Human MitoCarta3.0: 1136 mitochondrial genes

The MitoCarta3.0 human inventory is a collection of 1136 nuclear and mtDNA genes encoding proteins with strong support of mitochondrial localization. This table provides a summary of evidence supporting mitochondrial localization, the protein distribution across 14 tissues, as well as annotation of biological pathways (based on manual literature review) and sub-compartment (based on annotator's "best guess" integrating sequence features, experimental data, literature, and pathway context; note all subunits of protein complexes are assigned the same compartment). The genes are ordered by strength of mitochondrial evidence. More complete information is available for download in file Human.MitoCarta3.0.xls. See also MitoCarta3.0 home for a description of the project and MitoCarta3.0 documentation for information on the columns below.

Symbol Description Synonyms Maestro score Evidence Sub-compartment MitoPathways Tissues

CYC1 cytochrome c1 MC3DN6, UQCR4, P08574, gene:1537 43 literature, APEX_IMS, APEX_matrix, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain+, induction, coexpression++, MS/MS++ MIM OXPHOS > Complex III > CIII subunits;
Metabolism > Metals and cofactors > Heme-containing proteins;
Metabolism > Electron carriers > Cytochromes;
OXPHOS > OXPHOS subunits all 14

SDHB succinate dehydrogenase complex iron sulfur subunit B CWS2, IP, PGL4, SDH, SDH1, SDH2, SDHIP, P21912, gene:6390 43 literature, APEX_matrix, targetP signal+, yeast mito homolog, Rickettsial homolog, mito protein domain+, induction, coexpression++, MS/MS++ MIM OXPHOS > Complex II > CII subunits;
Metabolism > Carbohydrate metabolism > TCA cycle;
Metabolism > Metals and cofactors > Fe-S-containing proteins;
OXPHOS > OXPHOS subunits all 14

COQ7 coenzyme Q7, hydroxylase CAT5, CLK-1, CLK1, COQ10D8, Q99807, gene:10229 43 literature, targetP signal+, yeast mito homolog++, Rickettsial homolog, mito protein domain+, coexpression++, MS/MS++ MIM Metabolism > Metals and cofactors > Coenzyme Q metabolism all 14

SDHA succinate dehydrogenase complex flavoprotein subunit A CMD1GG, FP, PGL5, SDH1, SDH2, SDHF, P31040, gene:6389 42 literature, APEX_matrix, targetP signal+, yeast mito homolog++, Rickettsial homolog, mito protein domain, induction, coexpression++, MS/MS++ MIM OXPHOS > Complex II > CII subunits;
Metabolism > Carbohydrate metabolism > TCA cycle;
OXPHOS > OXPHOS subunits all 14

UQCRC1 ubiquinol-cytochrome c reductase core protein 1 D3S3191, QCR1, UQCR1, P31930, gene:7384 42 literature, APEX_matrix, targetP signal+, yeast mito homolog+, Rickettsial homolog, mito protein domain+, induction, coexpression++, MS/MS++ MIM Protein import, sorting and homeostasis > Protein import and sorting > Preprotein cleavage;
OXPHOS > Complex III > CIII subunits;
OXPHOS > OXPHOS subunits all 14

COQ5 coenzyme Q5, methyltransferase Q5HYK3, gene:84274 42 APEX_matrix, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain+, induction, coexpression++, MS/MS++ MIM Metabolism > Metals and cofactors > Coenzyme Q metabolism all 14

PDHA1 pyruvate dehydrogenase E1 subunit alpha 1 PDHA, PDHAD, PDHCE1A, PHE1A, P08559, gene:5160 42 literature, APEX_matrix, targetP signal+, yeast mito homolog+, Rickettsial homolog, mito protein domain+, induction, coexpression++, MS/MS++ Matrix Metabolism > Carbohydrate metabolism > Pyruvate metabolism all 14

COQ9 coenzyme Q9 C16orf49, COQ10D5, O75208, gene:57017 42 APEX_matrix, targetP signal+, yeast mito homolog++, Rickettsial homolog, mito protein domain+, induction, coexpression, MS/MS++ MIM Metabolism > Metals and cofactors > Coenzyme Q metabolism all 14

MRPL12 mitochondrial ribosomal protein L12 5c5-2, L12mt, MRP-L31/34, MRPL7, MRPL7/L12, RPML12, P52815, gene:6182 42 literature, targetP signal+, yeast mito homolog++, Rickettsial homolog, mito protein domain, induction, coexpression++, MS/MS++ Matrix Mitochondrial central dogma > mtRNA metabolism > Transcription;
Mitochondrial central dogma > Translation > Mitochondrial ribosome all 14

ATP5F1D ATP synthase F1 subunit delta ATP5D, MC5DN5, P30049, gene:513 42 literature, APEX_matrix, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain+, induction, coexpression++, MS/MS+ MIM OXPHOS > Complex V > CV subunits;
OXPHOS > OXPHOS subunits all 14

COX5A cytochrome c oxidase subunit 5A COX, COX-VA, VA, P20674, gene:9377 42 literature, APEX_IMS, APEX_matrix, targetP signal+, yeast mito homolog++, mito protein domain+, induction, coexpression++, MS/MS++ MIM OXPHOS > Complex IV > CIV subunits;
OXPHOS > OXPHOS subunits all 14

ISCA2 iron-sulfur cluster assembly 2 HBLD1, ISA2, MMDS4, c14_5557, Q86U28, gene:122961 42 APEX_matrix, targetP signal+, yeast mito homolog++, Rickettsial homolog, mito protein domain+, induction, coexpression, MS/MS++ Matrix Metabolism > Metals and cofactors > Fe-S cluster biosynthesis;
Metabolism > Metals and cofactors > Fe-S-containing proteins all 14

PMPCB peptidase, mitochondrial processing subunit beta Beta-MPP, MPP11, MPPB, MPPP52, P-52, O75439, gene:9512 41 literature, APEX_matrix, targetP signal+, yeast mito homolog+, Rickettsial homolog, mito protein domain+, induction, coexpression++, MS/MS++ Matrix Protein import, sorting and homeostasis > Protein import and sorting > Preprotein cleavage;
Protein import, sorting and homeostasis > Protein homeostasis > Proteases all 14

UQCRFS1 ubiquinol-cytochrome c reductase, Rieske iron-sulfur polypeptide 1 MC3DN10, RIP1, RIS1, RISP, UQCR5, P47985, gene:7386 40 literature, APEX_matrix, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain+, coexpression++, MS/MS++ MIM OXPHOS > Complex III > CIII subunits;
Metabolism > Metals and cofactors > Fe-S-containing proteins;
OXPHOS > OXPHOS subunits all 14

ATP5F1A ATP synthase F1 subunit alpha ATP5A, ATP5A1, ATP5AL2, ATPM, COXPD22, HEL-S-123m, MC5DN4, MOM2, OMR, ORM, hATP1, P25705, gene:498 40 literature, APEX_matrix, targetP signal+, yeast mito homolog++, Rickettsial homolog, mito protein domain, induction, coexpression++, MS/MS++ MIM OXPHOS > Complex V > CV subunits;
OXPHOS > OXPHOS subunits all 14

OGDH oxoglutarate dehydrogenase AKGDH, E1k, KGD1, OGDC, OGDH2, Q02218, gene:4967 40 literature, APEX_matrix, targetP signal, yeast mito homolog+, Rickettsial homolog, mito protein domain+, induction, coexpression++, MS/MS++ Matrix Metabolism > Carbohydrate metabolism > TCA cycle all 14

PDHB pyruvate dehydrogenase E1 subunit beta PDHBD, PDHE1-B, PDHE1B, PHE1B, P11177, gene:5162 40 literature, targetP signal, yeast mito homolog+, Rickettsial homolog, mito protein domain+, induction, coexpression++, MS/MS++ Matrix Metabolism > Carbohydrate metabolism > Pyruvate metabolism all 14

UQCRC2 ubiquinol-cytochrome c reductase core protein 2 MC3DN5, QCR2, UQCR2, P22695, gene:7385 40 literature, APEX_matrix, targetP signal, yeast mito homolog+, Rickettsial homolog, mito protein domain+, induction, coexpression++, MS/MS++ MIM Protein import, sorting and homeostasis > Protein import and sorting > Preprotein cleavage;
OXPHOS > Complex III > CIII subunits;
OXPHOS > OXPHOS subunits all 14

SDHD succinate dehydrogenase complex subunit D CBT1, CII-4, CWS3, PGL, PGL1, QPs3, SDH4, cybS, O14521, gene:6392 40 literature, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain+, induction, coexpression++, MS/MS+ MIM OXPHOS > Complex II > CII subunits;
Metabolism > Carbohydrate metabolism > TCA cycle;
Metabolism > Metals and cofactors > Heme-containing proteins;
OXPHOS > OXPHOS subunits all 14

MRPS35 mitochondrial ribosomal protein S35 HDCMD11P, MDS023, MRP-S28, MRPS28, P82673, gene:60488 39 literature, APEX_matrix, targetP signal, yeast mito homolog++, mito protein domain+, induction, coexpression++, MS/MS++ Matrix Mitochondrial central dogma > Translation > Mitochondrial ribosome all 14

UQCRQ ubiquinol-cytochrome c reductase complex III subunit VII MC3DN4, QCR8, QP-C, QPC, UQCR7, O14949, gene:27089 39 literature, APEX_IMS, targetP signal, yeast mito homolog++, mito protein domain+, induction, coexpression++, MS/MS++ MIM OXPHOS > Complex III > CIII subunits;
OXPHOS > OXPHOS subunits all 14

MRPL53 mitochondrial ribosomal protein L53 L53MT, Q96EL3, gene:116540 39 literature, APEX_matrix, targetP signal, yeast mito homolog++, mito protein domain+, induction, coexpression++, MS/MS++ Matrix Mitochondrial central dogma > Translation > Mitochondrial ribosome all 14

DBT dihydrolipoamide branched chain transacylase E2 BCATE2, BCKAD-E2, BCKADE2, BCKDH-E2, BCOADC-E2, E2, E2B, P11182, gene:1629 39 literature, APEX_matrix, targetP signal+, yeast mito homolog+, Rickettsial homolog, mito protein domain+, induction, coexpression+, MS/MS++ Matrix Metabolism > Amino acid metabolism > BCAA metabolism;
Metabolism > Amino acid metabolism > BCAA dehydrogenase complex all 14

PDK4 pyruvate dehydrogenase kinase 4 Q16654, gene:5166 38 literature, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain+, induction, coexpression, MS/MS++ MIM Metabolism > Carbohydrate metabolism > Pyruvate metabolism cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, testis

MDH2 malate dehydrogenase 2 EIEE51, M-MDH, MDH, MGC:3559, MOR1, P40926, gene:4191 38 literature, APEX_matrix, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain, induction, coexpression++, MS/MS++ Matrix Metabolism > Carbohydrate metabolism > Gluconeogenesis;
Metabolism > Carbohydrate metabolism > Malate-aspartate shuttle;
Metabolism > Carbohydrate metabolism > TCA cycle all 14

MRPS27 mitochondrial ribosomal protein S27 MRP-S27, S27mt, Q92552, gene:23107 38 literature, APEX_matrix, targetP signal+, yeast mito homolog++, mito protein domain+, induction, coexpression+, MS/MS++ Matrix Mitochondrial central dogma > Translation > Mitochondrial ribosome cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis

CS citrate synthase O75390, gene:1431 37 literature, APEX_matrix, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain, induction, coexpression++, MS/MS++ Matrix Metabolism > Carbohydrate metabolism > TCA cycle all 14

GRPEL1 GrpE like 1, mitochondrial GrpE, HMGE, mt-GrpE#1, Q9HAV7, gene:80273 37 literature, APEX_matrix, targetP signal+, Rickettsial homolog, mito protein domain+, induction, coexpression++, MS/MS++ Matrix Protein import, sorting and homeostasis > Protein import and sorting > Import motor;
Metabolism > Metals and cofactors > Fe-S cluster biosynthesis all 14

DLAT dihydrolipoamide S-acetyltransferase DLTA, E2, PBC, PDC-E2, PDCE2, P10515, gene:1737 37 literature, APEX_matrix, yeast mito homolog+, Rickettsial homolog, mito protein domain+, induction, coexpression++, MS/MS++ Matrix Metabolism > Carbohydrate metabolism > Pyruvate metabolism all 14

LRPPRC leucine rich pentatricopeptide repeat containing CLONE-23970, GP130, LRP130, LSFC, P42704, gene:10128 37 literature, APEX_matrix, targetP signal+, yeast mito homolog++, mito protein domain, induction, coexpression++, MS/MS++ Matrix Mitochondrial central dogma > mtRNA metabolism > mtRNA stability and decay;
Mitochondrial central dogma > Translation all 14

DLST dihydrolipoamide S-succinyltransferase DLTS, KGD2, PGL7, P36957, gene:1743 37 literature, APEX_IMS, APEX_matrix, targetP signal, yeast mito homolog+, Rickettsial homolog, mito protein domain+, induction, coexpression++, MS/MS+ Matrix Metabolism > Carbohydrate metabolism > TCA cycle;
Metabolism > Amino acid metabolism > Lysine metabolism all 14

PDHX pyruvate dehydrogenase complex component X DLDBP, E3BP, OPDX, PDHXD, PDX1, proX, O00330, gene:8050 37 literature, APEX_matrix, targetP signal, yeast mito homolog+, Rickettsial homolog, mito protein domain+, induction, coexpression++, MS/MS+ Matrix Metabolism > Carbohydrate metabolism > Pyruvate metabolism all 14

GFM1 G elongation factor mitochondrial 1 COXPD1, EFG, EFG1, EFGM, EGF1, GFM, hEFG1, mtEF-G1, Q96RP9, gene:85476 37 literature, APEX_matrix, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain, induction, coexpression++, MS/MS++ Matrix Mitochondrial central dogma > Translation > Translation factors all 14

MPC2 mitochondrial pyruvate carrier 2 BRP44, SLC54A2, O95563, gene:25874 37 literature, yeast mito homolog++, mito protein domain+, induction, coexpression++, MS/MS++ MIM Metabolism > Carbohydrate metabolism > Gluconeogenesis;
Metabolism > Carbohydrate metabolism > Pyruvate metabolism;
Metabolism > Carbohydrate metabolism > TCA-associated;
Small molecule transport all 14

NDUFS1 NADH:ubiquinone oxidoreductase core subunit S1 CI-75Kd, CI-75k, MC1DN5, PRO1304, P28331, gene:4719 36 literature, APEX_matrix, targetP signal, Rickettsial homolog, mito protein domain+, induction, coexpression++, MS/MS++ MIM OXPHOS > Complex I > CI subunits;
Metabolism > Metals and cofactors > Fe-S-containing proteins;
OXPHOS > OXPHOS subunits all 14

MRPL46 mitochondrial ribosomal protein L46 C15orf4, LIECG2, P2ECSL, Q9H2W6, gene:26589 36 literature, APEX_matrix, targetP signal+, yeast mito homolog++, mito protein domain, induction, coexpression++, MS/MS++ Matrix Mitochondrial central dogma > Translation > Mitochondrial ribosome cerebrum, cerebellum, spinalcord, kidney, liver, heart, adipose, smallintestine, largeintestine, placenta, testis

ATP5F1E ATP synthase F1 subunit epsilon ATP5E, ATPE, MC5DN3, P56381, gene:514 36 literature, yeast mito homolog++, mito protein domain+, induction, coexpression++, MS/MS++ MIM OXPHOS > Complex V > CV subunits;
OXPHOS > OXPHOS subunits all 14

SLC25A3 solute carrier family 25 member 3 OK/SW-cl.48, PHC, PTP, Q00325, gene:5250 36 literature, yeast mito homolog++, mito protein domain+, induction, coexpression++, MS/MS++ MIM Metabolism > Metals and cofactors > Copper metabolism;
Signaling > Calcium homeostasis > Calcium cycle;
Small molecule transport > SLC25A family all 14

MRPS23 mitochondrial ribosomal protein S23 CGI-138, COXPD46, HSPC329, MRP-S23, Q9Y3D9, gene:51649 36 literature, APEX_matrix, yeast mito homolog++, mito protein domain+, induction, coexpression++, MS/MS++ Matrix Mitochondrial central dogma > Translation > Mitochondrial ribosome all 14

FH fumarate hydratase FMRD, HLRCC, HsFH, LRCC, MCL, MCUL1, P07954, gene:2271 35 literature, APEX_matrix, targetP signal+, yeast mito homolog, Rickettsial homolog, mito protein domain, induction, coexpression++, MS/MS++ Matrix Metabolism > Carbohydrate metabolism > TCA cycle all 14

