Mitochondrial central dogma > Translation > Mitochondrial ribosome assembly | Mitochondrial dynamics and surveillance > Fission","Mitochondrial central dogma "," Translation "," Mitochondrial ribosome assembly "," Mitochondrial dynamics and surveillance "," Fission"]
["hsa:598",598,"BCL2L1","BCL-XL/S|BCL2L|BCLX|BCLXL|BCLXS|Bcl-X|DKFZp781P2092|PPP1R52|bcl-xL|bcl-xS",20,"BCL2-like 1","mitochondrion | intracellular | cytoplasm | mitochondrial outer membrane | cytosol | nuclear membrane | integral to membrane | mitochondrial membrane | nucleus | membrane | Bcl-2 family protein complex","protein binding | protein heterodimerization activity | BH3 domain binding | identical protein binding","male gonad development | germ cell development | negative regulation of survival gene product expression | positive regulation of apoptosis | cellular response to amino acid stimulus | apoptosis | release of cytochrome c from mitochondria | ovarian follicle development | negative regulation of neuron apoptosis | cellular response to alkaloid | response to cycloheximide | spermatogenesis | growth | positive regulation of cell proliferation | regulation of mitochondrial membrane permeability | negative regulation of release of cytochrome c from mitochondria | anti-apoptosis | apoptotic mitochondrial changes | fertilization | negative regulation of establishment of protein localization in plasma membrane | positive regulation of anti-apoptosis | in utero embryonic development | response to radiation | innate immune response | cellular response to gamma radiation | negative regulation of apoptosis | cellular process regulating host cell cycle in response to virus | induction of apoptosis by intracellular signals | regulation of mitochondrial membrane potential | response to cytokine stimulus | activation of innate immune response","BCL2L1","BCL2-like 1","MitoCarta3.0","Mitochondrial dynamics and surveillance > Apoptosis","MOM",1,"Bcl-2 | BH4","Apoptosis",null,null,null,"FirstInEnsemblFamily",null,"BCL2L1",0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,0,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,16,0,0,"Mitochondrial dynamics and surveillance > Apoptosis","Mitochondrial dynamics and surveillance "," Apoptosis"]
["hsa:23786",23786,"BCL2L13","BCL-RAMBO|Bcl2-L-13|MIL1",22,"BCL2-like 13 (apoptosis facilitator)","mitochondrion | integral to membrane | mitochondrial membrane | nucleus | membrane","caspase activator activity","activation of caspase activity | regulation of apoptosis | induction of apoptosis","BCL2L13","BCL2-like 13 (apoptosis facilitator)","MitoCarta3.0","Mitochondrial dynamics and surveillance > Mitophagy | Mitochondrial dynamics and surveillance > Autophagy | Mitochondrial dynamics and surveillance > Apoptosis","MOM",1,"Bcl-2",null,null,null,null,"FirstInEnsemblFamily",null,"BCL2L13",0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,0,1,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,16,0,0,"Mitochondrial dynamics and surveillance > Mitophagy | Mitochondrial dynamics and surveillance > Autophagy | Mitochondrial dynamics and surveillance > Apoptosis","Mitochondrial dynamics and surveillance "," Mitophagy ",null," Mitochondrial dynamics and surveillance "," Autophagy ",null," Mitochondrial dynamics and surveillance "," Apoptosis"]
["hsa:125988",125988,"C19orf70","P117|QIL1",19,"chromosome 19 open reading frame 70","mitochondrion",null,null,"P117","hypothetical protein P117","MitoCarta3.0","Mitochondrial dynamics and surveillance > Cristae formation > MICOS complex","MIM",0,null,null,null,null,null,"FirstInEnsemblFamily",null,"C19orf70",0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,1,1,0,0,0,0,1,0,1,0,0,0,0,0,0,1,0,0,1,1,0,1,1,1,1,1,1,1,1,16,0,0,"Mitochondrial dynamics and surveillance > Cristae formation > MICOS complex","Mitochondrial dynamics and surveillance "," Cristae formation "," MICOS complex"]
["hsa:56616",56616,"DIABLO","DFNA64|DIABLO-S|FLJ10537|FLJ25049|SMAC|SMAC3",12,"diablo, IAP-binding mitochondrial protein","cytosol | CD40 receptor complex | mitochondrion | internal side of plasma membrane | mitochondrial intermembrane space | cytoplasm","protein binding","activation of caspase activity | induction of apoptosis by oxidative stress | induction of apoptosis via death domain receptors | induction of apoptosis by intracellular signals | activation of caspase activity by cytochrome c | apoptosis | induction of apoptosis","DIABLO","diablo homolog (Drosophila)","MitoCarta3.0","Mitochondrial dynamics and surveillance > Apoptosis","IMS",1,"Smac_DIABLO",null,null,null,null,"FirstInEnsemblFamily","Deafness, autosomal dominant 64","DIABLO",0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,0,1,0,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,16,0,0,"Mitochondrial dynamics and surveillance > Apoptosis","Mitochondrial dynamics and surveillance "," Apoptosis"]