PMPCA peptidase, mitochondrial processing subunit alpha Alpha-MPP, CLA1, CPD3, INPP5E, P-55, SCAR2, Q10713, gene:23203 35 literature, APEX_matrix, targetP signal+, yeast mito homolog++, Rickettsial homolog, mito protein domain+, coexpression, MS/MS++ Matrix Protein import, sorting and homeostasis > Protein import and sorting > Preprotein cleavage;
Protein import, sorting and homeostasis > Protein homeostasis > Proteases all 14

ATP5F1B ATP synthase F1 subunit beta ATP5B, ATPMB, ATPSB, HEL-S-271, P06576, gene:506 35 literature, APEX_matrix, targetP signal+, yeast mito homolog++, Rickettsial homolog, mito protein domain, coexpression++, MS/MS++ MIM OXPHOS > Complex V > CV subunits;
OXPHOS > OXPHOS subunits all 14

SDHAF4 succinate dehydrogenase complex assembly factor 4 C6orf57, Sdh8, Q5VUM1, gene:135154 35 yeast mito homolog++, Rickettsial homolog, mito protein domain+, induction, coexpression++, MS/MS Matrix OXPHOS > Complex II > CII assembly factors;
OXPHOS > OXPHOS assembly factors adipose, testis

UQCR10 ubiquinol-cytochrome c reductase, complex III subunit X HSPC051, HSPC119, HSPC151, QCR9, UCCR7.2, UCRC, Q9UDW1, gene:29796 35 literature, yeast mito homolog++, mito protein domain+, induction, coexpression++, MS/MS++ MIM OXPHOS > Complex III > CIII subunits;
OXPHOS > OXPHOS subunits all 14

ISCA1 iron-sulfur cluster assembly 1 HBLD2, ISA1, MMDS5, hIscA, Q9BUE6, gene:81689 35 APEX_matrix, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain+, induction, MS/MS++ Matrix Metabolism > Metals and cofactors > Fe-S cluster biosynthesis;
Metabolism > Metals and cofactors > Fe-S-containing proteins cerebrum, cerebellum, brainstem, spinalcord, liver, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis

SUCLA2 succinate-CoA ligase ADP-forming subunit beta A-BETA, A-SCS, LINC00444, MTDPS5, SCS-betaA, Q9P2R7, gene:8803 35 literature, APEX_matrix, targetP signal+, yeast mito homolog+, Rickettsial homolog, mito protein domain, induction, coexpression+, MS/MS++ Matrix Metabolism > Carbohydrate metabolism > TCA cycle;
Metabolism > Carbohydrate metabolism > Itaconate metabolism;
Metabolism > Nucleotide metabolism > Nucleotide synthesis and processing all 14

COQ3 coenzyme Q3, methyltransferase DHHBMT, DHHBMTASE, UG0215E05, bA9819.1, Q9NZJ6, gene:51805 34 APEX_matrix, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain+, induction, coexpression+, MS/MS+ MIM Metabolism > Metals and cofactors > Coenzyme Q metabolism cerebrum, cerebellum, brainstem, spinalcord, liver, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis

IARS2 isoleucyl-tRNA synthetase 2, mitochondrial CAGSSS, ILERS, Q9NSE4, gene:55699 34 APEX_matrix, targetP signal+, yeast mito homolog++, Rickettsial homolog, mito protein domain, coexpression++, MS/MS++ Matrix Mitochondrial central dogma > Translation > mt-tRNA synthetases all 14

MRPS15 mitochondrial ribosomal protein S15 DC37, MPR-S15, RPMS15, S15mt, P82914, gene:64960 34 literature, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain, coexpression++, MS/MS++ Matrix Mitochondrial central dogma > Translation > Mitochondrial ribosome all 14

IDH3A isocitrate dehydrogenase (NAD(+)) 3 catalytic subunit alpha P50213, gene:3419 34 APEX_matrix, yeast mito homolog++, Rickettsial homolog, mito protein domain, induction, coexpression++, MS/MS++ Matrix Metabolism > Carbohydrate metabolism > TCA cycle all 14

COX11 cytochrome c oxidase copper chaperone COX11 COX11P, Q9Y6N1, gene:1353 34 literature, APEX_IMS, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain+, induction, coexpression, MS/MS MIM OXPHOS > Complex IV > CIV assembly factors;
Metabolism > Metals and cofactors > Copper metabolism;
OXPHOS > OXPHOS assembly factors cerebrum, cerebellum, spinalcord, skeletalmuscle, largeintestine, placenta

ETFDH electron transfer flavoprotein dehydrogenase ETFQO, MADD, Q16134, gene:2110 34 literature, APEX_matrix, yeast mito homolog++, mito protein domain+, induction, coexpression++, MS/MS++ MIM Metabolism > Lipid metabolism > Fatty acid oxidation;
Metabolism > Amino acid metabolism > BCAA metabolism;
Metabolism > Amino acid metabolism > Lysine metabolism;
Metabolism > Amino acid metabolism > Glycine metabolism;
Metabolism > Metals and cofactors > Fe-S-containing proteins;
Metabolism > Vitamin metabolism > Choline and betaine metabolism;
Metabolism > Electron carriers > Q-linked reactions, other all 14

TIMM10 translocase of inner mitochondrial membrane 10 TIM10, TIM10A, TIMM10A, P62072, gene:26519 34 literature, APEX_IMS, yeast mito homolog++, mito protein domain+, induction, coexpression++, MS/MS++ MIM Protein import, sorting and homeostasis > Protein homeostasis > Chaperones;
Protein import, sorting and homeostasis > Protein import and sorting cerebrum, cerebellum, brainstem, spinalcord, liver, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis

MRPL34 mitochondrial ribosomal protein L34 L34mt, Q9BQ48, gene:64981 34 literature, APEX_matrix, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain, induction, coexpression++, MS/MS Matrix Mitochondrial central dogma > Translation > Mitochondrial ribosome cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, heart, adipose, smallintestine, largeintestine, stomach, placenta, testis

MRPL2 mitochondrial ribosomal protein L2 CGI-22, MRP-L14, RPML14, Q5T653, gene:51069 34 literature, APEX_matrix, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain, induction, coexpression++, MS/MS++ Matrix Mitochondrial central dogma > Translation > Mitochondrial ribosome spinalcord, liver, heart, adipose, smallintestine, largeintestine, placenta, testis

BCKDHA branched chain keto acid dehydrogenase E1 subunit alpha BCKDE1A, MSU, MSUD1, OVD1A, P12694, gene:593 34 literature, APEX_matrix, targetP signal+, yeast mito homolog+, Rickettsial homolog, mito protein domain+, coexpression+, MS/MS++ MIM Metabolism > Amino acid metabolism > BCAA metabolism;
Metabolism > Amino acid metabolism > BCAA dehydrogenase complex cerebrum, spinalcord, kidney, liver, heart, skeletalmuscle, adipose, largeintestine, stomach, placenta, testis

UQCRH ubiquinol-cytochrome c reductase hinge protein QCR6, UQCR8, P07919, gene:7388 34 literature, APEX_IMS, yeast mito homolog++, mito protein domain+, induction, coexpression++, MS/MS++ MIM OXPHOS > Complex III > CIII subunits;
OXPHOS > OXPHOS subunits all 14

HIGD2A HIG1 hypoxia inducible domain family member 2A RCF1b, Q9BW72, gene:192286 34 APEX_IMS, yeast mito homolog++, mito protein domain+, induction, coexpression++, MS/MS++ MIM OXPHOS > OXPHOS assembly factors;
OXPHOS > Respirasome assembly all 14

ATP5PO ATP synthase peripheral stalk subunit OSCP ATP5O, ATPO, HMC08D05, OSCP, P48047, gene:539 34 literature, APEX_matrix, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain+, induction, MS/MS++ MIM OXPHOS > Complex V > CV subunits;
OXPHOS > OXPHOS subunits all 14

ECHS1 enoyl-CoA hydratase, short chain 1 ECHS1D, SCEH, P30084, gene:1892 33 literature, APEX_matrix, targetP signal+, yeast mito homolog+, Rickettsial homolog, mito protein domain, induction, coexpression+, MS/MS++ Matrix Metabolism > Lipid metabolism > Fatty acid oxidation;
Metabolism > Amino acid metabolism > BCAA metabolism;
Metabolism > Amino acid metabolism > Lysine metabolism all 14

LETMD1 LETM1 domain containing 1 1110019O13Rik, HCCR, HCCR-1, HCCR-2, HCCR1, HCCR2, HCRR-2, SLC55A3, Q6P1Q0, gene:25875 33 GFP, targetP signal+, yeast mito homolog+, mito protein domain+, induction, coexpression, MS/MS++ Membrane Small molecule transport cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, adipose, smallintestine, largeintestine, placenta, testis

COX6A1 cytochrome c oxidase subunit 6A1 CMTRID, COX6A, COX6AL, P12074, gene:1337 33 literature, targetP signal+, yeast mito homolog+, mito protein domain+, induction, coexpression, MS/MS++ MIM OXPHOS > Complex IV > CIV subunits;
OXPHOS > OXPHOS subunits all 14

COX15 cytochrome c oxidase assembly homolog COX15 CEMCOX2, Q7KZN9, gene:1355 33 literature, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain+, coexpression++, MS/MS MIM OXPHOS > Complex IV > CIV assembly factors;
Metabolism > Metals and cofactors > Heme synthesis and processing;
OXPHOS > OXPHOS assembly factors cerebrum, brainstem, spinalcord, kidney, liver, adipose, smallintestine, largeintestine, stomach, placenta, testis

AFG3L2 AFG3 like matrix AAA peptidase subunit 2 OPA12, SCA28, SPAX5, Q9Y4W6, gene:10939 33 literature, APEX_matrix, targetP signal, Rickettsial homolog, mito protein domain+, induction, coexpression++, MS/MS++ MIM Protein import, sorting and homeostasis > Protein homeostasis > Proteases all 14

HADHA hydroxyacyl-CoA dehydrogenase trifunctional multienzyme complex subunit alpha ECHA, GBP, HADH, LCEH, LCHAD, MTPA, TP-ALPHA, P40939, gene:3030 33 literature, APEX_matrix, targetP signal+, yeast mito homolog+, Rickettsial homolog, mito protein domain, coexpression++, MS/MS++ MIM Metabolism > Lipid metabolism > Fatty acid oxidation;
Metabolism > Lipid metabolism > Cardiolipin synthesis;
Metabolism > Amino acid metabolism > BCAA metabolism all 14

ETFA electron transfer flavoprotein subunit alpha EMA, GA2, MADD, P13804, gene:2108 33 literature, APEX_matrix, targetP signal, yeast mito homolog++, mito protein domain, induction, coexpression++, MS/MS++ Matrix Metabolism > Lipid metabolism > Fatty acid oxidation;
Metabolism > Amino acid metabolism > BCAA 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 all 14

NDUFS7 NADH:ubiquinone oxidoreductase core subunit S7 CI-20, CI-20KD, MC1DN3, MY017, PSST, O75251, gene:374291 33 literature, APEX_matrix, targetP signal+, Rickettsial homolog, mito protein domain, induction, coexpression++, MS/MS++ MIM OXPHOS > Complex I > CI subunits;
Metabolism > Metals and cofactors > Fe-S-containing proteins;
OXPHOS > OXPHOS subunits all 14

CPT2 carnitine palmitoyltransferase 2 CPT1, CPTASE, IIAE4, P23786, gene:1376 32 literature, APEX_matrix, targetP signal+, yeast mito homolog++, mito protein domain, induction, coexpression+, MS/MS++ MIM Metabolism > Lipid metabolism > Fatty acid oxidation;
Metabolism > Metals and cofactors > Carnitine synthesis and transport;
Metabolism > Metals and cofactors > Carnitine shuttle all 14

BCKDHB branched chain keto acid dehydrogenase E1 subunit beta BCKDE1B, BCKDH E1-beta, E1B, P21953, gene:594 32 literature, APEX_matrix, targetP signal, yeast mito homolog+, Rickettsial homolog, mito protein domain+, coexpression+, MS/MS++ MIM Metabolism > Amino acid metabolism > BCAA metabolism;
Metabolism > Amino acid metabolism > BCAA dehydrogenase complex cerebrum, cerebellum, brainstem, spinalcord, liver, heart, skeletalmuscle, adipose, largeintestine, stomach, placenta, testis

IDH3B isocitrate dehydrogenase (NAD(+)) 3 non-catalytic subunit beta RP46, O43837, gene:3420 32 literature, APEX_matrix, targetP signal+, yeast mito homolog+, Rickettsial homolog, mito protein domain, induction, coexpression, MS/MS++ Matrix Metabolism > Carbohydrate metabolism > TCA cycle all 14

LARS2 leucyl-tRNA synthetase 2, mitochondrial HLASA, LEURS, PRLTS4, mtLeuRS, Q15031, gene:23395 32 literature, APEX_matrix, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain, coexpression++, MS/MS++ Matrix Mitochondrial central dogma > Translation > mt-tRNA synthetases all 14

ACADS acyl-CoA dehydrogenase short chain ACAD3, SCAD, P16219, gene:35 32 literature, APEX_matrix, targetP signal+, mito protein domain+, induction, coexpression++, MS/MS++ Matrix Metabolism > Lipid metabolism > Fatty acid oxidation all 14

LETM1 leucine zipper and EF-hand containing transmembrane protein 1 SLC55A1, O95202, gene:3954 32 literature, APEX_IMS, APEX_matrix, targetP signal, yeast mito homolog+, mito protein domain+, coexpression++, MS/MS++ MIM Signaling > Calcium homeostasis > Calcium cycle all 14

ATP5ME ATP synthase membrane subunit e ATP5I, ATP5K, P56385, gene:521 32 literature, APEX_IMS, yeast mito homolog++, mito protein domain+, coexpression++, MS/MS++ MIM OXPHOS > Complex V > CV subunits;
OXPHOS > OXPHOS subunits;
Mitochondrial dynamics and surveillance > Cristae formation all 14

OPA1 OPA1 mitochondrial dynamin like GTPase BERHS, MGM1, MTDPS14, NPG, NTG, largeG, O60313, gene:4976 32 literature, APEX_IMS, targetP signal, yeast mito homolog++, mito protein domain, induction, coexpression++, MS/MS++ MIM Mitochondrial dynamics and surveillance > Fusion;
Mitochondrial dynamics and surveillance > Cristae formation all 14

AUH AU RNA binding methylglutaconyl-CoA hydratase Q13825, gene:549 32 literature, APEX_matrix, yeast mito homolog+, Rickettsial homolog, mito protein domain, induction, coexpression++, MS/MS++ Matrix Metabolism > Carbohydrate metabolism > Itaconate metabolism;
Metabolism > Amino acid metabolism > BCAA metabolism all 14

SUCLG1 succinate-CoA ligase GDP/ADP-forming subunit alpha GALPHA, MTDPS9, SUCLA1, P53597, gene:8802 32 yeast mito homolog+, Rickettsial homolog, mito protein domain, induction, coexpression++, MS/MS++ Matrix Metabolism > Carbohydrate metabolism > TCA cycle;
Metabolism > Carbohydrate metabolism > Itaconate metabolism;
Metabolism > Nucleotide metabolism > Nucleotide synthesis and processing all 14

NDUFV2 NADH:ubiquinone oxidoreductase core subunit V2 CI-24k, MC1DN7, P19404, gene:4729 32 literature, APEX_matrix, targetP signal+, Rickettsial homolog, induction, coexpression++, MS/MS++ MIM OXPHOS > Complex I > CI subunits;
Metabolism > Metals and cofactors > Fe-S-containing proteins;
OXPHOS > OXPHOS subunits all 14

COQ6 coenzyme Q6, monooxygenase CGI-10, CGI10, COQ10D6, Q9Y2Z9, gene:51004 32 literature, APEX_matrix, targetP signal+, yeast mito homolog++, Rickettsial homolog, mito protein domain, induction, coexpression+, MS/MS+ MIM Metabolism > Metals and cofactors > Coenzyme Q metabolism cerebrum, cerebellum, spinalcord, kidney, liver, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis

MRPL43 mitochondrial ribosomal protein L43 L43mt, MRP-L43, bMRP36a, Q8N983, gene:84545 31 literature, targetP signal+, yeast mito homolog++, mito protein domain, induction, coexpression, MS/MS++ Matrix Mitochondrial central dogma > Translation > Mitochondrial ribosome all 14

ABHD11 abhydrolase domain containing 11 PP1226, WBSCR21, Q8NFV4, gene:83451 31 literature, APEX_matrix, targetP signal+, yeast mito homolog++, Rickettsial homolog, mito protein domain, coexpression+, MS/MS++ Matrix Metabolism > Carbohydrate metabolism > TCA-associated cerebrum, brainstem, spinalcord, kidney, liver, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis

ATP5PF ATP synthase peripheral stalk subunit F6 ATP5, ATP5A, ATP5J, ATPM, CF6, F6, P18859, gene:522 31 literature, APEX_IMS, targetP signal+, mito protein domain+, induction, coexpression++, MS/MS++ MIM OXPHOS > Complex V > CV subunits;
OXPHOS > OXPHOS subunits all 14

NDUFB8 NADH:ubiquinone oxidoreductase subunit B8 ASHI, CI-ASHI, MC1DN32, O95169, gene:4714 31 literature, APEX_IMS, APEX_matrix, targetP signal+, mito protein domain+, induction, coexpression++, MS/MS++ MIM OXPHOS > Complex I > CI subunits;
OXPHOS > OXPHOS subunits all 14

LONP1 lon peptidase 1, mitochondrial CODASS, LON, LONP, LonHS, PIM1, PRSS15, hLON, P36776, gene:9361 31 literature, APEX_matrix, targetP signal+, yeast mito homolog+, Rickettsial homolog, mito protein domain, induction, coexpression, MS/MS++ Matrix Protein import, sorting and homeostasis > Protein homeostasis > Proteases all 14

DLD dihydrolipoamide dehydrogenase DLDD, DLDH, E3, GCSL, LAD, OGDC-E3, PHE3, P09622, gene:1738 31 literature, APEX_matrix, targetP signal, yeast mito homolog, Rickettsial homolog, mito protein domain, induction, coexpression++, MS/MS++ Matrix Metabolism > Carbohydrate metabolism > Pyruvate metabolism;
Metabolism > Carbohydrate metabolism > TCA cycle;
Metabolism > Amino acid metabolism > BCAA metabolism;
Metabolism > Amino acid metabolism > BCAA dehydrogenase complex;
Metabolism > Amino acid metabolism > Lysine metabolism;
Metabolism > Amino acid metabolism > Glycine metabolism;
Metabolism > Amino acid metabolism > Glycine cleavage system all 14

AIFM1 apoptosis inducing factor mitochondria associated 1 AIF, AUNX1, CMT2D, CMTX4, COWCK, COXPD6, DFNX5, NADMR, NAMSD, PDCD8, SEMDHL, O95831, gene:9131 31 literature, APEX_IMS, targetP signal, Rickettsial homolog, mito protein domain, induction, coexpression++, MS/MS++ MIM Protein import, sorting and homeostasis > Protein import and sorting > MIA40;
OXPHOS > Complex I > CI assembly factors;
OXPHOS > OXPHOS assembly factors;
Mitochondrial dynamics and surveillance > Apoptosis all 14

ECHDC3 enoyl-CoA hydratase domain containing 3 Q96DC8, gene:79746 31 targetP signal+, yeast mito homolog+, Rickettsial homolog, mito protein domain, induction, coexpression, MS/MS++ Matrix none spinalcord, kidney, liver, heart, adipose, largeintestine, stomach, placenta, testis

APOOL apolipoprotein O like CXorf33, FAM121A, MICOS27, Mic27, UNQ8193, Q6UXV4, gene:139322 31 literature, yeast mito homolog, mito protein domain+, induction, coexpression++, MS/MS++ MIM Mitochondrial dynamics and surveillance > Cristae formation > MICOS complex all 14

MRPL10 mitochondrial ribosomal protein L10 L10MT, MRP-L10, MRP-L8, MRPL8, RPML8, Q7Z7H8, gene:124995 31 literature, APEX_matrix, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain, coexpression+, MS/MS++ Matrix Mitochondrial central dogma > Translation > Mitochondrial ribosome all 14

MRRF mitochondrial ribosome recycling factor MRFF, MTRRF, RRF, Q96E11, gene:92399 30 literature, APEX_matrix, targetP signal+, yeast mito homolog++, Rickettsial homolog, mito protein domain, coexpression, MS/MS++ Matrix Mitochondrial central dogma > Translation > Translation factors all 14

NDUFS8 NADH:ubiquinone oxidoreductase core subunit S8 CI-23k, CI23KD, MC1DN2, TYKY, O00217, gene:4728 30 literature, APEX_matrix, targetP signal, Rickettsial homolog, mito protein domain, induction, coexpression++, MS/MS++ MIM OXPHOS > Complex I > CI subunits;
Metabolism > Metals and cofactors > Fe-S-containing proteins;
OXPHOS > OXPHOS subunits all 14

ACADM acyl-CoA dehydrogenase medium chain ACAD1, MCAD, MCADH, P11310, gene:34 30 literature, APEX_matrix, targetP signal, mito protein domain+, induction, coexpression++, MS/MS++ Matrix Metabolism > Lipid metabolism > Fatty acid oxidation all 14

IMMT inner membrane mitochondrial protein HMP, MICOS60, MINOS2, Mic60, P87, P87/89, P89, PIG4, PIG52, Q16891, gene:10989 30 literature, targetP signal, yeast mito homolog, mito protein domain+, coexpression++, MS/MS++ MIM Mitochondrial dynamics and surveillance > Cristae formation > MICOS complex all 14

TIMM9 translocase of inner mitochondrial membrane 9 TIM9, TIM9A, Q9Y5J7, gene:26520 30 literature, APEX_IMS, yeast mito homolog++, mito protein domain+, induction, coexpression+, MS/MS++ MIM Protein import, sorting and homeostasis > Protein homeostasis > Chaperones;
Protein import, sorting and homeostasis > Protein import and sorting all 14

SLC25A4 solute carrier family 25 member 4 AAC1, ANT, ANT 1, ANT1, MTDPS12, MTDPS12A, PEO2, PEO3, PEOA2, T1, P12235, gene:291 30 literature, yeast mito homolog++, mito protein domain+, induction, coexpression, MS/MS++ MIM Metabolism > Nucleotide metabolism > Nucleotide import;
Signaling > Calcium homeostasis > Mitochondrial permeability transition pore;
Small molecule transport > SLC25A family all 14

SAMM50 SAMM50 sorting and assembly machinery component CGI-51, OMP85, SAM50, TOB55, TRG-3, YNL026W, Q9Y512, gene:25813 30 literature, APEX_IMS, APEX_matrix, yeast mito homolog++, Rickettsial homolog, induction, coexpression++, MS/MS++ MOM Protein import, sorting and homeostasis > Protein import and sorting > SAM;
Mitochondrial dynamics and surveillance > Intramitochondrial membrane interactions all 14

NDUFS2 NADH:ubiquinone oxidoreductase core subunit S2 CI-49, MC1DN6, O75306, gene:4720 30 literature, APEX_matrix, targetP signal, Rickettsial homolog, mito protein domain, induction, coexpression++, MS/MS++ MIM OXPHOS > Complex I > CI subunits;
Metabolism > Metals and cofactors > Fe-S-containing proteins;
OXPHOS > OXPHOS subunits all 14

NDUFV1 NADH:ubiquinone oxidoreductase core subunit V1 CI-51K, CI51KD, MC1DN4, UQOR1, P49821, gene:4723 30 literature, APEX_matrix, targetP signal, Rickettsial homolog, mito protein domain, induction, coexpression++, MS/MS++ MIM OXPHOS > Complex I > CI subunits;
Metabolism > Metals and cofactors > Fe-S-containing proteins;
OXPHOS > OXPHOS subunits all 14

ACO2 aconitase 2 ACONM, HEL-S-284, ICRD, OCA8, OPA9, Q99798, gene:50 30 literature, APEX_matrix, targetP signal, Rickettsial homolog, mito protein domain, induction, coexpression++, MS/MS++ Matrix Metabolism > Carbohydrate metabolism > TCA cycle;
Metabolism > Metals and cofactors > Fe-S-containing proteins all 14

SUPV3L1 Suv3 like RNA helicase SUV3, Q8IYB8, gene:6832 30 literature, APEX_matrix, targetP signal+, yeast mito homolog++, mito protein domain, induction, coexpression+, MS/MS+ Matrix Mitochondrial central dogma > mtRNA metabolism > mtRNA granules;
Mitochondrial central dogma > mtRNA metabolism > mtRNA stability and decay cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, heart, skeletalmuscle, adipose, largeintestine, stomach, placenta, testis

FECH ferrochelatase EPP, EPP1, FCE, P22830, gene:2235 30 literature, APEX_matrix, targetP signal+, yeast mito homolog++, Rickettsial homolog, mito protein domain, coexpression, MS/MS++ MIM Metabolism > Metals and cofactors > Heme synthesis and processing;
Metabolism > Metals and cofactors > Fe-S-containing proteins all 14

MTIF2 mitochondrial translational initiation factor 2 P46199, gene:4528 30 literature, APEX_matrix, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain, induction, coexpression++, MS/MS Matrix Mitochondrial central dogma > Translation > Translation factors all 14

PHB prohibitin HEL-215, HEL-S-54e, PHB1, P35232, gene:5245 29 literature, yeast mito homolog+, Rickettsial homolog, mito protein domain, induction, coexpression++, MS/MS++ MIM Protein import, sorting and homeostasis > Protein homeostasis > Chaperones all 14

HIBCH 3-hydroxyisobutyryl-CoA hydrolase HIBYLCOAH, Q6NVY1, gene:26275 29 literature, APEX_matrix, targetP signal+, yeast mito homolog+, mito protein domain, induction, coexpression, MS/MS++ Matrix Metabolism > Amino acid metabolism > BCAA metabolism all 14

MRPS2 mitochondrial ribosomal protein S2 CGI-91, COXPD36, MRP-S2, S2mt, Q9Y399, gene:51116 29 literature, APEX_matrix, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain, induction, coexpression+, MS/MS+ Matrix Mitochondrial central dogma > Translation > Mitochondrial ribosome cerebrum, cerebellum, brainstem, spinalcord, liver, heart, adipose, smallintestine, largeintestine, stomach, placenta, testis

HSPA9 heat shock protein family A (Hsp70) member 9 CRP40, CSA, EVPLS, GRP-75, GRP75, HEL-S-124m, HSPA9B, MOT, MOT2, MTHSP75, PBP74, SAAN, SIDBA4, P38646, gene:3313 29 APEX_matrix, targetP signal+, yeast mito homolog, Rickettsial homolog, mito protein domain, induction, coexpression, MS/MS++ Matrix Metabolism > Metals and cofactors > Fe-S cluster biosynthesis all 14

SURF1 SURF1 cytochrome c oxidase assembly factor CMT4K, Q15526, gene:6834 29 literature, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain+, coexpression, MS/MS MIM OXPHOS > Complex IV > CIV assembly factors;
OXPHOS > OXPHOS assembly factors cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, adipose, smallintestine, largeintestine, stomach, placenta, testis

PRDX3 peroxiredoxin 3 AOP-1, AOP1, HBC189, MER5, PRO1748, SP-22, prx-III, P30048, gene:10935 29 literature, APEX_IMS, APEX_matrix, targetP signal+, Rickettsial homolog, mito protein domain, induction, coexpression++, MS/MS++ Matrix Metabolism > Detoxification > ROS and glutathione metabolism all 14

GHITM growth hormone inducible transmembrane protein DERP2, HSPC282, MICS1, My021, PTD010, TMBIM5, Q9H3K2, gene:27069 29 literature, APEX_IMS, targetP signal+, Rickettsial homolog, mito protein domain+, induction, coexpression++, MS/MS MIM Mitochondrial dynamics and surveillance > Apoptosis all 14

GUF1 GUF1 homolog, GTPase EF-4, EF4, EIEE40, Q8N442, gene:60558 29 APEX_matrix, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain+, coexpression, MS/MS+ Matrix Mitochondrial central dogma > Translation cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis

TIMM13 translocase of inner mitochondrial membrane 13 TIM13, TIM13B, TIMM13A, TIMM13B, ppv1, Q9Y5L4, gene:26517 29 literature, APEX_IMS, yeast mito homolog++, mito protein domain+, induction, coexpression, MS/MS++ MIM Protein import, sorting and homeostasis > Protein homeostasis > Chaperones;
Protein import, sorting and homeostasis > Protein import and sorting all 14

LYRM4 LYR motif containing 4 C6orf149, CGI-203, COXPD19, ISD11, Q9HD34, gene:57128 29 literature, APEX_matrix, targetP signal+, yeast mito homolog, mito protein domain+, induction, coexpression, MS/MS++ Matrix Metabolism > Metals and cofactors > Fe-S cluster biosynthesis all 14

MRPL16 mitochondrial ribosomal protein L16 L16mt, MRP-L16, PNAS-111, Q9NX20, gene:54948 28 literature, yeast mito homolog++, Rickettsial homolog, mito protein domain, coexpression++, MS/MS+ Matrix Mitochondrial central dogma > Translation > Mitochondrial ribosome cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, heart, skeletalmuscle, adipose, smallintestine, largeintestine, placenta, testis

MRPL40 mitochondrial ribosomal protein L40 L40mt, MRP-L22, MRP-L40, MRPL22, NLVCF, URIM, Q9NQ50, gene:64976 28 literature, APEX_matrix, targetP signal+, yeast mito homolog++, mito protein domain+, induction, coexpression, MS/MS Matrix Mitochondrial central dogma > Translation > Mitochondrial ribosome cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis

IDH3G isocitrate dehydrogenase (NAD(+)) 3 non-catalytic subunit gamma H-IDHG, P51553, gene:3421 28 literature, APEX_matrix, yeast mito homolog+, Rickettsial homolog, mito protein domain, induction, coexpression++, MS/MS++ Matrix Metabolism > Carbohydrate metabolism > TCA cycle all 14

SDHC succinate dehydrogenase complex subunit C CYB560, CYBL, PGL3, QPS1, SDH3, Q99643, gene:6391 28 literature, targetP signal+, Rickettsial homolog, mito protein domain, induction, coexpression++, MS/MS+ MIM OXPHOS > Complex II > CII subunits;
Metabolism > Carbohydrate metabolism > TCA cycle;
Metabolism > Metals and cofactors > Heme-containing proteins;
OXPHOS > OXPHOS subunits all 14

NDUFB5 NADH:ubiquinone oxidoreductase subunit B5 CISGDH, SGDH, O43674, gene:4711 28 literature, APEX_IMS, APEX_matrix, targetP signal+, mito protein domain+, induction, coexpression++, MS/MS+ MIM OXPHOS > Complex I > CI subunits;
OXPHOS > OXPHOS subunits all 14

SDHAF2 succinate dehydrogenase complex assembly factor 2 C11orf79, PGL2, SDH5, Q9NX18, gene:54949 28 GFP, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain+, induction, coexpression, MS/MS Matrix OXPHOS > Complex II > CII assembly factors;
OXPHOS > OXPHOS assembly factors cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, heart, adipose, smallintestine, largeintestine, stomach, placenta, testis

COQ10A coenzyme Q10A Q96MF6, gene:93058 28 targetP signal, yeast mito homolog+, Rickettsial homolog, mito protein domain+, induction, MS/MS++ MIM Metabolism > Metals and cofactors > Coenzyme Q metabolism cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, heart, skeletalmuscle, adipose, largeintestine, stomach

GATD3A glutamine amidotransferase like class 1 domain containing 3A C21orf33, ES1, GATD3, GT335, HES1, KNPH, KNPI, P30042, gene:8209 28 APEX_matrix, targetP signal+, mito protein domain, induction, coexpression++, MS/MS++ Matrix none all 14

TXN2 thioredoxin 2 COXPD29, MT-TRX, MTRX, TRX2, TXN, Q99757, gene:25828 28 literature, APEX_matrix, targetP signal+, Rickettsial homolog, mito protein domain, induction, coexpression++, MS/MS+ Matrix Metabolism > Vitamin metabolism > Vitamin C metabolism;
Metabolism > Detoxification > ROS and glutathione metabolism all 14

MRPS18A mitochondrial ribosomal protein S18A HumanS18b, MRP-S18-3, MRP-S18-a, MRPS18-3, S18bmt, S18mt-a, Q9NVS2, gene:55168 28 literature, APEX_matrix, targetP signal, Rickettsial homolog, mito protein domain, induction, coexpression++, MS/MS++ Matrix Mitochondrial central dogma > Translation > Mitochondrial ribosome cerebrum, brainstem, spinalcord, liver, heart, adipose, smallintestine, largeintestine, stomach, placenta, testis

COX6C cytochrome c oxidase subunit 6C P09669, gene:1345 28 literature, APEX_IMS, APEX_matrix, targetP signal, mito protein domain+, induction, coexpression++, MS/MS++ MIM OXPHOS > Complex IV > CIV subunits;
OXPHOS > OXPHOS subunits all 14