["hsa:55260",55260,"TMEM143","FLJ10922",19,"transmembrane protein 143","mitochondrion | integral to membrane | membrane","molecular_function","biological_process","TMEM143","transmembrane protein 143","MitoCarta3.0",0,"MIM",2,null,null,null,null,null,"FirstInEnsemblFamily",null,"TMEM143",0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,1,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,1,1,1,1,1,0,0,0,0,1,0,0,1,1,0,1,1,1,1,16,0,0,0,0]
["hsa:10017",10017,"BCL2L10","BCL-B|Boo|Diva|MGC129810|MGC129811",15,"BCL2-like 10 (apoptosis facilitator)","nuclear membrane | cytosol | mitochondrion | integral to membrane | nucleus | membrane | membrane fraction","protein binding","activation of caspase activity | spermatogenesis | regulation of apoptosis | female gamete generation | anti-apoptosis","BCL2L10","BCL2-like 10 (apoptosis facilitator)","MitoCarta3.0","Mitochondrial dynamics and surveillance > Apoptosis","MOM",1,"Bcl-2",null,null,null,null,"FirstInEnsemblFamily",null,"BCL2L10",0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,0,0,1,0,0,1,1,1,1,1,1,1,1,1,1,1,0,1,1,15,0,0,"Mitochondrial dynamics and surveillance > Apoptosis","Mitochondrial dynamics and surveillance "," Apoptosis"]
["hsa:25953",25953,"PNKD","BRP17|DKFZp564N1362|DYT8|FPD1|KIAA1184|KIPP1184|MGC31943|MR-1|MR1|PDC|PKND1|TAHCCP2",2,"paroxysmal nonkinesigenic dyskinesia","mitochondrion | nucleus | membrane | cytoplasm","hydrolase activity | zinc ion binding | hydroxyacylglutathione hydrolase activity | metal ion binding",null,"PNKD","paroxysmal nonkinesiogenic dyskinesia","MitoCarta3.0","OXPHOS > Complex IV > CIV assembly factors | OXPHOS > OXPHOS assembly factors","MIM",2,"Lactamase_B",null,null,null,null,"FirstInEnsemblFamily","Paroxysmal nonkinesigenic dyskinesia","PNKD",0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,0,0,1,1,0,0,0,0,0,0,1,1,1,1,1,0,1,1,1,0,1,1,1,1,15,0,0,"OXPHOS > Complex IV > CIV assembly factors | OXPHOS > OXPHOS assembly factors","OXPHOS "," Complex IV "," CIV assembly factors "," OXPHOS "," OXPHOS assembly factors"]
["hsa:55863",55863,"TMEM126B","MGC111203",11,"transmembrane protein 126B","integral to membrane | membrane | cellular_component","molecular_function","biological_process","TMEM126B","transmembrane protein 126B","MitoCarta3.0","OXPHOS > Complex I > CI assembly factors | OXPHOS > OXPHOS assembly factors","MIM",3,"DUF1370",null,null,null,null,"FirstInEnsemblFamily",null,"TMEM126B",0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,0,0,0,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,15,0,0,"OXPHOS > Complex I > CI assembly factors | OXPHOS > OXPHOS assembly factors","OXPHOS "," Complex I "," CI assembly factors "," OXPHOS "," OXPHOS assembly factors"]
["hsa:133957",133957,"CCDC127","FLJ25701",5,"coiled-coil domain containing 127",null,null,null,"CCDC127","coiled-coil domain containing 127","MitoCarta3.0",0,"Membrane",0,null,null,null,null,null,"FirstInEnsemblFamily",null,"CCDC127",0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,0,1,0,0,0,1,1,1,1,1,1,1,1,1,0,1,0,1,1,14,0,0,0,0]
["hsa:92259",92259,"MRPS36","MGC22896|MRP-S36",5,"mitochondrial ribosomal protein S36","mitochondrion | ribosome | mitochondrial small ribosomal subunit","structural constituent of ribosome","translation","MRPS36","mitochondrial ribosomal protein S36","MitoCarta3.0","Mitochondrial central dogma > Translation > Mitochondrial ribosome | Metabolism > Carbohydrate metabolism > TCA cycle","Matrix",0,null,null,null,null,null,"FirstInEnsemblFamily",null,"MRPS36",0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,0,0,0,0,0,0,0,0,0,0,1,0,0,0,1,0,1,1,1,1,0,1,1,1,1,1,1,1,14,0,0,"Mitochondrial central dogma > Translation > Mitochondrial ribosome | Metabolism > Carbohydrate metabolism > TCA cycle","Mitochondrial central dogma "," Translation "," Mitochondrial ribosome "," Metabolism "," Carbohydrate metabolism "," TCA cycle"]
["hsa:125170",125170,"SMCR7","MGC23130|MID49",17,"Smith-Magenis syndrome chromosome region, candidate 7","mitochondrial outer membrane | mitochondrion | integral to membrane | membrane","protein binding","mitochondrion organization","SMCR7","Smith-Magenis syndrome chromosome region, candidate 7","MitoCarta3.0","Mitochondrial dynamics and surveillance > Fission","MOM",1,null,null,"SMITH MAGENIS SYNDROME CHROMOSOMAL REGION CANDIDATE GENE 7","ENSFM00250000003315",2,null,null,"SMCR7",0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,1,1,1,1,1,1,1,1,1,1,1,1,1,14,0,0,"Mitochondrial dynamics and surveillance > Fission","Mitochondrial dynamics and surveillance "," Fission"]
["hsa:54998",54998,"AURKAIP1","AIP|AKIP|FLJ20608",1,"aurora kinase A interacting protein 1","intracellular membrane-bounded organelle | mitochondrion | nucleolus | nucleus","protein binding","positive regulation of proteolysis | negative regulation of mitosis","AURKAIP1","aurora kinase A interacting protein 1","MitoCarta3.0","Mitochondrial central dogma > Translation > Mitochondrial ribosome","Matrix",0,"DUF1713",null,null,null,null,"FirstInEnsemblFamily",null,"AURKAIP1",0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,1,1,1,1,1,1,1,1,0,1,1,1,1,13,0,0,"Mitochondrial central dogma > Translation > Mitochondrial ribosome","Mitochondrial central dogma "," Translation "," Mitochondrial ribosome"]