NDUFB9 NADH:ubiquinone oxidoreductase subunit B9 B22, CI-B22, LYRM3, MC1DN24, UQOR22, Q9Y6M9, gene:4715 28 literature, APEX_IMS, APEX_matrix, targetP signal, mito protein domain+, induction, coexpression++, MS/MS++ MIM OXPHOS > Complex I > CI subunits;
OXPHOS > OXPHOS subunits all 14

MTCH2 mitochondrial carrier 2 HSPC032, MIMP, SLC25A50, Q9Y6C9, gene:23788 28 literature, APEX_IMS, yeast mito homolog+, mito protein domain+, induction, coexpression+, MS/MS++ MOM Small molecule transport > SLC25A family;
Mitochondrial dynamics and surveillance > Fusion all 14

NDUFA6 NADH:ubiquinone oxidoreductase subunit A6 B14, CI-B14, LYRM6, MC1DN33, NADHB14, P56556, gene:4700 28 literature, APEX_matrix, mito protein domain+, induction, coexpression++, MS/MS++ MIM OXPHOS > Complex I > CI subunits;
OXPHOS > OXPHOS subunits all 14

SLC25A20 solute carrier family 25 member 20 CAC, CACT, O43772, gene:788 28 literature, yeast mito homolog+, mito protein domain+, induction, coexpression, MS/MS++ MIM 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 all 14

MRPL1 mitochondrial ribosomal protein L1 BM022, L1MT, MRP-L1, Q9BYD6, gene:65008 28 literature, APEX_matrix, targetP signal, yeast mito homolog++, mito protein domain, induction, coexpression, MS/MS++ Matrix Mitochondrial central dogma > Translation > Mitochondrial ribosome cerebrum, cerebellum, spinalcord, liver, heart, adipose, smallintestine, largeintestine, stomach, placenta, testis

TIMM44 translocase of inner mitochondrial membrane 44 TIM44, O43615, gene:10469 28 literature, APEX_matrix, targetP signal, yeast mito homolog, mito protein domain, induction, coexpression++, MS/MS++ MIM Protein import, sorting and homeostasis > Protein import and sorting > Import motor all 14

COX17 cytochrome c oxidase copper chaperone COX17 Q14061, gene:10063 28 literature, APEX_IMS, yeast mito homolog, mito protein domain+, induction, coexpression++, MS/MS++ IMS OXPHOS > Complex IV > CIV assembly factors;
Metabolism > Metals and cofactors > Copper metabolism;
OXPHOS > OXPHOS assembly factors heart, adipose, smallintestine, placenta, testis

MICOS10 mitochondrial contact site and cristae organizing system subunit 10 C1orf151, MINOS1, MIO10, Mic10, Q5TGZ0, gene:440574 28 literature, APEX_IMS, yeast mito homolog, mito protein domain+, induction, coexpression++, MS/MS++ MIM Mitochondrial dynamics and surveillance > Cristae formation > MICOS complex all 14

SOD2 superoxide dismutase 2 GClnc1, IPO-B, IPOB, MNSOD, MVCD6, Mn-SOD, P04179, gene:6648 28 literature, APEX_matrix, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain, induction, MS/MS++ Matrix Metabolism > Detoxification > ROS and glutathione metabolism all 14

COX6B1 cytochrome c oxidase subunit 6B1 COX6B, COXG, COXVIb1, P14854, gene:1340 28 literature, APEX_IMS, yeast mito homolog+, mito protein domain+, coexpression++, MS/MS++ MIM OXPHOS > Complex IV > CIV subunits;
OXPHOS > OXPHOS subunits all 14

VDAC1 voltage dependent anion channel 1 PORIN, VDAC-1, P21796, gene:7416 28 literature, APEX_IMS, yeast mito homolog+, mito protein domain+, coexpression++, MS/MS++ MOM Signaling > Calcium homeostasis > Mitochondrial permeability transition pore;
Mitochondrial dynamics and surveillance > Organelle contact sites;
Small molecule transport all 14

CLPP caseinolytic mitochondrial matrix peptidase proteolytic subunit DFNB81, PRLTS3, Q16740, gene:8192 28 literature, APEX_matrix, targetP signal+, Rickettsial homolog, mito protein domain, induction, coexpression+, MS/MS++ Matrix Protein import, sorting and homeostasis > Protein homeostasis > Proteases all 14

HADH hydroxyacyl-CoA dehydrogenase HAD, HADH1, HADHSC, HCDH, HHF4, MSCHAD, SCHAD, Q16836, gene:3033 28 literature, APEX_matrix, targetP signal, Rickettsial homolog, mito protein domain, induction, coexpression++, MS/MS++ Matrix Metabolism > Lipid metabolism > Fatty acid oxidation;
Metabolism > Amino acid metabolism > Lysine metabolism all 14

ACADL acyl-CoA dehydrogenase long chain ACAD4, LCAD, P28330, gene:33 28 literature, targetP signal+, mito protein domain+, induction, coexpression+, MS/MS++ MIM Metabolism > Lipid metabolism > Fatty acid oxidation all 14

ACAT1 acetyl-CoA acetyltransferase 1 ACAT, MAT, T2, THIL, P24752, gene:38 28 literature, APEX_matrix, targetP signal+, Rickettsial homolog, mito protein domain, induction, coexpression+, MS/MS++ Matrix Metabolism > Carbohydrate metabolism > Ketone metabolism;
Metabolism > Lipid metabolism > Fatty acid oxidation;
Metabolism > Amino acid metabolism > BCAA metabolism;
Metabolism > Amino acid metabolism > Lysine metabolism all 14

MALSU1 mitochondrial assembly of ribosomal large subunit 1 C7orf30, mtRsfA, Q96EH3, gene:115416 28 literature, APEX_matrix, targetP signal+, yeast mito homolog++, Rickettsial homolog, mito protein domain, coexpression+, MS/MS Matrix Mitochondrial central dogma > Translation > Mitochondrial ribosome assembly cerebrum, cerebellum, liver, smallintestine, largeintestine, placenta

CLPX caseinolytic mitochondrial matrix peptidase chaperone subunit X EPP2, O76031, gene:10845 27 literature, APEX_matrix, yeast mito homolog++, Rickettsial homolog, mito protein domain, induction, coexpression, MS/MS++ Matrix Protein import, sorting and homeostasis > Protein homeostasis > Proteases all 14

NDUFS4 NADH:ubiquinone oxidoreductase subunit S4 AQDQ, CI-18, CI-18 kDa, CI-AQDQ, MC1DN1, O43181, gene:4724 27 literature, APEX_matrix, targetP signal+, mito protein domain+, coexpression++, MS/MS++ MIM OXPHOS > Complex I > CI subunits;
OXPHOS > OXPHOS subunits all 14

MRPL4 mitochondrial ribosomal protein L4 CGI-28, L4mt, Q9BYD3, gene:51073 27 literature, APEX_matrix, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain, induction, MS/MS++ Matrix Mitochondrial central dogma > Translation > Mitochondrial ribosome cerebrum, brainstem, spinalcord, kidney, liver, adipose, smallintestine, largeintestine, placenta, testis

C1QBP complement C1q binding protein COXPD33, GC1QBP, HABP1, SF2AP32, SF2p32, gC1Q-R, gC1qR, p32, Q07021, gene:708 27 literature, APEX_matrix, targetP signal+, yeast mito homolog++, mito protein domain, induction, coexpression, MS/MS++ Matrix Signaling > Immune response all 14

PITRM1 pitrilysin metallopeptidase 1 MP1, PreP, Q5JRX3, gene:10531 27 literature, APEX_matrix, targetP signal, yeast mito homolog++, mito protein domain+, coexpression, MS/MS++ Matrix Protein import, sorting and homeostasis > Protein homeostasis > Proteases all 14

UQCC1 ubiquinol-cytochrome c reductase complex assembly factor 1 BFZB, C20orf44, CBP3, UQCC, Q9NVA1, gene:55245 27 literature, APEX_matrix, targetP signal, yeast mito homolog, mito protein domain+, coexpression++, MS/MS++ MIM OXPHOS > Complex III > CIII assembly factors;
OXPHOS > OXPHOS assembly factors cerebrum, cerebellum, brainstem, spinalcord, liver, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis

MRPL55 mitochondrial ribosomal protein L55 AAVG5835, L55nt, MRP-L55, PRO19675, Q7Z7F7, gene:128308 27 literature, APEX_matrix, targetP signal, mito protein domain+, induction, coexpression++, MS/MS+ Matrix Mitochondrial central dogma > Translation > Mitochondrial ribosome cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, heart, adipose, smallintestine, largeintestine, stomach, placenta, testis

MRM1 mitochondrial rRNA methyltransferase 1 Q6IN84, gene:79922 27 APEX_matrix, targetP signal+, yeast mito homolog++, Rickettsial homolog, mito protein domain+, induction, coexpression Matrix Mitochondrial central dogma > mtRNA metabolism > mtRNA granules;
Mitochondrial central dogma > mtRNA metabolism > mt-rRNA modifications spinalcord, placenta, testis

MECR mitochondrial trans-2-enoyl-CoA reductase CGI-63, DYTOABG, ETR1, FASN2B, NRBF1, Q9BV79, gene:51102 27 literature, APEX_matrix, targetP signal+, yeast mito homolog++, mito protein domain, coexpression, MS/MS++ Matrix Metabolism > Lipid metabolism > Type II fatty acid synthesis all 14

MRPL44 mitochondrial ribosomal protein L44 COXPD16, L44MT, MRP-L44, Q9H9J2, gene:65080 27 literature, APEX_matrix, targetP signal, yeast mito homolog++, induction, coexpression+, MS/MS++ Matrix Mitochondrial central dogma > Translation > Mitochondrial ribosome cerebrum, spinalcord, liver, heart, adipose, smallintestine, largeintestine, stomach, placenta, testis

HSDL2 hydroxysteroid dehydrogenase like 2 C9orf99, SDR13C1, Q6YN16, gene:84263 27 APEX_matrix, GFP, Rickettsial homolog, mito protein domain+, induction, coexpression+, MS/MS++ Matrix none all 14

MRPS14 mitochondrial ribosomal protein S14 COXPD38, DJ262D12.2, HSMRPS14, MRP-S14, S14mt, O60783, gene:63931 27 literature, APEX_matrix, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain, coexpression, MS/MS++ Matrix Mitochondrial central dogma > Translation > Mitochondrial ribosome cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis

ABCB8 ATP binding cassette subfamily B member 8 EST328128, M-ABC1, MABC1, MITOSUR, Q9NUT2, gene:11194 27 literature, targetP signal+, Rickettsial homolog, mito protein domain, coexpression++, MS/MS++ MIM Small molecule transport > ABC transporters all 14

ATPAF2 ATP synthase mitochondrial F1 complex assembly factor 2 ATP12, ATP12p, LP3663, MC5DN1, Q8N5M1, gene:91647 27 literature, targetP signal, yeast mito homolog++, mito protein domain+, induction, coexpression, MS/MS Matrix OXPHOS > Complex V > CV assembly factors;
OXPHOS > OXPHOS assembly factors cerebrum, cerebellum, brainstem, spinalcord, liver, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis

NDUFA9 NADH:ubiquinone oxidoreductase subunit A9 CC6, CI-39k, CI39k, COQ11, MC1DN26, NDUFS2L, SDR22E1, Q16795, gene:4704 27 literature, APEX_matrix, targetP signal+, mito protein domain, induction, coexpression++, MS/MS++ MIM OXPHOS > Complex I > CI subunits;
OXPHOS > OXPHOS subunits all 14

COA6 cytochrome c oxidase assembly factor 6 C1orf31, CEMCOX4, Q5JTJ3, gene:388753 27 APEX_IMS, yeast mito homolog++, mito protein domain+, induction, coexpression+, MS/MS IMS OXPHOS > Complex IV > CIV assembly factors;
OXPHOS > OXPHOS assembly factors cerebrum, cerebellum, spinalcord, liver, adipose, smallintestine, largeintestine, placenta, testis

POLRMT RNA polymerase mitochondrial APOLMT, MTRNAP, MTRPOL, h-mtRPOL, O00411, gene:5442 27 literature, APEX_matrix, targetP signal, yeast mito homolog++, mito protein domain+, coexpression++, MS/MS Matrix Mitochondrial central dogma > mtDNA maintenance > mtDNA replication;
Mitochondrial central dogma > mtDNA maintenance > mtDNA nucleoid;
Mitochondrial central dogma > mtRNA metabolism > Transcription cerebrum, cerebellum, brainstem, spinalcord, kidney, skeletalmuscle, adipose, largeintestine, stomach, placenta, testis

ISCU iron-sulfur cluster assembly enzyme 2310020H20Rik, HML, ISU2, NIFU, NIFUN, hnifU, Q9H1K1, gene:23479 27 literature, APEX_matrix, targetP signal+, yeast mito homolog++, Rickettsial homolog, mito protein domain, MS/MS++ Matrix Metabolism > Metals and cofactors > Fe-S cluster biosynthesis;
Metabolism > Metals and cofactors > Fe-S-containing proteins all 14

RTN4IP1 reticulon 4 interacting protein 1 NIMP, OPA10, Q8WWV3, gene:84816 27 literature, APEX_matrix, targetP signal, yeast mito homolog, mito protein domain, induction, coexpression++, MS/MS++ Matrix none all 14

OGDHL oxoglutarate dehydrogenase L Q9ULD0, gene:55753 27 APEX_matrix, targetP signal+, yeast mito homolog+, Rickettsial homolog, mito protein domain+, MS/MS++ Matrix Metabolism > Carbohydrate metabolism > TCA cycle all 14

ATP5F1C ATP synthase F1 subunit gamma ATP5C, ATP5C1, ATP5CL1, P36542, gene:509 27 literature, yeast mito homolog++, Rickettsial homolog, mito protein domain, induction, MS/MS++ MIM OXPHOS > Complex V > CV subunits;
OXPHOS > OXPHOS subunits all 14

BCKDK branched chain keto acid dehydrogenase kinase BCKDKD, BDK, O14874, gene:10295 27 literature, APEX_matrix, targetP signal, yeast mito homolog+, Rickettsial homolog, mito protein domain+, MS/MS++ Matrix Metabolism > Amino acid metabolism > BCAA metabolism;
Metabolism > Amino acid metabolism > BCAA dehydrogenase complex cerebrum, cerebellum, spinalcord, kidney, liver, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis

GFM2 GTP dependent ribosome recycling factor mitochondrial 2 EF-G2mt, EFG2, MRRF2, MST027, MSTP027, RRF, RRF2, RRF2mt, hEFG2, mEF-G 2, Q969S9, gene:84340 27 literature, APEX_matrix, yeast mito homolog++, Rickettsial homolog, mito protein domain, coexpression++, MS/MS++ Matrix Mitochondrial central dogma > Translation > Translation factors all 14

NDUFAF4 NADH:ubiquinone oxidoreductase complex assembly factor 4 C6orf66, HRPAP20, HSPC125, MC1DN15, My013, bA22L21.1, Q9P032, gene:29078 27 APEX_matrix, GFP, mito protein domain+, induction, coexpression++, MS/MS++ MIM OXPHOS > Complex I > CI assembly factors;
OXPHOS > OXPHOS assembly factors all 14

SLC25A35 solute carrier family 25 member 35 Q3KQZ1, gene:399512 27 APEX_matrix, yeast mito homolog+, mito protein domain+, induction, coexpression, MS/MS++ MIM Small molecule transport > SLC25A family cerebrum, cerebellum, kidney, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis

SLC25A11 solute carrier family 25 member 11 OGC, PGL6, SLC20A4, Q02978, gene:8402 27 literature, yeast mito homolog+, mito protein domain+, induction, coexpression, MS/MS++ MIM Metabolism > Carbohydrate metabolism > Gluconeogenesis;
Metabolism > Carbohydrate metabolism > Malate-aspartate shuttle;
Metabolism > Carbohydrate metabolism > TCA-associated;
Small molecule transport > SLC25A family all 14

TIMM8B translocase of inner mitochondrial membrane 8 homolog B DDP2, TIM8B, Q9Y5J9, gene:26521 27 literature, yeast mito homolog+, mito protein domain+, induction, coexpression, MS/MS++ MIM Protein import, sorting and homeostasis > Protein homeostasis > Chaperones;
Protein import, sorting and homeostasis > Protein import and sorting cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, heart, adipose, smallintestine, largeintestine, stomach, placenta, testis

MCAT malonyl-CoA-acyl carrier protein transacylase FASN2C, MCT, MCT1, MT, NET62, fabD, Q8IVS2, gene:27349 26 literature, APEX_matrix, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain, coexpression, MS/MS++ Matrix Metabolism > Lipid metabolism > Type II fatty acid synthesis spinalcord, kidney, liver, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis

IBA57 iron-sulfur cluster assembly factor IBA57 C1orf69, MMDS3, SPG74, Q5T440, gene:200205 26 APEX_matrix, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain, coexpression, MS/MS++ Matrix Metabolism > Metals and cofactors > Fe-S cluster biosynthesis cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, heart, adipose, smallintestine, largeintestine, stomach, placenta, testis

PHB2 prohibitin 2 BAP, BCAP37, Bap37, PNAS-141, REA, hBAP, p22, Q99623, gene:11331 26 literature, yeast mito homolog++, Rickettsial homolog, mito protein domain, induction, coexpression, MS/MS++ MIM Protein import, sorting and homeostasis > Protein homeostasis > Chaperones all 14

DAP3 death associated protein 3 DAP-3, MRP-S29, MRPS29, S29mt, bMRP-10, P51398, gene:7818 26 literature, APEX_matrix, targetP signal, yeast mito homolog++, mito protein domain+, coexpression, MS/MS++ Matrix Mitochondrial central dogma > Translation > Mitochondrial ribosome all 14

CMC2 C-X9-C motif containing 2 2310061C15Rik, C16orf61, DC13, Q9NRP2, gene:56942 26 APEX_IMS, yeast mito homolog++, mito protein domain+, induction, coexpression, MS/MS+ IMS OXPHOS > Complex IV > CIV assembly factors;
OXPHOS > OXPHOS assembly factors cerebrum, cerebellum, spinalcord, liver, heart, adipose, largeintestine, stomach, placenta, testis

SDHAF1 succinate dehydrogenase complex assembly factor 1 LYRM8, A6NFY7, gene:644096 26 literature, targetP signal+, yeast mito homolog, mito protein domain+, induction, coexpression+, MS/MS Matrix OXPHOS > Complex II > CII assembly factors;
OXPHOS > OXPHOS assembly factors cerebrum, cerebellum, brainstem, spinalcord, kidney, heart, skeletalmuscle, adipose, largeintestine, stomach, placenta, testis

MRPS17 mitochondrial ribosomal protein S17 HSPC011, MRP-S17, RPMS17, S17mt, Q9Y2R5, gene:51373 26 literature, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain, induction, coexpression, MS/MS+ Matrix Mitochondrial central dogma > Translation > Mitochondrial ribosome all 14

ADHFE1 alcohol dehydrogenase iron containing 1 ADH8, HMFT2263, HOT, Q8IWW8, gene:137872 26 targetP signal+, yeast mito homolog++, mito protein domain, coexpression, MS/MS++ Matrix Metabolism > Amino acid metabolism > GABA metabolism kidney, liver, heart, adipose, stomach, placenta

NFS1 NFS1 cysteine desulfurase HUSSY-08, IscS, NIFS, Q9Y697, gene:9054 26 literature, APEX_matrix, targetP signal, Rickettsial homolog, mito protein domain+, coexpression+, MS/MS++ Matrix Metabolism > Metals and cofactors > Fe-S cluster biosynthesis;
Metabolism > Metals and cofactors > Fe-S-containing proteins all 14

NDUFAF5 NADH:ubiquinone oxidoreductase complex assembly factor 5 C20orf7, MC1DN16, bA526K24.2, dJ842G6.1, Q5TEU4, gene:79133 26 literature, APEX_matrix, GFP, targetP signal+, Rickettsial homolog, mito protein domain+, induction, coexpression, MS/MS MIM OXPHOS > Complex I > CI assembly factors;
OXPHOS > OXPHOS assembly factors cerebellum, brainstem, skeletalmuscle, largeintestine, stomach, placenta

MRPL17 mitochondrial ribosomal protein L17 L17mt, LIP2, MRP-L17, MRP-L26, RPL17L, RPML26, Q9NRX2, gene:63875 26 literature, APEX_matrix, targetP signal+, yeast mito homolog++, Rickettsial homolog, mito protein domain, coexpression, MS/MS Matrix Mitochondrial central dogma > Translation > Mitochondrial ribosome all 14

TACO1 translational activator of cytochrome c oxidase I CCDC44, Q9BSH4, gene:51204 26 literature, APEX_matrix, targetP signal+, yeast mito homolog, Rickettsial homolog, mito protein domain+, MS/MS++ Matrix Mitochondrial central dogma > Translation > Translation factors;
OXPHOS > Complex IV > CIV assembly factors;
OXPHOS > OXPHOS assembly factors all 14

MFN1 mitofusin 1 hfzo1, hfzo2, Q8IWA4, gene:55669 26 literature, yeast mito homolog++, mito protein domain+, induction, coexpression, MS/MS+ MOM Mitochondrial dynamics and surveillance > Fusion;
Mitochondrial dynamics and surveillance > Organelle contact sites cerebellum, brainstem, spinalcord, kidney, skeletalmuscle, adipose, largeintestine, stomach, placenta, testis

PPTC7 protein phosphatase targeting COQ7 TA-PP2C, TAPP2C, Q8NI37, gene:160760 26 APEX_matrix, yeast mito homolog, mito protein domain+, induction, coexpression, MS/MS++ Matrix Signaling cerebrum, cerebellum, brainstem, spinalcord, heart, skeletalmuscle, largeintestine, placenta, testis

MRPL11 mitochondrial ribosomal protein L11 CGI-113, L11MT, MRP-L11, Q9Y3B7, gene:65003 26 literature, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain, coexpression+, MS/MS+ Matrix Mitochondrial central dogma > Translation > Mitochondrial ribosome all 14

COX4I1 cytochrome c oxidase subunit 4I1 COX IV-1, COX4, COX4-1, COXIV, COXIV-1, P13073, gene:1327 26 literature, APEX_IMS, APEX_matrix, targetP signal, mito protein domain+, coexpression++, MS/MS++ MIM OXPHOS > Complex IV > CIV subunits;
OXPHOS > OXPHOS subunits all 14

NDUFS6 NADH:ubiquinone oxidoreductase subunit S6 CI-13kA, CI-13kD-A, CI13KDA, MC1DN9, O75380, gene:4726 26 literature, APEX_matrix, targetP signal, mito protein domain+, coexpression++, MS/MS++ MIM OXPHOS > Complex I > CI subunits;
OXPHOS > OXPHOS subunits all 14

MRPL13 mitochondrial ribosomal protein L13 L13, L13A, L13mt, RPL13, RPML13, Q9BYD1, gene:28998 26 literature, APEX_matrix, targetP signal, yeast mito homolog, Rickettsial homolog, mito protein domain, induction, coexpression, MS/MS++ Matrix Mitochondrial central dogma > Translation > Mitochondrial ribosome all 14

OXCT1 3-oxoacid CoA-transferase 1 OXCT, SCOT, P55809, gene:5019 26 literature, APEX_matrix, targetP signal, mito protein domain, induction, coexpression++, MS/MS++ Matrix Metabolism > Carbohydrate metabolism > Ketone metabolism cerebrum, cerebellum, brainstem, spinalcord, kidney, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis

COX5B cytochrome c oxidase subunit 5B COXVB, P10606, gene:1329 26 literature, APEX_IMS, APEX_matrix, targetP signal+, yeast mito homolog++, mito protein domain+, MS/MS++ MIM OXPHOS > Complex IV > CIV subunits;
OXPHOS > OXPHOS subunits all 14

PDK2 pyruvate dehydrogenase kinase 2 PDHK2, PDKII, Q15119, gene:5164 26 literature, APEX_matrix, yeast mito homolog+, mito protein domain+, induction, coexpression, MS/MS++ Matrix Metabolism > Carbohydrate metabolism > Pyruvate metabolism all 14

ALAS1 5'-aminolevulinate synthase 1 ALAS, ALAS-H, ALAS3, ALASH, MIG4, P13196, gene:211 26 literature, APEX_matrix, targetP signal, yeast mito homolog+, Rickettsial homolog, mito protein domain+, induction, coexpression, MS/MS Matrix Metabolism > Metals and cofactors > Heme synthesis and processing liver, heart, adipose, largeintestine, placenta

MRPL33 mitochondrial ribosomal protein L33 C2orf1, L33mt, MRP-L33, RPL33L, O75394, gene:9553 26 literature, yeast mito homolog++, Rickettsial homolog, mito protein domain, coexpression, MS/MS++ Matrix Mitochondrial central dogma > Translation > Mitochondrial ribosome cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, adipose, smallintestine, stomach, placenta, testis

MTO1 mitochondrial tRNA translation optimization 1 CGI-02, COXPD10, Q9Y2Z2, gene:25821 26 literature, APEX_matrix, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain, coexpression++ Matrix Mitochondrial central dogma > mtRNA metabolism > mt-tRNA modifications;
Metabolism > Vitamin metabolism > Folate and 1-C metabolism cerebellum, spinalcord, stomach, placenta

LIAS lipoic acid synthetase HGCLAS, HUSSY-01, LAS, LIP1, LS, PDHLD, O43766, gene:11019 26 literature, APEX_matrix, targetP signal, yeast mito homolog, Rickettsial homolog, mito protein domain, induction, coexpression++, MS/MS Matrix Metabolism > Lipid metabolism > Lipoate insertion;
Metabolism > Metals and cofactors > Fe-S-containing proteins brainstem, skeletalmuscle, adipose, largeintestine, placenta

NDUFA5 NADH:ubiquinone oxidoreductase subunit A5 B13, CI-13KD-B, CI-13kB, NUFM, UQOR13, Q16718, gene:4698 26 literature, APEX_matrix, mito protein domain+, induction, coexpression++, MS/MS++ MIM OXPHOS > Complex I > CI subunits;
OXPHOS > OXPHOS subunits all 14

NDUFB6 NADH:ubiquinone oxidoreductase subunit B6 B17, CI, O95139, gene:4712 26 literature, APEX_IMS, APEX_matrix, mito protein domain+, induction, coexpression++, MS/MS++ MIM OXPHOS > Complex I > CI subunits;
OXPHOS > OXPHOS subunits all 14

MTX2 metaxin 2 metaxin-2, O75431, gene:10651 26 literature, mito protein domain+, induction, coexpression++, MS/MS++ MOM Protein import, sorting and homeostasis > Protein import and sorting > SAM;
Mitochondrial dynamics and surveillance > Intramitochondrial membrane interactions all 14

SUCLG2 succinate-CoA ligase GDP-forming subunit beta G-SCS, GBETA, GTPSCS, Q96I99, gene:8801 26 literature, APEX_matrix, targetP signal, yeast mito homolog+, Rickettsial homolog, mito protein domain, coexpression, MS/MS++ Matrix Metabolism > Carbohydrate metabolism > TCA cycle;
Metabolism > Carbohydrate metabolism > Itaconate metabolism;
Metabolism > Nucleotide metabolism > Nucleotide synthesis and processing all 14

FDX1 ferredoxin 1 ADX, FDX, LOH11CR1D, P10109, gene:2230 26 literature, APEX_matrix, targetP signal, yeast mito homolog+, Rickettsial homolog, mito protein domain, induction, coexpression, MS/MS+ Matrix 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 > Metals and cofactors > Fe-S-containing proteins;
Metabolism > Vitamin metabolism > Vitamin D metabolism all 14

SLC25A1 solute carrier family 25 member 1 CMS23, CTP, D2L2AD, SEA, SLC20A3, P53007, gene:6576 26 literature, yeast mito homolog++, mito protein domain+, coexpression, MS/MS++ MIM Metabolism > Carbohydrate metabolism > Gluconeogenesis;
Metabolism > Carbohydrate metabolism > TCA-associated;
Metabolism > Lipid metabolism;
Small molecule transport > SLC25A family cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, adipose, smallintestine, largeintestine, stomach, placenta, testis

MRPS28 mitochondrial ribosomal protein S28 COXPD47, HSPC007, MRP-S28, MRP-S35, MRPS35, Q9Y2Q9, gene:28957 26 literature, targetP signal, mito protein domain+, coexpression++, MS/MS++ Matrix Mitochondrial central dogma > Translation > Mitochondrial ribosome all 14

TIMM50 translocase of inner mitochondrial membrane 50 MGCA9, TIM50, TIM50L, Q3ZCQ8, gene:92609 26 literature, APEX_IMS, yeast mito homolog++, mito protein domain, coexpression++, MS/MS++ MIM Protein import, sorting and homeostasis > Protein import and sorting > TIM23 presequence pathway all 14

VDAC2 voltage dependent anion channel 2 POR, P45880, gene:7417 26 literature, APEX_IMS, yeast mito homolog+, mito protein domain+, induction, coexpression, MS/MS++ MOM Small molecule transport all 14

SLC25A42 solute carrier family 25 member 42 MECREN, Q86VD7, gene:284439 26 literature, yeast mito homolog+, mito protein domain+, induction, coexpression, MS/MS++ MIM Metabolism > Metals and cofactors > Coenzyme A metabolism;
Small molecule transport > SLC25A family all 14

TOMM7 translocase of outer mitochondrial membrane 7 TOM7, Q9P0U1, gene:54543 26 literature, yeast mito homolog++, mito protein domain+, coexpression, MS/MS++ MOM Protein import, sorting and homeostasis > Protein import and sorting > TOM;
Mitochondrial dynamics and surveillance > Mitophagy;
Mitochondrial dynamics and surveillance > Autophagy cerebrum, cerebellum, spinalcord, kidney, liver, skeletalmuscle, adipose, smallintestine, stomach, placenta, testis

ECH1 enoyl-CoA hydratase 1 HPXEL, Q13011, gene:1891 26 APEX_matrix, targetP signal+, yeast mito homolog+, mito protein domain, coexpression+, MS/MS++ Matrix Metabolism > Lipid metabolism all 14

GATB glutamyl-tRNA amidotransferase subunit B COXPD41, HSPC199, PET112, PET112L, O75879, gene:5188 25 literature, APEX_matrix, targetP signal, yeast mito homolog, Rickettsial homolog, mito protein domain+, coexpression+, MS/MS Matrix Mitochondrial central dogma > Translation > mt-tRNA synthetases cerebrum, skeletalmuscle, adipose, stomach, testis

BCS1L BCS1 homolog, ubiquinol-cytochrome c reductase complex chaperone BCS, BCS1, BJS, FLNMS, GRACILE, Hs.6719, MC3DN1, PTD, h-BCS, h-BCS1, Q9Y276, gene:617 25 literature, APEX_matrix, yeast mito homolog++, Rickettsial homolog, mito protein domain+, coexpression+, MS/MS+ MIM OXPHOS > Complex III > CIII assembly factors;
OXPHOS > OXPHOS assembly factors cerebrum, cerebellum, brainstem, spinalcord, liver, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis

ERAL1 Era like 12S mitochondrial rRNA chaperone 1 CEGA, ERA, ERA-W, ERAL1A, ERAL1B, H-ERA, HERA-A, HERA-B, PRLTS6, O75616, gene:26284 25 APEX_matrix, targetP signal+, yeast mito homolog+, Rickettsial homolog, mito protein domain, induction, coexpression++ Matrix Mitochondrial central dogma > mtRNA metabolism > mtRNA granules;
Mitochondrial central dogma > Translation > Mitochondrial ribosome assembly none

CMC1 C-X9-C motif containing 1 C3orf68, Q7Z7K0, gene:152100 25 APEX_IMS, GFP, yeast mito homolog++, mito protein domain+, induction, coexpression, MS/MS+ IMS OXPHOS > Complex IV > CIV assembly factors;
OXPHOS > OXPHOS assembly factors cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, adipose, smallintestine, largeintestine, stomach, placenta, testis

MRPL28 mitochondrial ribosomal protein L28 MAAT1, p15, Q13084, gene:10573 25 literature, APEX_matrix, yeast mito homolog++, Rickettsial homolog, coexpression, MS/MS++ Matrix Mitochondrial central dogma > Translation > Mitochondrial ribosome cerebrum, cerebellum, brainstem, spinalcord, liver, heart, adipose, smallintestine, largeintestine, stomach, placenta, testis

TSFM Ts translation elongation factor, mitochondrial EFTS, EFTSMT, P43897, gene:10102 25 literature, APEX_matrix, targetP signal+, Rickettsial homolog, mito protein domain, induction, coexpression, MS/MS++ Matrix Mitochondrial central dogma > Translation > Translation factors cerebrum, cerebellum, brainstem, spinalcord, liver, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis

FXN frataxin CyaY, FA, FARR, FRDA, X25, Q16595, gene:2395 25 literature, APEX_matrix, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain, coexpression, MS/MS+ Matrix Metabolism > Metals and cofactors > Fe-S cluster biosynthesis all 14

NFU1 NFU1 iron-sulfur cluster scaffold CGI-33, HIRIP, HIRIP5, MMDS1, NIFUC, Nfu, NifU, Q9UMS0, gene:27247 25 APEX_matrix, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain, coexpression, MS/MS+ Matrix Metabolism > Metals and cofactors > Fe-S cluster biosynthesis;
Metabolism > Metals and cofactors > Fe-S-containing proteins all 14