["hsa:572",572,"BAD","BBC2|BCL2L8",11,"BCL2-associated agonist of cell death","cytosol | mitochondrial outer membrane | mitochondrion | membrane | cytoplasm","lipid binding | protein phosphatase binding | phospholipid binding | protein binding | caspase activator activity | protein kinase binding | protein heterodimerization activity","pore complex assembly | fibroblast growth factor receptor signaling pathway | apoptosis | induction of apoptosis | positive regulation of mitochondrial membrane potential | cellular response to mechanical stimulus | type B pancreatic cell proliferation | activation of caspase activity | positive regulation of epithelial cell proliferation | positive regulation of T cell differentiation | activation of pro-apoptotic gene products | cellular response to hypoxia | cellular process regulating host cell cycle in response to virus | nerve growth factor receptor signaling pathway | ATP metabolic process | cellular response to nicotine | positive regulation of apoptosis | positive regulation of B cell differentiation | glucose homeostasis | positive regulation of caspase activity | glucose catabolic process | ADP metabolic process | induction of apoptosis by extracellular signals | cytokine-mediated signaling pathway | regulation of mitochondrial membrane permeability | cellular response to lipid | phosphatidylinositol-mediated signaling | positive regulation of glucokinase activity | positive regulation of insulin secretion | induction of apoptosis by intracellular signals | positive regulation of insulin secretion involved in cellular response to glucose stimulus | positive regulation of type B pancreatic cell development","BAD","BCL2-antagonist of cell death","MitoCarta3.0","Mitochondrial dynamics and surveillance > Apoptosis","MOM",0,null,"Focal adhesion | Apoptosis",null,null,null,"FirstInEnsemblFamily",null,"BAD",0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,0,0,0,0,0,1,1,1,1,1,0,0,1,1,1,1,1,1,1,13,0,0,"Mitochondrial dynamics and surveillance > Apoptosis","Mitochondrial dynamics and surveillance "," Apoptosis"]
["hsa:84709",84709,"C4orf49","CESP-1|HUMMR|MGARP|MGC125827|MGC125828|OSAP",4,"chromosome 4 open reading frame 49","integral to membrane | membrane",null,null,"OSAP","ovary-specific acidic protein","MitoCarta3.0","Mitochondrial dynamics and surveillance > Trafficking","Membrane",1,null,null,null,null,null,"FirstInEnsemblFamily",null,"C4orf49",0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,1,1,1,1,1,1,1,0,1,1,1,1,1,13,0,0,"Mitochondrial dynamics and surveillance > Trafficking","Mitochondrial dynamics and surveillance "," Trafficking"]
["hsa:1349",1349,"COX7B","-","X","cytochrome c oxidase subunit VIIb","mitochondrial respiratory chain | mitochondrion | integral to membrane | mitochondrial inner membrane | membrane","cytochrome-c oxidase activity","respiratory electron transport chain","COX7B","cytochrome c oxidase subunit VIIb","MitoCarta3.0","OXPHOS > Complex IV > CIV subunits | OXPHOS > OXPHOS subunits","MIM",0,"COX7B","Oxidative phosphorylation",null,null,null,"FirstInEnsemblFamily",null,"COX7B",0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,1,1,1,1,1,1,1,1,1,1,0,1,1,13,0,0,"OXPHOS > Complex IV > CIV subunits | OXPHOS > OXPHOS subunits","OXPHOS "," Complex IV "," CIV subunits "," OXPHOS "," OXPHOS subunits"]
["hsa:170712",170712,"COX7B2","MGC102754",4,"cytochrome c oxidase subunit VIIb2","mitochondrial respiratory chain | mitochondrion | integral to membrane | membrane","cytochrome-c oxidase activity",null,null,null,"MitoCarta3.0","OXPHOS > Complex IV > CIV subunits | OXPHOS > OXPHOS subunits","MIM",null,"COX7B","Oxidative phosphorylation",null,null,null,"FirstInEnsemblFamily",null,"COX7B2",0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,1,1,1,1,1,1,1,1,1,1,0,1,1,13,0,0,"OXPHOS > Complex IV > CIV subunits | OXPHOS > OXPHOS subunits","OXPHOS "," Complex IV "," CIV subunits "," OXPHOS "," OXPHOS subunits"]
["hsa:4731",4731,"NDUFV3","CI-10k|CI-9KD",21,"NADH dehydrogenase (ubiquinone) flavoprotein 3, 10kDa","mitochondrion | respiratory chain | mitochondrial inner membrane | nucleolus | nucleus | mitochondrial respiratory chain complex I | membrane | cytoplasm","NADH dehydrogenase (ubiquinone) activity","transport | mitochondrial electron transport, NADH to ubiquinone | respiratory electron transport chain","NDUFV3","NADH dehydrogenase (ubiquinone) flavoprotein 3, 10kDa","MitoCarta3.0","OXPHOS > Complex I > CI subunits | OXPHOS > OXPHOS subunits","MIM",0,null,"Oxidative phosphorylation",null,null,null,"FirstInEnsemblFamily",null,"NDUFV3",0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,1,1,1,1,1,1,1,0,0,1,1,1,1,13,0,0,"OXPHOS > Complex I > CI subunits | OXPHOS > OXPHOS subunits","OXPHOS "," Complex I "," CI subunits "," OXPHOS "," OXPHOS subunits"]