YARS2 tyrosyl-tRNA synthetase 2 CGI-04, MLASA2, MT-TYRRS, TYRRS, Q9Y2Z4, gene:51067 25 literature, APEX_matrix, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain, coexpression, MS/MS+ Matrix Mitochondrial central dogma > Translation > mt-tRNA synthetases brainstem, spinalcord, kidney, adipose, smallintestine, largeintestine, stomach, placenta, testis

MRPL21 mitochondrial ribosomal protein L21 L21mt, MRP-L21, Q7Z2W9, gene:219927 25 literature, APEX_matrix, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain, coexpression, MS/MS+ Matrix Mitochondrial central dogma > Translation > Mitochondrial ribosome all 14

ALDH4A1 aldehyde dehydrogenase 4 family member A1 ALDH4, P5CD, P5CDh, P30038, gene:8659 25 literature, APEX_matrix, targetP signal+, yeast mito homolog++, mito protein domain, coexpression, MS/MS++ Matrix Metabolism > Amino acid metabolism > Proline metabolism;
Metabolism > Amino acid metabolism > Glyoxylate metabolism all 14

TOMM40L translocase of outer mitochondrial membrane 40 like TOMM40B, Q969M1, gene:84134 25 targetP signal, yeast mito homolog+, mito protein domain+, induction, coexpression+, MS/MS MOM Protein import, sorting and homeostasis > Protein import and sorting > TOM cerebrum, cerebellum, brainstem, spinalcord, adipose, largeintestine, stomach, testis

NDUFAF1 NADH:ubiquinone oxidoreductase complex assembly factor 1 CGI-65, CGI65, CIA30, MC1DN11, Q9Y375, gene:51103 25 literature, APEX_matrix, targetP signal, mito protein domain, induction, coexpression++, MS/MS++ MIM OXPHOS > Complex I > CI assembly factors;
OXPHOS > OXPHOS assembly factors cerebellum, brainstem, spinalcord, heart, adipose, smallintestine, largeintestine, stomach, placenta, testis

NDUFB10 NADH:ubiquinone oxidoreductase subunit B10 PDSW, O96000, gene:4716 25 literature, APEX_matrix, mito protein domain+, induction, coexpression++, MS/MS++ MIM OXPHOS > Complex I > CI subunits;
OXPHOS > OXPHOS subunits all 14

ACADSB acyl-CoA dehydrogenase short/branched chain 2-MEBCAD, ACAD7, SBCAD, P45954, gene:36 25 literature, APEX_matrix, targetP signal, mito protein domain+, induction, coexpression, MS/MS++ Matrix Metabolism > Lipid metabolism > Fatty acid oxidation;
Metabolism > Amino acid metabolism > BCAA metabolism all 14

MRPS9 mitochondrial ribosomal protein S9 MRP-S9, RPMS9, S9mt, P82933, gene:64965 25 literature, APEX_matrix, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain, induction, coexpression, MS/MS Matrix Mitochondrial central dogma > mtRNA metabolism > mtRNA granules;
Mitochondrial central dogma > Translation > Mitochondrial ribosome all 14

COQ8A coenzyme Q8A ADCK3, ARCA2, CABC1, COQ10D4, COQ8, SCAR9, Q8NI60, gene:56997 25 literature, APEX_matrix, yeast mito homolog+, Rickettsial homolog, mito protein domain, induction, coexpression+, MS/MS++ MIM Metabolism > Metals and cofactors > Coenzyme Q metabolism all 14

ACADVL acyl-CoA dehydrogenase very long chain ACAD6, LCACD, VLCAD, P49748, gene:37 25 literature, APEX_matrix, targetP signal+, mito protein domain+, coexpression+, MS/MS++ MIM Metabolism > Lipid metabolism > Fatty acid oxidation all 14

COQ4 coenzyme Q4 CGI-92, COQ10D7, Q9Y3A0, gene:51117 25 literature, targetP signal+, yeast mito homolog++, mito protein domain+, coexpression, MS/MS MIM Metabolism > Metals and cofactors > Coenzyme Q metabolism cerebrum, cerebellum, brainstem, kidney, liver, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta

MRPL15 mitochondrial ribosomal protein L15 HSPC145, L15mt, MRP-L15, MRP-L7, RPML7, Q9P015, gene:29088 25 literature, APEX_matrix, yeast mito homolog++, Rickettsial homolog, mito protein domain, induction, coexpression, MS/MS Matrix Mitochondrial central dogma > Translation > Mitochondrial ribosome cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, adipose, smallintestine, largeintestine, stomach, placenta, testis

NDUFA2 NADH:ubiquinone oxidoreductase subunit A2 B8, CD14, CIB8, MC1DN13, O43678, gene:4695 25 literature, APEX_matrix, targetP signal, mito protein domain, induction, coexpression++, MS/MS++ MIM OXPHOS > Complex I > CI subunits;
OXPHOS > OXPHOS subunits all 14

MRPL24 mitochondrial ribosomal protein L24 L24mt, MRP-L18, MRP-L24, Q96A35, gene:79590 25 literature, APEX_matrix, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain, coexpression, MS/MS++ Matrix Mitochondrial central dogma > Translation > Mitochondrial ribosome cerebrum, cerebellum, brainstem, spinalcord, liver, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis

HSPD1 heat shock protein family D (Hsp60) member 1 CPN60, GROEL, HLD4, HSP-60, HSP60, HSP65, HuCHA60, SPG13, P10809, gene:3329 25 literature, APEX_matrix, targetP signal+, yeast mito homolog++, Rickettsial homolog, mito protein domain, MS/MS++ Matrix Protein import, sorting and homeostasis > Protein homeostasis > Chaperones all 14

NDUFS5 NADH:ubiquinone oxidoreductase subunit S5 CI-15k, CI15K, O43920, gene:4725 25 literature, APEX_IMS, mito protein domain+, induction, coexpression++, MS/MS++ MIM OXPHOS > Complex I > CI subunits;
OXPHOS > OXPHOS subunits all 14

AK3 adenylate kinase 3 AK3L1, AK6, AKL3L, AKL3L1, FIX, Q9UIJ7, gene:50808 25 literature, APEX_matrix, targetP signal, Rickettsial homolog, mito protein domain, induction, coexpression, MS/MS++ Matrix Metabolism > Nucleotide metabolism > Nucleotide synthesis and processing all 14

CYCS cytochrome c, somatic CYC, HCS, THC4, P99999, gene:54205 24 literature, APEX_IMS, yeast mito homolog++, Rickettsial homolog, mito protein domain+, MS/MS++ IMS Metabolism > Metals and cofactors > Heme-containing proteins;
Metabolism > Electron carriers > Cytochromes;
OXPHOS > OXPHOS subunits;
OXPHOS > Cytochrome C;
Mitochondrial dynamics and surveillance > Apoptosis all 14

MIPEP mitochondrial intermediate peptidase COXPD31, HMIP, MIP, Q99797, gene:4285 24 literature, APEX_matrix, targetP signal+, yeast mito homolog++, mito protein domain, induction, MS/MS++ Matrix Protein import, sorting and homeostasis > Protein import and sorting > Preprotein cleavage;
Protein import, sorting and homeostasis > Protein homeostasis > Proteases cerebrum, spinalcord, kidney, liver, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis

LYRM7 LYR motif containing 7 C5orf31, MC3DN8, MZM1L, Q5U5X0, gene:90624 24 literature, APEX_matrix, targetP signal, yeast mito homolog++, mito protein domain+, MS/MS++ Matrix OXPHOS > Complex III > CIII assembly factors;
OXPHOS > OXPHOS assembly factors cerebrum, cerebellum, brainstem, spinalcord, liver, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis

CRAT carnitine O-acetyltransferase CAT, CAT1, NBIA8, P43155, gene:1384 24 literature, APEX_matrix, targetP signal, yeast mito homolog+, mito protein domain, induction, coexpression, MS/MS++ MIM Metabolism > Lipid metabolism > Fatty acid oxidation all 14

PCCB propionyl-CoA carboxylase subunit beta P05166, gene:5096 24 literature, targetP signal+, Rickettsial homolog, mito protein domain, coexpression+, MS/MS++ Matrix Metabolism > Carbohydrate metabolism > Propanoate metabolism;
Metabolism > Lipid metabolism > Fatty acid oxidation all 14

MRPS7 mitochondrial ribosomal protein S7 COXPD34, MRP-S, MRP-S7, RP-S7, RPMS7, S7mt, bMRP27a, Q9Y2R9, gene:51081 24 literature, APEX_matrix, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain, coexpression+, MS/MS Matrix Mitochondrial central dogma > mtRNA metabolism > mtRNA granules;
Mitochondrial central dogma > Translation > Mitochondrial ribosome all 14

MRPL3 mitochondrial ribosomal protein L3 COXPD9, MRL3, RPML3, P09001, gene:11222 24 literature, APEX_matrix, yeast mito homolog++, Rickettsial homolog, mito protein domain, induction, coexpression Matrix Mitochondrial central dogma > Translation > Mitochondrial ribosome cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, heart, skeletalmuscle, adipose, smallintestine, largeintestine, placenta, testis

PRODH proline dehydrogenase 1 HSPOX2, PIG6, POX, PRODH1, PRODH2, TP53I6, O43272, gene:5625 24 literature, targetP signal, yeast mito homolog+, mito protein domain+, coexpression, MS/MS++ MIM Metabolism > Amino acid metabolism > Proline metabolism;
Metabolism > Electron carriers > Q-linked reactions, other cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis

PCCA propionyl-CoA carboxylase subunit alpha P05165, gene:5095 24 literature, targetP signal, Rickettsial homolog, mito protein domain, induction, coexpression+, MS/MS++ Matrix Metabolism > Carbohydrate metabolism > Propanoate metabolism;
Metabolism > Lipid metabolism > Fatty acid oxidation;
Metabolism > Vitamin metabolism > Biotin utilizing proteins all 14

MCCC1 methylcrotonoyl-CoA carboxylase 1 MCC-B, MCCA, Q96RQ3, gene:56922 24 literature, targetP signal, Rickettsial homolog, mito protein domain, induction, coexpression+, MS/MS++ Matrix Metabolism > Amino acid metabolism > BCAA metabolism;
Metabolism > Vitamin metabolism > Biotin utilizing proteins all 14

MRPS12 mitochondrial ribosomal protein S12 MPR-S12, MT-RPS12, RPMS12, RPS12, RPSM12, O15235, gene:6183 24 literature, yeast mito homolog++, Rickettsial homolog, mito protein domain, induction, coexpression+, MS/MS+ Matrix Mitochondrial central dogma > Translation > Mitochondrial ribosome cerebrum, cerebellum, brainstem, spinalcord, liver, heart, adipose, smallintestine, largeintestine, stomach, placenta, testis

CLPB caseinolytic mitochondrial matrix peptidase chaperone subunit B ANKCLB, HSP78, MEGCANN, MGCA7, SKD3, Q9H078, gene:81570 24 APEX_IMS, targetP signal+, yeast mito homolog+, Rickettsial homolog, mito protein domain, MS/MS++ IMS Protein import, sorting and homeostasis > Protein homeostasis > Proteases cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis

PDK1 pyruvate dehydrogenase kinase 1 Q15118, gene:5163 24 literature, APEX_matrix, targetP signal+, yeast mito homolog+, mito protein domain+, MS/MS++ Matrix Metabolism > Carbohydrate metabolism > Pyruvate metabolism all 14

MRPL49 mitochondrial ribosomal protein L49 C11orf4, L49mt, MRP-L49, NOF, NOF1, Q13405, gene:740 24 literature, APEX_matrix, targetP signal, yeast mito homolog++, mito protein domain, coexpression, MS/MS++ Matrix Mitochondrial central dogma > Translation > Mitochondrial ribosome all 14

COX7A2 cytochrome c oxidase subunit 7A2 COX7AL, COX7AL1, COXVIIAL, COXVIIa-L, VIIAL, P14406, gene:1347 24 literature, mito protein domain+, induction, coexpression++, MS/MS++ MIM OXPHOS > Complex IV > CIV subunits;
OXPHOS > OXPHOS subunits all 14

TMEM126A transmembrane protein 126A OPA7, Q9H061, gene:84233 24 literature, mito protein domain+, induction, coexpression++, MS/MS++ MIM OXPHOS > Complex I > CI assembly factors;
OXPHOS > OXPHOS assembly factors all 14

ECHDC2 enoyl-CoA hydratase domain containing 2 Q86YB7, gene:55268 24 targetP signal, yeast mito homolog+, Rickettsial homolog, mito protein domain, coexpression, MS/MS++ Matrix none all 14

HCCS holocytochrome c synthase CCHL, LSDMCA1, MCOPS7, MLS, P53701, gene:3052 24 literature, APEX_IMS, yeast mito homolog++, mito protein domain+, induction, coexpression, MS/MS IMS Metabolism > Metals and cofactors > Heme synthesis and processing;
OXPHOS > OXPHOS subunits;
OXPHOS > Cytochrome C cerebrum, spinalcord, heart, adipose, smallintestine, largeintestine, placenta, testis

HIBADH 3-hydroxyisobutyrate dehydrogenase NS5ATP1, P31937, gene:11112 24 literature, APEX_matrix, targetP signal+, mito protein domain, induction, coexpression+, MS/MS++ Matrix Metabolism > Amino acid metabolism > BCAA metabolism all 14

MRPL19 mitochondrial ribosomal protein L19 L19mt, MRP-L15, MRP-L19, MRPL15, RLX1, RPML15, P49406, gene:9801 24 literature, APEX_matrix, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain, coexpression, MS/MS+ Matrix Mitochondrial central dogma > Translation > Mitochondrial ribosome cerebrum, cerebellum, brainstem, spinalcord, liver, adipose, smallintestine, largeintestine, stomach, placenta, testis

MRPL36 mitochondrial ribosomal protein L36 BRIP1, L36mt, MRP-L36, PRPL36, RPMJ, Q9P0J6, gene:64979 24 literature, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain, induction, coexpression+ Matrix Mitochondrial central dogma > Translation > Mitochondrial ribosome none

SLC25A30 solute carrier family 25 member 30 KMCP1, Q5SVS4, gene:253512 24 literature, yeast mito homolog+, mito protein domain+, coexpression, MS/MS++ MIM Metabolism > Sulfur metabolism;
Small molecule transport > SLC25A family cerebrum, cerebellum, liver, placenta, testis

BDH1 3-hydroxybutyrate dehydrogenase 1 BDH, SDR9C1, Q02338, gene:622 24 literature, APEX_matrix, targetP signal+, Rickettsial homolog, mito protein domain, induction, coexpression, MS/MS++ MIM Metabolism > Carbohydrate metabolism > Ketone metabolism all 14

FARS2 phenylalanyl-tRNA synthetase 2, mitochondrial COXPD14, FARS1, HSPC320, PheRS, SPG77, mtPheRS, O95363, gene:10667 23 literature, APEX_matrix, targetP signal, yeast mito homolog+, Rickettsial homolog, mito protein domain, induction, coexpression, MS/MS Matrix Mitochondrial central dogma > Translation > mt-tRNA synthetases adipose, largeintestine, stomach, placenta, testis

ABCB7 ATP binding cassette subfamily B member 7 ABC7, ASAT, Atm1p, EST140535, O75027, gene:22 23 literature, APEX_matrix, targetP signal, yeast mito homolog+, Rickettsial homolog, mito protein domain, coexpression+, MS/MS+ MIM 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 all 14

MTX1 metaxin 1 MTX, MTXN, Q13505, gene:4580 23 literature, yeast mito homolog++, mito protein domain+, coexpression, MS/MS++ MOM Protein import, sorting and homeostasis > Protein import and sorting > SAM;
Mitochondrial dynamics and surveillance > Intramitochondrial membrane interactions all 14

NDUFA7 NADH:ubiquinone oxidoreductase subunit A7 B14.5a, CI-B14.5a, O95182, gene:4701 23 literature, APEX_matrix, mito protein domain+, coexpression++, MS/MS++ MIM OXPHOS > Complex I > CI subunits;
OXPHOS > OXPHOS subunits all 14

TIMM17A translocase of inner mitochondrial membrane 17A TIM17, TIM17A, Q99595, gene:10440 23 literature, APEX_IMS, yeast mito homolog+, mito protein domain+, induction, coexpression, MS/MS+ MIM Protein import, sorting and homeostasis > Protein import and sorting > TIM23 presequence pathway all 14