["hsa:54470",54470,"ARMCX6","FLJ20811","X","armadillo repeat containing, X-linked 6","integral to membrane | membrane",null,null,"ARMCX6","armadillo repeat containing, X-linked 6","MitoCarta3.0",0,"Membrane",1,"DUF634",null,null,null,null,"FirstInEnsemblFamily",null,"ARMCX6",0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,1,0,0,1,1,1,1,1,1,1,1,1,1,12,0,0,0,0]
["hsa:283951",283951,"C16orf91","CCSMST1|MGC104723|gs103",16,"chromosome 16 open reading frame 91","integral to membrane | membrane",null,null,null,null,"MitoCarta3.0",0,"IMS",null,null,null,null,null,null,"FirstInEnsemblFamily",null,"C16orf91",0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,0,0,0,0,0,0,0,0,0,0,0,0,0,1,0,0,0,0,1,0,0,1,1,1,0,1,1,1,1,1,1,12,0,0,0,0]
["hsa:79680",79680,"C22orf29","FLJ21125",22,"chromosome 22 open reading frame 29",null,null,null,null,null,"MitoCarta3.0","Mitochondrial dynamics and surveillance > Apoptosis","MOM",null,null,null,null,null,null,"FirstInEnsemblFamily",null,"C22orf29",0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,1,0,0,1,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,0,0,0,0,0,0,0,0,0,0,0,1,0,0,0,1,0,0,0,0,1,1,1,1,1,12,0,0,"Mitochondrial dynamics and surveillance > Apoptosis","Mitochondrial dynamics and surveillance "," Apoptosis"]
["hsa:4712",4712,"NDUFB6","B17|CI|MGC13675",9,"NADH dehydrogenase (ubiquinone) 1 beta subcomplex, 6, 17kDa","mitochondrion | respiratory chain | integral to membrane | mitochondrial inner membrane | mitochondrial membrane | mitochondrial respiratory chain complex I | membrane","NADH dehydrogenase (ubiquinone) activity","transport | mitochondrial electron transport, NADH to ubiquinone | respiratory electron transport chain","NDUFB6","NADH dehydrogenase (ubiquinone) 1 beta subcomplex, 6, 17kDa","MitoCarta3.0","OXPHOS > Complex I > CI subunits | OXPHOS > OXPHOS subunits","MIM",1,null,"Oxidative phosphorylation",null,null,null,"FirstInEnsemblFamily",null,"NDUFB6",0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,1,1,1,1,1,1,0,0,1,1,1,0,1,1,12,0,0,"OXPHOS > Complex I > CI subunits | OXPHOS > OXPHOS subunits","OXPHOS "," Complex I "," CI subunits "," OXPHOS "," OXPHOS subunits"]
["hsa:4718",4718,"NDUFC2","B14.5b|CI-B14.5b|HLC-1|NADHDH2",11,"NADH dehydrogenase (ubiquinone) 1, subcomplex unknown, 2, 14.5kDa","mitochondrion | respiratory chain | integral to membrane | mitochondrial inner membrane | mitochondrial respiratory chain complex I | membrane | cytoplasm","NADH dehydrogenase (ubiquinone) activity","transport | mitochondrial electron transport, NADH to ubiquinone | respiratory electron transport chain","NDUFC2","NADH dehydrogenase (ubiquinone) 1, subcomplex unknown, 2, 14.5kDa","MitoCarta3.0","OXPHOS > Complex I > CI subunits | OXPHOS > OXPHOS subunits","MIM",1,"NDUF_C2","Oxidative phosphorylation","NADH DEHYDROGENASE [UBIQUINONE] 1 SUBUNIT C2 COMPLEX I B14 5B CI B14 5B NADH UBIQUINONE OXIDOREDUCTASE SUBUNIT B14 5B","ENSFM00500000277881",2,"FirstInEnsemblFamily",null,"NDUFC2",0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,1,1,1,1,1,0,0,1,1,1,1,1,1,12,0,0,"OXPHOS > Complex I > CI subunits | OXPHOS > OXPHOS subunits","OXPHOS "," Complex I "," CI subunits "," OXPHOS "," OXPHOS subunits"]
["hsa:90871",90871,"C9orf123","MGC4730",9,"chromosome 9 open reading frame 123","integral to membrane | membrane",null,null,"C9orf123","chromosome 9 open reading frame 123","MitoCarta3.0","OXPHOS > Complex I > CI assembly factors | OXPHOS > OXPHOS assembly factors","MIM",2,null,null,null,null,null,"FirstInEnsemblFamily",null,"C9orf123",0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,0,0,0,0,0,1,0,0,0,1,0,0,1,1,1,1,1,1,1,11,0,0,"OXPHOS > Complex I > CI assembly factors | OXPHOS > OXPHOS assembly factors","OXPHOS "," Complex I "," CI assembly factors "," OXPHOS "," OXPHOS assembly factors"]
["hsa:1351",1351,"COX8A","COX|COX8|COX8-2|COX8L|VIII|VIII-L",11,"cytochrome c oxidase subunit VIIIA (ubiquitous)","mitochondrion | integral to membrane | mitochondrial inner membrane | membrane","cytochrome-c oxidase activity","generation of precursor metabolites and energy | respiratory electron transport chain","COX8A","cytochrome c oxidase subunit 8A (ubiquitous)","MitoCarta3.0","OXPHOS > Complex IV > CIV subunits | OXPHOS > OXPHOS subunits","MIM",1,"COX8","Oxidative phosphorylation",null,null,null,"FirstInEnsemblFamily",null,"COX8A",0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,1,0,1,1,1,0,0,1,1,1,1,1,1,11,0,0,"OXPHOS > Complex IV > CIV subunits | OXPHOS > OXPHOS subunits","OXPHOS "," Complex IV "," CIV subunits "," OXPHOS "," OXPHOS subunits"]