ALDH9A1 aldehyde dehydrogenase 9 family member A1 ALDH4, ALDH7, ALDH9, E3, TMABA-DH, TMABADH, TMABALDH, P49189, gene:223 23 APEX_matrix, targetP signal+, yeast mito homolog+, mito protein domain, coexpression, MS/MS++ Matrix Metabolism > Amino acid metabolism > GABA metabolism;
Metabolism > Detoxification > ROS and glutathione metabolism brainstem, spinalcord, kidney, liver, adipose, smallintestine, largeintestine, stomach, placenta, testis

MRPS18C mitochondrial ribosomal protein S18C CGI-134, MRP-S18-1, MRP-S18-c, MRPS18-1, S18mt-c, mrps18-c, Q9Y3D5, gene:51023 23 literature, yeast mito homolog++, Rickettsial homolog, mito protein domain, coexpression, MS/MS++ Matrix Mitochondrial central dogma > Translation > Mitochondrial ribosome cerebrum, cerebellum, brainstem, spinalcord, liver, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis

MARS2 methionyl-tRNA synthetase 2, mitochondrial COXPD25, MetRS, mtMetRS, Q96GW9, gene:92935 23 literature, APEX_matrix, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain, coexpression, MS/MS+ Matrix Mitochondrial central dogma > Translation > mt-tRNA synthetases cerebrum, cerebellum, brainstem, spinalcord, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis

ALDH6A1 aldehyde dehydrogenase 6 family member A1 MMSADHA, MMSDH, Q02252, gene:4329 23 literature, APEX_matrix, targetP signal, yeast mito homolog+, mito protein domain, coexpression, MS/MS++ MIM Metabolism > Amino acid metabolism > BCAA metabolism all 14

FDXR ferredoxin reductase ADR, ADXR, ANOA, P22570, gene:2232 23 literature, APEX_matrix, targetP signal+, yeast mito homolog++, coexpression, MS/MS++ Matrix 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 spinalcord, kidney, liver, adipose, smallintestine, largeintestine, stomach, placenta, testis

GATC glutamyl-tRNA amidotransferase subunit C 15E1.2, COXPD42, O43716, gene:283459 23 literature, APEX_matrix, targetP signal, Rickettsial homolog, mito protein domain+, induction, coexpression, MS/MS Matrix Mitochondrial central dogma > Translation > mt-tRNA synthetases cerebrum, cerebellum, spinalcord, kidney, largeintestine, testis

TRAP1 TNF receptor associated protein 1 HSP 75, HSP75, HSP90L, TRAP-1, Q12931, gene:10131 23 APEX_matrix, GFP, targetP signal+, Rickettsial homolog, mito protein domain, coexpression++, MS/MS+ Matrix Protein import, sorting and homeostasis > Protein homeostasis > Chaperones all 14

ACAD8 acyl-CoA dehydrogenase family member 8 ACAD-8, ARC42, IBDH, Q9UKU7, gene:27034 22 literature, APEX_matrix, targetP signal+, mito protein domain+, coexpression, MS/MS++ Matrix Metabolism > Amino acid metabolism > BCAA metabolism all 14

ALDH2 aldehyde dehydrogenase 2 family member ALDH-E2, ALDHI, ALDM, P05091, gene:217 22 literature, APEX_matrix, targetP signal+, yeast mito homolog+, mito protein domain, coexpression, MS/MS++ Matrix Metabolism > Detoxification > Xenobiotic metabolism all 14

PPIF peptidylprolyl isomerase F CYP3, CyP-M, Cyp-D, CypD, P30405, gene:10105 22 literature, APEX_matrix, targetP signal+, mito protein domain, induction, coexpression, MS/MS++ Matrix Signaling > Calcium homeostasis > Mitochondrial permeability transition pore all 14

TIMM22 translocase of inner mitochondrial membrane 22 COXPD43, TEX4, TIM22, Q9Y584, gene:29928 22 literature, yeast mito homolog++, mito protein domain+, induction, coexpression, MS/MS MIM Protein import, sorting and homeostasis > Protein import and sorting > TIM22 carrier pathway all 14

IVD isovaleryl-CoA dehydrogenase ACAD2, P26440, gene:3712 22 literature, APEX_matrix, targetP signal, mito protein domain+, coexpression+, MS/MS++ Matrix Metabolism > Amino acid metabolism > BCAA metabolism all 14

L2HGDH L-2-hydroxyglutarate dehydrogenase C14orf160, L2HGA, Q9H9P8, gene:79944 22 literature, APEX_matrix, targetP signal+, mito protein domain, induction, coexpression, MS/MS++ MIM Metabolism > Carbohydrate metabolism > TCA-associated cerebrum, spinalcord, kidney, liver, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, testis

ETHE1 ETHE1 persulfide dioxygenase HSCO, YF13H12, O95571, gene:23474 22 literature, APEX_matrix, targetP signal, yeast mito homolog+, mito protein domain, induction, MS/MS++ Matrix Metabolism > Sulfur metabolism cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta

MRPL20 mitochondrial ribosomal protein L20 L20mt, MRP-L20, Q9BYC9, gene:55052 22 literature, APEX_matrix, targetP signal+, Rickettsial homolog, mito protein domain, induction, coexpression, MS/MS Matrix Mitochondrial central dogma > Translation > Mitochondrial ribosome cerebrum, cerebellum, brainstem, spinalcord, liver, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis

SLC25A5 solute carrier family 25 member 5 2F1, AAC2, ANT2, T2, T3, P05141, gene:292 22 literature, yeast mito homolog+, mito protein domain+, induction, MS/MS++ MIM Metabolism > Nucleotide metabolism > Nucleotide import;
Signaling > Calcium homeostasis > Mitochondrial permeability transition pore;
Small molecule transport > SLC25A family all 14

SLC25A12 solute carrier family 25 member 12 AGC1, ARALAR, EIEE39, O75746, gene:8604 22 literature, yeast mito homolog+, mito protein domain+, induction, MS/MS++ MIM Metabolism > Carbohydrate metabolism > Malate-aspartate shuttle;
Metabolism > Amino acid metabolism > Glutamate metabolism;
Signaling > Calcium homeostasis > EF hand proteins;
Small molecule transport > SLC25A family all 14

MRPS21 mitochondrial ribosomal protein S21 MDS016, MRP-S21, RPMS21, P82921, gene:54460 22 literature, yeast mito homolog++, Rickettsial homolog, mito protein domain, induction, MS/MS++ Matrix Mitochondrial central dogma > Translation > Mitochondrial ribosome all 14

TOMM22 translocase of outer mitochondrial membrane 22 1C9-2, MST065, MSTP065, TOM22, Q9NS69, gene:56993 22 literature, yeast mito homolog++, mito protein domain+, coexpression, MS/MS++ MOM Protein import, sorting and homeostasis > Protein import and sorting > TOM all 14

ACAA2 acetyl-CoA acyltransferase 2 DSAEC, P42765, gene:10449 22 literature, APEX_matrix, targetP signal, Rickettsial homolog, mito protein domain, induction, coexpression+, MS/MS++ Matrix Metabolism > Carbohydrate metabolism > Ketone metabolism;
Metabolism > Lipid metabolism > Fatty acid oxidation;
Metabolism > Amino acid metabolism > Lysine metabolism all 14

MRPL30 mitochondrial ribosomal protein L30 L28MT, L30MT, MRP-L28, MRP-L30, MRPL28, MRPL28M, RPML28, Q8TCC3, gene:51263 22 literature, APEX_matrix, yeast mito homolog++, mito protein domain, coexpression++, MS/MS Matrix Mitochondrial central dogma > Translation > Mitochondrial ribosome all 14

DNAJA3 DnaJ heat shock protein family (Hsp40) member A3 HCA57, TID1, hTID-1, Q96EY1, gene:9093 22 literature, APEX_matrix, targetP signal, Rickettsial homolog, mito protein domain, induction, coexpression++, MS/MS+ Matrix Protein import, sorting and homeostasis > Protein homeostasis > Chaperones all 14

NDUFB2 NADH:ubiquinone oxidoreductase subunit B2 AGGG, CI-AGGG, O95178, gene:4708 22 literature, targetP signal, induction, coexpression++, MS/MS++ MIM OXPHOS > Complex I > CI subunits;
OXPHOS > OXPHOS subunits all 14

MRPS34 mitochondrial ribosomal protein S34 COXPD32, MRP-S12, MRP-S34, MRPS12, P82930, gene:65993 22 literature, APEX_matrix, targetP signal, induction, coexpression++, MS/MS++ Matrix Mitochondrial central dogma > Translation > Mitochondrial ribosome all 14

ETFB electron transfer flavoprotein subunit beta FP585, MADD, P38117, gene:2109 22 literature, APEX_matrix, yeast mito homolog, mito protein domain, induction, coexpression+, MS/MS++ Matrix Metabolism > Lipid metabolism > Fatty acid oxidation;
Metabolism > Amino acid metabolism > BCAA 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 all 14

AFG1L AFG1 like ATPase AFG1, LACE1, c222389, Q8WV93, gene:246269 22 APEX_matrix, yeast mito homolog++, Rickettsial homolog, coexpression, MS/MS+ MIM Protein import, sorting and homeostasis > Protein homeostasis > Proteases cerebrum, cerebellum, spinalcord, heart, adipose, smallintestine, largeintestine, stomach, placenta

ATP5IF1 ATP synthase inhibitory factor subunit 1 ATPI, ATPIF1, ATPIP, IP, Q9UII2, gene:93974 22 literature, APEX_matrix, targetP signal, yeast mito homolog++, mito protein domain+, MS/MS++ Matrix OXPHOS > Complex V > CV subunits;
OXPHOS > OXPHOS subunits all 14

ATP5PB ATP synthase peripheral stalk-membrane subunit b ATP5F1, PIG47, P24539, gene:515 22 literature, APEX_matrix, yeast mito homolog++, mito protein domain+, MS/MS++ MIM OXPHOS > Complex V > CV subunits;
OXPHOS > OXPHOS subunits all 14

COX7C cytochrome c oxidase subunit 7C P15954, gene:1350 22 literature, APEX_IMS, yeast mito homolog++, mito protein domain+, MS/MS++ MIM OXPHOS > Complex IV > CIV subunits;
OXPHOS > OXPHOS subunits all 14

CHCHD3 coiled-coil-helix-coiled-coil-helix domain containing 3 MICOS19, MINOS3, Mic19, PPP1R22, Q9NX63, gene:54927 22 literature, APEX_IMS, APEX_matrix, GFP, yeast mito homolog, induction, coexpression++, MS/MS++ MIM Mitochondrial dynamics and surveillance > Cristae formation > MICOS complex;
Mitochondrial dynamics and surveillance > Intramitochondrial membrane interactions all 14

COQ10B coenzyme Q10B Q9H8M1, gene:80219 22 GFP, targetP signal, yeast mito homolog+, Rickettsial homolog, mito protein domain+, MS/MS+ MIM Metabolism > Metals and cofactors > Coenzyme Q metabolism cerebrum, cerebellum, brainstem, spinalcord, liver, heart, skeletalmuscle, adipose, largeintestine, stomach, placenta

ACSF3 acyl-CoA synthetase family member 3 Q4G176, gene:197322 22 APEX_matrix, targetP signal+, Rickettsial homolog, mito protein domain, coexpression+, MS/MS++ Matrix Metabolism > Lipid metabolism > Type II fatty acid synthesis all 14

POLDIP2 DNA polymerase delta interacting protein 2 PDIP38, POLD4, p38, Q9Y2S7, gene:26073 22 literature, APEX_matrix, targetP signal+, induction, coexpression++, MS/MS++ Matrix Mitochondrial central dogma > mtDNA maintenance > mtDNA nucleoid all 14

SLC25A10 solute carrier family 25 member 10 DIC, MTDPS19, Q9UBX3, gene:1468 22 literature, yeast mito homolog+, mito protein domain+, coexpression, MS/MS++ MIM Metabolism > Carbohydrate metabolism > Gluconeogenesis;
Metabolism > Carbohydrate metabolism > Malate-aspartate shuttle;
Small molecule transport > SLC25A family cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, adipose, smallintestine, largeintestine, stomach, placenta, testis

SLC25A13 solute carrier family 25 member 13 ARALAR2, CITRIN, CTLN2, NICCD, Q9UJS0, gene:10165 22 literature, yeast mito homolog+, mito protein domain+, coexpression, MS/MS++ MIM Metabolism > Carbohydrate metabolism > Malate-aspartate shuttle;
Metabolism > Amino acid metabolism > Urea cycle;
Signaling > Calcium homeostasis > EF hand proteins;
Small molecule transport > SLC25A family all 14

PDK3 pyruvate dehydrogenase kinase 3 CMTX6, GS1-358P8.4, Q15120, gene:5165 21 literature, APEX_matrix, targetP signal, yeast mito homolog+, mito protein domain+, MS/MS++ Matrix Metabolism > Carbohydrate metabolism > Pyruvate metabolism cerebrum, cerebellum, brainstem, spinalcord, kidney, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis

ME3 malic enzyme 3 NADP-ME, Q16798, gene:10873 21 literature, yeast mito homolog+, Rickettsial homolog, mito protein domain, coexpression, MS/MS++ Matrix Metabolism > Carbohydrate metabolism > TCA-associated cerebrum, cerebellum, brainstem, spinalcord, kidney, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis

MRPL22 mitochondrial ribosomal protein L22 HSPC158, L22mt, MRP-L22, MRP-L25, RPML25, Q9NWU5, gene:29093 21 literature, APEX_matrix, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain, MS/MS++ Matrix Mitochondrial central dogma > Translation > Mitochondrial ribosome all 14

IDH2 isocitrate dehydrogenase (NADP(+)) 2 D2HGA2, ICD-M, IDH, IDHM, IDP, IDPM, mNADP-IDH, P48735, gene:3418 21 literature, APEX_matrix, targetP signal+, Rickettsial homolog, mito protein domain, induction, coexpression, MS/MS++ Matrix Metabolism > Carbohydrate metabolism > TCA cycle all 14

GCDH glutaryl-CoA dehydrogenase ACAD5, GCD, Q92947, gene:2639 21 literature, APEX_matrix, targetP signal+, mito protein domain+, coexpression, MS/MS++ Matrix Metabolism > Amino acid metabolism > Lysine metabolism all 14

MRPL47 mitochondrial ribosomal protein L47 CGI-204, L47mt, MRP-L47, NCM1, Q9HD33, gene:57129 21 literature, APEX_matrix, targetP signal+, yeast mito homolog++, mito protein domain, induction, coexpression Matrix Mitochondrial central dogma > mtRNA metabolism > mtRNA granules;
Mitochondrial central dogma > Translation > Mitochondrial ribosome all 14

PPA2 inorganic pyrophosphatase 2 HSPC124, SCFAI, SCFI, SID6-306, Q9H2U2, gene:27068 21 literature, APEX_matrix, targetP signal+, Rickettsial homolog, mito protein domain, coexpression, MS/MS++ Matrix Mitochondrial central dogma > mtDNA maintenance;
Mitochondrial central dogma > mtRNA metabolism;
Mitochondrial central dogma > Translation;
Metabolism > Nucleotide metabolism all 14

MRPL9 mitochondrial ribosomal protein L9 L9mt, Q9BYD2, gene:65005 21 literature, APEX_matrix, targetP signal, Rickettsial homolog, mito protein domain, induction, coexpression+, MS/MS Matrix Mitochondrial central dogma > Translation > Mitochondrial ribosome all 14

CHCHD10 coiled-coil-helix-coiled-coil-helix domain containing 10 C22orf16, FTDALS2, IMMD, MIX17A, N27C7-4, SMAJ, Q8WYQ3, gene:400916 21 literature, targetP signal, yeast mito homolog+, mito protein domain, induction, coexpression++ IMS none none

WARS2 tryptophanyl tRNA synthetase 2, mitochondrial NEMMLAS, TrpRS, mtTrpRS, Q9UGM6, gene:10352 21 literature, APEX_matrix, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain, coexpression, MS/MS Matrix Mitochondrial central dogma > Translation > mt-tRNA synthetases cerebrum, cerebellum, brainstem, kidney, skeletalmuscle, adipose, largeintestine, placenta, testis

SLC25A19 solute carrier family 25 member 19 DNC, MCPHA, MUP1, THMD3, THMD4, TPC, Q9HC21, gene:60386 21 literature, yeast mito homolog++, mito protein domain+, induction, coexpression, MS/MS MIM Metabolism > Vitamin metabolism > Vitamin B1 metabolism;
Small molecule transport > SLC25A family cerebellum, spinalcord, adipose, testis

CBR4 carbonyl reductase 4 SDR45C1, Q8N4T8, gene:84869 21 literature, APEX_matrix, Rickettsial homolog, mito protein domain, induction, coexpression+, MS/MS++ Matrix Metabolism > Lipid metabolism > Type II fatty acid synthesis all 14