["hsa:10249",10249,"GLYAT","ACGNAT|CAT|GAT",11,"glycine-N-acyltransferase","mitochondrion | mitochondrial matrix","glycine N-acyltransferase activity | transferase activity, transferring acyl groups","xenobiotic metabolic process | acyl-CoA metabolic process | response to toxin","GLYAT","glycine-N-acyltransferase","MitoCarta3.0","Metabolism > Amino acid metabolism > Glycine metabolism | Metabolism > Detoxification > Xenobiotic metabolism","Matrix",0,"Gly_acyl_tr_N | Gly_acyl_tr_C",null,"GLYCINE N ACYLTRANSFERASE EC_2.3.1.13 ACYL COA:GLYCINE N ACYLTRANSFERASE AAC ARALKYL ACYL COA N ACYLTRANSFERASE ARALKYL ACYL COA:AMINO ACID N ACYLTRANSFERASE","ENSFM00250000003091",3,"FirstInEnsemblFamily",null,"GLYAT",0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,1,0,0,1,1,0,1,1,1,1,1,1,1,11,0,0,"Metabolism > Amino acid metabolism > Glycine metabolism | Metabolism > Detoxification > Xenobiotic metabolism","Metabolism "," Amino acid metabolism "," Glycine metabolism "," Metabolism "," Detoxification "," Xenobiotic metabolism"]
["hsa:4696",4696,"NDUFA3","B9",19,"NADH dehydrogenase (ubiquinone) 1 alpha subcomplex, 3, 9kDa","mitochondrion | respiratory chain | integral to membrane | mitochondrial inner membrane | mitochondrial respiratory chain complex I | membrane","NADH dehydrogenase (ubiquinone) activity","transport | mitochondrial electron transport, NADH to ubiquinone | respiratory electron transport chain","NDUFA3","NADH dehydrogenase (ubiquinone) 1 alpha subcomplex, 3, 9kDa","MitoCarta3.0","OXPHOS > Complex I > CI subunits | OXPHOS > OXPHOS subunits","MIM",1,null,"Oxidative phosphorylation",null,null,null,"FirstInEnsemblFamily",null,"NDUFA3",0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,1,1,1,1,0,0,0,1,1,1,1,1,1,11,0,0,"OXPHOS > Complex I > CI subunits | OXPHOS > OXPHOS subunits","OXPHOS "," Complex I "," CI subunits "," OXPHOS "," OXPHOS subunits"]
["hsa:637",637,"BID","FP497|MGC15319|MGC42355",22,"BH3 interacting domain death agonist","cytosol | mitochondrial outer membrane | integral to mitochondrial membrane | mitochondrion | membrane | membrane fraction | cytoplasm","protein binding | death receptor binding | ubiquitin protein ligase binding","neuron apoptosis | induction of apoptosis via death domain receptors | positive regulation of release of cytochrome c from mitochondria | positive regulation of protein homooligomerization | apoptosis | release of cytochrome c from mitochondria | establishment of protein localization in membrane | glial cell apoptosis | protein targeting to mitochondrion | regulation of mitochondrial membrane permeability | apoptotic mitochondrial changes | regulation of cell proliferation | response to estradiol stimulus | activation of pro-apoptotic gene products | brain development | induction of apoptosis by intracellular signals | regulation of G1/S transition of mitotic cell cycle","BID","BH3 interacting domain death agonist","MitoCarta3.0","Mitochondrial dynamics and surveillance > Apoptosis","MOM",0,"BID","p53 signaling pathway | Apoptosis",null,null,null,"FirstInEnsemblFamily",null,"BID",0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,1,1,1,1,1,1,1,1,1,10,0,0,"Mitochondrial dynamics and surveillance > Apoptosis","Mitochondrial dynamics and surveillance "," Apoptosis"]
["hsa:388962",388962,"BOLA3","MMDS2",2,"bolA homolog 3 (E. coli)","extracellular region | cellular_component","molecular_function","biological_process","BOLA3","bolA homolog 3 (E. coli)","MitoCarta3.0","Metabolism > Metals and cofactors > Fe-S cluster biosynthesis | Metabolism > Metals and cofactors > Fe-S-containing proteins","Matrix",0,"BolA",null,null,null,null,"FirstInEnsemblFamily","Multiple mitochondrial dysfunctions syndrome 2","BOLA3",0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,0,0,0,0,0,1,1,0,0,1,0,0,0,1,1,1,1,1,1,10,0,0,"Metabolism > Metals and cofactors > Fe-S cluster biosynthesis | Metabolism > Metals and cofactors > Fe-S-containing proteins","Metabolism "," Metals and cofactors "," Fe-S cluster biosynthesis "," Metabolism "," Metals and cofactors "," Fe-S-containing proteins"]
["hsa:4717",4717,"NDUFC1","KFYI|MGC117464|MGC126847|MGC138266",4,"NADH dehydrogenase (ubiquinone) 1, subcomplex unknown, 1, 6kDa","mitochondrion | respiratory chain | integral to membrane | mitochondrial inner membrane | mitochondrial respiratory chain complex I | membrane","NADH dehydrogenase (ubiquinone) activity","transport | mitochondrial electron transport, NADH to ubiquinone | respiratory electron transport chain","NDUFC1","NADH dehydrogenase (ubiquinone) 1, subcomplex unknown, 1, 6kDa","MitoCarta3.0","OXPHOS > Complex I > CI subunits | OXPHOS > OXPHOS subunits","MIM",0,null,"Oxidative phosphorylation",null,null,null,"FirstInEnsemblFamily",null,"NDUFC1",0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,1,1,0,1,1,1,1,1,1,1,10,0,0,"OXPHOS > Complex I > CI subunits | OXPHOS > OXPHOS subunits","OXPHOS "," Complex I "," CI subunits "," OXPHOS "," OXPHOS subunits"]
["hsa:10018",10018,"BCL2L11","BAM|BIM|BIM-alpha6|BIM-beta6|BIM-beta7|BOD|BimEL|BimL",2,"BCL2-like 11 (apoptosis facilitator)","endomembrane system | mitochondrion | peripheral to membrane of membrane fraction | BIM-BCL-2 complex | BIM-BCL-xl complex | cytoplasm | mitochondrial outer membrane | cytosol | microtubule | plasma membrane","microtubule binding | protein binding","male gonad development | positive regulation of release of cytochrome c from mitochondria | positive regulation of protein homooligomerization | regulation of organ growth | positive regulation of cell cycle | positive regulation of apoptosis | kidney development | apoptosis | regulation of developmental pigmentation | induction of apoptosis | spermatogenesis | mammary gland development | spleen development | B cell homeostasis | induction of apoptosis by extracellular signals | activation of caspase activity | post-embryonic organ morphogenesis | post-embryonic development | positive regulation of neuron apoptosis | cell-matrix adhesion | in utero embryonic development | tube formation | B cell apoptosis | thymus development | activation of pro-apoptotic gene products | cellular process regulating host cell cycle in response to virus | induction of apoptosis by intracellular signals | nerve growth factor receptor signaling pathway | T cell homeostasis | ear development | odontogenesis of dentine-containing tooth","BCL2L11","BCL2-like 11 (apoptosis facilitator)","MitoCarta3.0","Mitochondrial dynamics and surveillance > Apoptosis","MOM",0,"Bim_N | Bclx_interact",null,null,null,null,"FirstInEnsemblFamily",null,"BCL2L11",0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,0,0,0,1,0,0,1,1,1,1,1,1,1,9,0,0,"Mitochondrial dynamics and surveillance > Apoptosis","Mitochondrial dynamics and surveillance "," Apoptosis"]
["hsa:9556",9556,"C14orf2","MP68|PLPM",14,"chromosome 14 open reading frame 2","mitochondrion | cellular_component","molecular_function","biological_process",null,null,"MitoCarta3.0","OXPHOS > Complex V > CV subunits | OXPHOS > OXPHOS subunits","MIM",null,"Mit_preoteolip",null,null,null,null,"FirstInEnsemblFamily",null,"C14orf2",0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,1,1,1,1,1,1,0,1,1,9,0,0,"OXPHOS > Complex V > CV subunits | OXPHOS > OXPHOS subunits","OXPHOS "," Complex V "," CV subunits "," OXPHOS "," OXPHOS subunits"]
["hsa:401505",401505,"TOMM5","C9orf105|RP11-263I4.1|Tom5|bA613M10.3",9,"translocase of outer mitochondrial membrane 5 homolog (yeast)","mitochondrion | integral to membrane | mitochondrial outer membrane translocase complex | membrane","protein transporter activity","protein targeting to mitochondrion",null,null,"MitoCarta3.0","Protein import, sorting and homeostasis > Protein import and sorting > TOM","MOM",null,null,null,null,null,null,"FirstInEnsemblFamily",null,"TOMM5",0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,0,0,1,1,1,0,0,0,1,1,1,1,1,9,0,0,"Protein import, sorting and homeostasis > Protein import and sorting > TOM","Protein import, sorting and homeostasis "," Protein import and sorting "," TOM"]
["hsa:100188893",100188893,"TOMM6","DKFZp761H221|FLJ32622|OBTP",6,"translocase of outer mitochondrial membrane 6 homolog (yeast)","mitochondrial outer membrane | mitochondrion | membrane",null,"protein transport",null,null,"MitoCarta3.0","Protein import, sorting and homeostasis > Protein import and sorting > TOM","MOM",null,null,null,null,null,null,"FirstInEnsemblFamily",null,"TOMM6",0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,1,0,1,0,0,1,1,1,1,1,1,9,0,0,"Protein import, sorting and homeostasis > Protein import and sorting > TOM","Protein import, sorting and homeostasis "," Protein import and sorting "," TOM"]
["hsa:4509",4509,"ATP8","ATPase8|MTATP8","MT","ATP synthase F0 subunit 8","mitochondrion | integral to membrane | mitochondrial inner membrane | membrane | mitochondrial proton-transporting ATP synthase complex | mitochondrial proton-transporting ATP synthase complex, coupling factor F(o)","hydrogen ion transmembrane transporter activity | transmembrane transporter activity | ATPase activity","ion transport | response to hyperoxia | mitochondrial ATP synthesis coupled proton transport | ATP catabolic process | respiratory electron transport chain | ATP synthesis coupled proton transport","ATP8","ATP synthase F0 subunit 8","MitoCarta3.0","OXPHOS > Complex V > CV subunits | OXPHOS > OXPHOS subunits","MIM",1,"ATP-synt_8","Oxidative phosphorylation",null,null,null,"FirstInEnsemblFamily",null,"ATP8",0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,1,1,1,1,1,1,7,0,0,"OXPHOS > Complex V > CV subunits | OXPHOS > OXPHOS subunits","OXPHOS "," Complex V "," CV subunits "," OXPHOS "," OXPHOS subunits"]
["hsa:219938",219938,"SPATA19","CT132|FLJ25851|SPAS1|spergen1",11,"spermatogenesis associated 19","mitochondrial outer membrane | mitochondrion | membrane",null,"cell differentiation | multicellular organismal development | spermatogenesis","SPATA19","spermatogenesis associated 19","MitoCarta3.0",0,"unknown",0,null,null,null,null,null,"FirstInEnsemblFamily",null,"SPATA19",0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,1,1,1,1,1,1,7,0,0,0,0]
["hsa:790955",790955,"C11orf83","CCDS41658.1|MGC87549|UNQ655",11,"chromosome 11 open reading frame 83","extracellular region",null,null,null,null,"MitoCarta3.0","OXPHOS > Complex III > CIII assembly factors | OXPHOS > OXPHOS assembly factors","MIM",null,null,null,null,null,null,"FirstInEnsemblFamily",null,"C11orf83",0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,0,0,1,0,0,0,0,0,0,1,1,1,1,6,0,0,"OXPHOS > Complex III > CIII assembly factors | OXPHOS > OXPHOS assembly factors","OXPHOS "," Complex III "," CIII assembly factors "," OXPHOS "," OXPHOS assembly factors"]
["hsa:5366",5366,"PMAIP1","APR|NOXA",18,"phorbol-12-myristate-13-acetate-induced protein 1","cytosol | mitochondrial outer membrane | mitochondrion | nucleus","protein binding","positive regulation of glucose metabolic process | positive regulation of release of cytochrome c from mitochondria | virus-infected cell apoptosis | reactive oxygen species metabolic process | cellular component disassembly involved in apoptosis | positive regulation of DNA damage response, signal transduction by p53 class mediator | positive regulation of apoptosis | apoptosis | negative regulation of fibroblast proliferation | induction of apoptosis | protein insertion into mitochondrial membrane involved in induction of apoptosis | positive regulation of caspase activity | signal transduction by p53 class mediator resulting in induction of apoptosis | regulation of mitochondrial membrane permeability | activation of caspase activity | response to UV | cellular response to glucose starvation | positive regulation of neuron apoptosis | positive regulation of protein oligomerization | response to DNA damage stimulus | activation of pro-apoptotic gene products | cellular response to hypoxia | induction of apoptosis by intracellular signals | negative regulation of mitochondrial membrane potential | proteasomal protein catabolic process | response to dsRNA | response to X-ray | T cell homeostasis","PMAIP1","phorbol-12-myristate-13-acetate-induced protein 1","MitoCarta3.0","Mitochondrial dynamics and surveillance > Apoptosis","MOM",0,null,"p53 signaling pathway",null,null,null,"FirstInEnsemblFamily",null,"PMAIP1",0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,0,0,0,1,1,1,1,1,6,0,0,"Mitochondrial dynamics and surveillance > Apoptosis","Mitochondrial dynamics and surveillance "," Apoptosis"]
["hsa:84987",84987,"C12orf62","MGC14288",12,"chromosome 12 open reading frame 62","integral to membrane | membrane",null,null,"C12orf62","chromosome 12 open reading frame 62","MitoCarta3.0","Mitochondrial central dogma > Translation > Translation factors | OXPHOS > Complex IV > CIV assembly factors | OXPHOS > OXPHOS assembly factors","MIM",1,null,null,null,null,null,"FirstInEnsemblFamily",null,"C12orf62",0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,0,1,0,0,1,0,1,1,5,0,0,"Mitochondrial central dogma > Translation > Translation factors | OXPHOS > Complex IV > CIV assembly factors | OXPHOS > OXPHOS assembly factors","Mitochondrial central dogma "," Translation "," Translation factors "," OXPHOS "," Complex IV "," CIV assembly factors "," OXPHOS "," OXPHOS assembly factors"]
["hsa:341947",341947,"COX8C","COX8-3|MGC119774|MGC119775",14,"cytochrome c oxidase subunit VIIIC","mitochondrion | integral to membrane | mitochondrial inner membrane | membrane","cytochrome-c oxidase activity",null,"COX8C","cytochrome c oxidase subunit 8C","MitoCarta3.0","OXPHOS > Complex IV > CIV subunits | OXPHOS > OXPHOS subunits","MIM",1,null,"Oxidative phosphorylation",null,null,null,"FirstInEnsemblFamily",null,"COX8C",0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,0,0,0,1,1,0,1,1,5,0,0,"OXPHOS > Complex IV > CIV subunits | OXPHOS > OXPHOS subunits","OXPHOS "," Complex IV "," CIV subunits "," OXPHOS "," OXPHOS subunits"]
["hsa:57506",57506,"MAVS","CARDIF|DKFZp547C224|DKFZp666M015|FLJ27482|FLJ31698|FLJ35386|FLJ38051|FLJ41962|IPS-1|IPS1|KIAA1271|MGC3260|VISA",20,"mitochondrial antiviral signaling protein","mitochondrial outer membrane | mitochondrion | integral to membrane | membrane","protein binding | protein kinase binding | CARD domain binding | signal transducer activity","positive regulation of tumor necrosis factor production | positive regulation of I-kappaB kinase/NF-kappaB cascade | positive regulation of protein import into nucleus, translocation | interspecies interaction between organisms | positive regulation of transcription from RNA polymerase II promoter | positive regulation of interferon-beta production | response to virus | negative regulation of type I interferon production | positive regulation of interferon-alpha production | positive regulation of sequence-specific DNA binding transcription factor activity | positive regulation of transcription factor import into nucleus | innate immune response | positive regulation of type I interferon-mediated signaling pathway | signal transduction | positive regulation of protein phosphorylation | positive regulation of interleukin-8 production | cellular response to exogenous dsRNA | defense response to bacterium | positive regulation of defense response to virus by host | positive regulation of chemokine (C-C motif) ligand 5 production | positive regulation of IP-10 production | activation of innate immune response","VISA","virus-induced signaling adapter","MitoCarta3.0","Signaling > Immune response","MOM",1,null,null,null,null,null,"FirstInEnsemblFamily",null,"MAVS",0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,1,1,1,1,5,0,0,"Signaling > Immune response","Signaling "," Immune response"]
["hsa:401250",401250,"MCCD1","-",6,"mitochondrial coiled-coil domain 1","mitochondrion",null,null,null,null,"MitoCarta3.0",0,"unknown",null,null,null,null,null,null,"FirstInEnsemblFamily",null,"MCCD1",0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,1,0,1,1,1,5,0,0,0,0]
["hsa:54968",54968,"TMEM70","FLJ20533|MC5DN2",8,"transmembrane protein 70","integral to mitochondrial membrane | mitochondrion | integral to membrane | mitochondrial inner membrane | membrane","molecular_function","mitochondrial proton-transporting ATP synthase complex assembly","TMEM70","transmembrane protein 70","MitoCarta3.0","OXPHOS > Complex I > CI assembly factors | OXPHOS > Complex V > CV assembly factors | OXPHOS > OXPHOS assembly factors","MIM",2,"DUF1301",null,null,null,null,"FirstInEnsemblFamily","Mitochondrial complex V (ATP synthase) deficiency, nuclear type 2","TMEM70",0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,1,1,1,1,5,0,0,"OXPHOS > Complex I > CI assembly factors | OXPHOS > Complex V > CV assembly factors | OXPHOS > OXPHOS assembly factors","OXPHOS "," Complex I "," CI assembly factors "," OXPHOS "," Complex V "," CV assembly factors "," OXPHOS "," OXPHOS assembly factors"]
["hsa:638",638,"BIK","BIP1|BP4|NBK",22,"BCL2-interacting killer (apoptosis-inducing)","mitochondrial envelope | endomembrane system | integral to membrane | membrane","protein binding | protein heterodimerization activity | BH domain binding","male gonad development | spermatogenesis | positive regulation of release of cytochrome c from mitochondria | positive regulation of protein homooligomerization | regulation of apoptosis | induction of apoptosis","BIK","BCL2-interacting killer (apoptosis-inducing)","MitoCarta3.0","Mitochondrial dynamics and surveillance > Apoptosis","MOM",1,null,null,null,null,null,"FirstInEnsemblFamily",null,"BIK",0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,0,1,1,3,0,0,"Mitochondrial dynamics and surveillance > Apoptosis","Mitochondrial dynamics and surveillance "," Apoptosis"]
["hsa:27113",27113,"BBC3","FLJ42994|JFY-1|JFY1|PUMA",19,"BCL2 binding component 3","cytosol | mitochondrial outer membrane | mitochondrion","protein binding","positive regulation of release of cytochrome c from mitochondria | positive regulation of protein homooligomerization | DNA damage response, signal transduction by p53 class mediator resulting in induction of apoptosis | apoptosis | release of cytochrome c from mitochondria | induction of apoptosis | protein insertion into mitochondrial membrane involved in induction of apoptosis | positive regulation of thymocyte apoptosis | release of sequestered calcium ion into cytosol | activation of caspase activity | activation of pro-apoptotic gene products | negative regulation of growth | cellular response to hypoxia | induction of apoptosis by intracellular signals | reduction of endoplasmic reticulum calcium ion concentration | determination of adult lifespan","BBC3","BCL2 binding component 3","MitoCarta3.0","Mitochondrial dynamics and surveillance > Apoptosis","MOM",0,null,"p53 signaling pathway",null,null,null,"FirstInEnsemblFamily",null,"BBC3",0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,2,0,0,"Mitochondrial dynamics and surveillance > Apoptosis","Mitochondrial dynamics and surveillance "," Apoptosis"]
["hsa:79145",79145,"CHCHD7","FLJ40966|MGC2217",8,"coiled-coil-helix-coiled-coil-helix domain containing 7","mitochondrion",null,null,"CHCHD7","coiled-coil-helix-coiled-coil-helix domain containing 7","MitoCarta3.0","Metabolism > Metals and cofactors > Copper metabolism","IMS",0,null,null,null,null,null,"FirstInEnsemblFamily",null,"CHCHD7",0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,0,1,2,0,0,"Metabolism > Metals and cofactors > Copper metabolism","Metabolism "," Metals and cofactors "," Copper metabolism"]
["hsa:63929",63929,"XPNPEP3","APP3|NPHPL1",22,"X-prolyl aminopeptidase (aminopeptidase P) 3, putative","mitochondrion","metallopeptidase activity | manganese ion binding | peptidase activity | aminopeptidase activity | metal ion binding","proteolysis | cellular process",null,null,"MitoCarta3.0","Protein import, sorting and homeostasis > Protein import and sorting > Preprotein cleavage | Protein import, sorting and homeostasis > Protein homeostasis > Proteases","Matrix",null,"AMP_N | Peptidase_M24",null,null,null,null,"FirstInEnsemblFamily","Nephronophthisis-like nephropathy 1","XPNPEP3",0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,0,1,1,0,0,"Protein import, sorting and homeostasis > Protein import and sorting > Preprotein cleavage | Protein import, sorting and homeostasis > Protein homeostasis > Proteases","Protein import, sorting and homeostasis "," Protein import and sorting "," Preprotein cleavage "," Protein import, sorting and homeostasis "," Protein homeostasis "," Proteases"]