SMDT1 single-pass membrane protein with aspartate rich tail 1 C22orf32, DDDD, EMRE, Q9H4I9, gene:91689 21 literature, targetP signal, mito protein domain+, induction, coexpression+, MS/MS MIM Signaling > Calcium homeostasis > Calcium cycle;
Small molecule transport > Calcium uniporter cerebrum, cerebellum, brainstem, spinalcord, kidney, adipose, smallintestine, largeintestine, stomach, placenta, testis

HAGH hydroxyacylglutathione hydrolase GLO2, GLX2, GLXII, HAGH1, Q16775, gene:3029 21 literature, APEX_matrix, targetP signal+, mito protein domain, induction, coexpression, MS/MS++ Matrix Metabolism > Detoxification > ROS and glutathione metabolism cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, adipose, smallintestine, largeintestine, stomach, placenta, testis

COX7A1 cytochrome c oxidase subunit 7A1 COX7A, COX7AH, COX7AM, P24310, gene:1346 21 literature, targetP signal, mito protein domain+, induction, coexpression, MS/MS+ MIM OXPHOS > Complex IV > CIV subunits;
OXPHOS > OXPHOS subunits cerebrum, brainstem, spinalcord, kidney, liver, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis

MTG1 mitochondrial ribosome associated GTPase 1 GTP, GTPBP7, Q9BT17, gene:92170 21 literature, APEX_matrix, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain, coexpression+, MS/MS Matrix Mitochondrial central dogma > Translation > Mitochondrial ribosome assembly largeintestine, testis

COX14 cytochrome c oxidase assembly factor COX14 C12orf62, PCAG1, Q96I36, gene:84987 21 literature, GFP, mito protein domain+, induction, coexpression++, MS/MS+ MIM Mitochondrial central dogma > Translation > Translation factors;
OXPHOS > Complex IV > CIV assembly factors;
OXPHOS > OXPHOS assembly factors cerebrum, cerebellum, brainstem, spinalcord, kidney, heart, smallintestine, testis

NDUFA12 NADH:ubiquinone oxidoreductase subunit A12 B17.2, DAP13, MC1DN23, Q9UI09, gene:55967 21 literature, APEX_matrix, Rickettsial homolog, coexpression++, MS/MS++ MIM OXPHOS > Complex I > CI subunits;
OXPHOS > OXPHOS subunits all 14

MRPS16 mitochondrial ribosomal protein S16 CGI-132, COXPD2, MRP-S16, RPMS16, Q9Y3D3, gene:51021 21 literature, APEX_matrix, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain, MS/MS+ Matrix Mitochondrial central dogma > Translation > Mitochondrial ribosome all 14

MTERF2 mitochondrial transcription termination factor 2 MTERFD3, mTERFL, Q49AM1, gene:80298 21 literature, APEX_matrix, targetP signal+, mito protein domain+, induction, coexpression, MS/MS Matrix Mitochondrial central dogma > mtDNA maintenance > mtDNA nucleoid cerebrum, cerebellum, brainstem, spinalcord, liver, heart, skeletalmuscle, adipose, largeintestine, testis

ETFRF1 electron transfer flavoprotein regulatory factor 1 LYRM5, Q6IPR1, gene:144363 21 mito protein domain+, induction, coexpression, MS/MS++ Matrix Metabolism > Electron carriers > Q-linked reactions, other cerebrum, brainstem, spinalcord, kidney, liver, heart, adipose, smallintestine, largeintestine, stomach, placenta

GPD2 glycerol-3-phosphate dehydrogenase 2 GDH2, GPDM, mGDH, mGPDH, P43304, gene:2820 21 literature, APEX_IMS, yeast mito homolog++, mito protein domain, coexpression, MS/MS++ MIM Metabolism > Carbohydrate metabolism > Glycerol phosphate shuttle;
Metabolism > Electron carriers > Q-linked reactions, other cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis

NDUFA10 NADH:ubiquinone oxidoreductase subunit A10 CI-42KD, CI-42k, MC1DN22, O95299, gene:4705 21 literature, targetP signal, mito protein domain, coexpression++, MS/MS++ MIM OXPHOS > Complex I > CI subunits;
OXPHOS > OXPHOS subunits all 14

NDUFC2 NADH:ubiquinone oxidoreductase subunit C2 B14.5b, CI-B14.5b, HLC-1, NADHDH2, O95298, gene:4718 21 literature, APEX_IMS, mito protein domain+, coexpression++, MS/MS++ MIM OXPHOS > Complex I > CI subunits;
OXPHOS > OXPHOS subunits all 14

SLIRP SRA stem-loop interacting RNA binding protein C14orf156, DC50, PD04872, Q9GZT3, gene:81892 21 literature, APEX_matrix, GFP, targetP signal+, mito protein domain, induction, coexpression, MS/MS++ Matrix Mitochondrial central dogma > mtRNA metabolism > mtRNA stability and decay;
Mitochondrial central dogma > Translation all 14

MT-ATP6 ATP synthase F0 subunit 6 ATPase6, MTATP6, P00846, gene:4508 21 literature, yeast mito homolog++, Rickettsial homolog, mito protein domain+, MS/MS+ MIM OXPHOS > Complex V > CV subunits;
OXPHOS > OXPHOS subunits all 14

MT-CO2 cytochrome c oxidase subunit II COII, MTCO2, P00403, gene:4513 21 literature, APEX_IMS, APEX_matrix, yeast mito homolog++, Rickettsial homolog, mito protein domain+, MS/MS+ MIM OXPHOS > Complex IV > CIV subunits;
OXPHOS > OXPHOS subunits all 14

TIMM21 translocase of inner mitochondrial membrane 21 C18orf55, HSPC154, TIM21, Q9BVV7, gene:29090 21 APEX_IMS, GFP, yeast mito homolog++, mito protein domain+, coexpression, MS/MS MIM Mitochondrial central dogma > Translation > Translation factors;
Protein import, sorting and homeostasis > Protein import and sorting > TIM23 presequence pathway;
OXPHOS > Complex IV > CIV assembly factors;
OXPHOS > OXPHOS assembly factors cerebrum, cerebellum, brainstem, spinalcord, liver, heart, skeletalmuscle, adipose, largeintestine, stomach, placenta, testis

NDUFA8 NADH:ubiquinone oxidoreductase subunit A8 CI-19KD, CI-PGIV, PGIV, P51970, gene:4702 21 literature, APEX_IMS, mito protein domain, induction, coexpression++, MS/MS++ MIM OXPHOS > Complex I > CI subunits;
OXPHOS > OXPHOS subunits all 14

GLS glutaminase AAD20, CASGID, EIEE71, GAC, GAM, GDPAG, GLS1, KGA, O94925, gene:2744 21 literature, APEX_matrix, targetP signal+, Rickettsial homolog, mito protein domain+, MS/MS++ MIM Metabolism > Amino acid metabolism > Glutamate metabolism cerebrum, cerebellum, brainstem, spinalcord, kidney, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis

NDUFB11 NADH:ubiquinone oxidoreductase subunit B11 CI-ESSS, ESSS, MC1DN30, NP17.3, Np15, P17.3, Q9NX14, gene:54539 20 literature, APEX_IMS, APEX_matrix, targetP signal+, coexpression++, MS/MS++ MIM OXPHOS > Complex I > CI subunits;
OXPHOS > OXPHOS subunits all 14

DHTKD1 dehydrogenase E1 and transketolase domain containing 1 AMOXAD, CMT2Q, Q96HY7, gene:55526 20 APEX_matrix, targetP signal, yeast mito homolog+, Rickettsial homolog, mito protein domain+, MS/MS Matrix Metabolism > Amino acid metabolism > Lysine metabolism cerebrum, kidney, liver, adipose, placenta, testis

FAHD2A fumarylacetoacetate hydrolase domain containing 2A CGI-105, Q96GK7, gene:51011 20 APEX_matrix, targetP signal, mito protein domain, induction, coexpression, MS/MS++ Matrix none all 14

HSD17B8 hydroxysteroid 17-beta dehydrogenase 8 D6S2245E, FABG, FABGL, H2-KE6, HKE6, KE6, RING2, SDR30C1, dJ1033B10.9, Q92506, gene:7923 20 APEX_matrix, targetP signal+, Rickettsial homolog, mito protein domain, coexpression, MS/MS++ Matrix Metabolism > Lipid metabolism > Type II fatty acid synthesis;
Metabolism > Lipid metabolism > Cholesterol, bile acid, steroid synthesis all 14

HINT2 histidine triad nucleotide binding protein 2 HIT-17, Q9BX68, gene:84681 20 APEX_matrix, targetP signal+, Rickettsial homolog, mito protein domain, coexpression, MS/MS++ Matrix Metabolism > Lipid metabolism > Cholesterol, bile acid, steroid synthesis all 14

MRPS5 mitochondrial ribosomal protein S5 MRP-S5, S5mt, P82675, gene:64969 20 literature, APEX_matrix, yeast mito homolog++, Rickettsial homolog, mito protein domain, induction, coexpression, MS/MS Matrix Mitochondrial central dogma > Translation > Mitochondrial ribosome cerebrum, kidney, liver, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis

C6orf136 chromosome 6 open reading frame 136 Q5SQH8, gene:221545 20 targetP signal, induction, coexpression++, MS/MS+ Matrix none all 14

SPRYD4 SPRY domain containing 4 Q8WW59, gene:283377 20 APEX_matrix, GFP, targetP signal+, mito protein domain, coexpression+, MS/MS++ Matrix none all 14

LIPT2 lipoyl(octanoyl) transferase 2 A6NK58, gene:387787 20 targetP signal, yeast mito homolog, Rickettsial homolog, mito protein domain, coexpression, MS/MS Matrix Metabolism > Lipid metabolism > Lipoate insertion cerebrum, cerebellum, brainstem, spinalcord, skeletalmuscle, adipose, largeintestine, placenta, testis

DECR1 2,4-dienoyl-CoA reductase 1 DECR, NADPH, SDR18C1, Q16698, gene:1666 20 literature, APEX_matrix, targetP signal, Rickettsial homolog, mito protein domain, coexpression+, MS/MS++ Matrix Metabolism > Lipid metabolism > Fatty acid oxidation all 14

MRPS26 mitochondrial ribosomal protein S26 C20orf193, GI008, MRP-S13, MRP-S26, MRPS13, NY-BR-87, RPMS13, dJ534B8.3, Q9BYN8, gene:64949 20 literature, APEX_matrix, targetP signal, mito protein domain+, induction, coexpression, MS/MS+ Matrix Mitochondrial central dogma > Translation > Mitochondrial ribosome all 14

SLC25A15 solute carrier family 25 member 15 D13S327, HHH, LNC-HC, ORC1, ORNT1, Q9Y619, gene:10166 20 literature, yeast mito homolog+, mito protein domain+, coexpression, MS/MS++ MIM Metabolism > Amino acid metabolism > Urea cycle;
Small molecule transport > SLC25A family spinalcord, kidney, liver, adipose, smallintestine, largeintestine, placenta

NDUFV3 NADH:ubiquinone oxidoreductase subunit V3 CI-10k, CI-9KD, P56181, gene:4731 20 literature, APEX_matrix, targetP signal, induction, coexpression++, MS/MS++ MIM OXPHOS > Complex I > CI subunits;
OXPHOS > OXPHOS subunits all 14

BPHL biphenyl hydrolase like BPH-RP, MCNAA, VACVASE, Q86WA6, gene:670 20 targetP signal+, Rickettsial homolog, mito protein domain, coexpression, MS/MS++ Matrix Metabolism > Detoxification > Xenobiotic metabolism all 14

STOML2 stomatin like 2 HSPC108, SLP-2, Q9UJZ1, gene:30968 20 literature, APEX_matrix, targetP signal+, Rickettsial homolog, mito protein domain, coexpression, MS/MS++ MIM Protein import, sorting and homeostasis > Protein homeostasis all 14

MSRB2 methionine sulfoxide reductase B2 CBS-1, CBS1, CGI-131, MSRB, PILB, Q9Y3D2, gene:22921 20 APEX_matrix, targetP signal, yeast mito homolog+, mito protein domain, coexpression, MS/MS++ Matrix Metabolism > Detoxification > ROS and glutathione metabolism;
Metabolism > Sulfur metabolism cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, heart, skeletalmuscle, adipose, largeintestine, stomach, placenta, testis

LACTB lactamase beta G24, MRPL56, P83111, gene:114294 20 literature, APEX_IMS, targetP signal+, Rickettsial homolog, mito protein domain, coexpression, MS/MS++ IMS Protein import, sorting and homeostasis > Protein homeostasis > Proteases;
Metabolism > Lipid metabolism > Phospholipid metabolism all 14

TOMM40 translocase of outer mitochondrial membrane 40 C19orf1, D19S1177E, PER-EC1, PEREC1, TOM40, O96008, gene:10452 20 literature, yeast mito homolog+, mito protein domain+, coexpression, MS/MS++ MOM Protein import, sorting and homeostasis > Protein import and sorting > TOM cerebrum, spinalcord, kidney, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis

SLC25A25 solute carrier family 25 member 25 MCSC, PCSCL, SCAMC-2, Q6KCM7, gene:114789 20 literature, yeast mito homolog+, mito protein domain+, coexpression, MS/MS++ MIM Metabolism > Nucleotide metabolism > Nucleotide import;
Signaling > Calcium homeostasis > EF hand proteins;
Small molecule transport > SLC25A family cerebrum, brainstem, spinalcord, liver, adipose, smallintestine, largeintestine, placenta

ADCK5 aarF domain containing kinase 5 Q3MIX3, gene:203054 20 targetP signal, yeast mito homolog+, Rickettsial homolog, mito protein domain, induction, coexpression, MS/MS unknown none cerebrum, largeintestine, testis

ACAD10 acyl-CoA dehydrogenase family member 10 Q6JQN1, gene:80724 20 APEX_matrix, targetP signal+, mito protein domain+, coexpression, MS/MS++ Matrix Metabolism > Lipid metabolism > Fatty acid oxidation cerebrum, spinalcord, kidney, liver, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis

VWA8 von Willebrand factor A domain containing 8 KIAA0564, P7BP2, A3KMH1, gene:23078 20 APEX_matrix, targetP signal, mito protein domain, coexpression+, MS/MS++ Matrix none all 14

CCDC90B coiled-coil domain containing 90B MDS011, MDS025, Q9GZT6, gene:60492 20 literature, APEX_matrix, targetP signal+, yeast mito homolog+, mito protein domain+, MS/MS MIM none all 14

DNAJC11 DnaJ heat shock protein family (Hsp40) member C11 dJ126A5.1, Q9NVH1, gene:55735 20 GFP, yeast mito homolog+, Rickettsial homolog, mito protein domain, coexpression, MS/MS++ MOM Protein import, sorting and homeostasis > Protein import and sorting;
Mitochondrial dynamics and surveillance > Intramitochondrial membrane interactions all 14

ALKBH7 alkB homolog 7 ABH7, SPATA11, UNQ6002, Q9BT30, gene:84266 20 APEX_matrix, targetP signal, mito protein domain, induction, coexpression++, MS/MS Matrix none cerebellum, brainstem, largeintestine, testis

GLDC glycine decarboxylase GCE, GCSP, HYGN1, P23378, gene:2731 20 literature, APEX_matrix, targetP signal, yeast mito homolog, mito protein domain+, coexpression, MS/MS++ Matrix Metabolism > Amino acid metabolism > Glycine metabolism;
Metabolism > Amino acid metabolism > Glycine cleavage system cerebrum, cerebellum, kidney, liver, adipose, placenta

MPC1L mitochondrial pyruvate carrier 1 like SLC54A3, P0DKB6, gene:347411 20 targetP signal, yeast mito homolog+, mito protein domain+, MS/MS++ MIM Metabolism > Carbohydrate metabolism > Gluconeogenesis;
Metabolism > Carbohydrate metabolism > Pyruvate metabolism;
Metabolism > Carbohydrate metabolism > TCA-associated;
Small molecule transport testis

THEM4 thioesterase superfamily member 4 CTMP, Q5T1C6, gene:117145 20 literature, APEX_matrix, targetP signal+, yeast mito homolog, mito protein domain, induction, MS/MS++ MIM Metabolism > Lipid metabolism cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, adipose, smallintestine, largeintestine, placenta, testis

SSBP1 single stranded DNA binding protein 1 Mt-SSB, SOSS-B1, SSBP, mtSSB, Q04837, gene:6742 19 literature, APEX_matrix, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain, MS/MS Matrix Mitochondrial central dogma > mtDNA maintenance > mtDNA replication;
Mitochondrial central dogma > mtDNA maintenance > mtDNA nucleoid all 14

MRPL27 mitochondrial ribosomal protein L27 L27mt, Q9P0M9, gene:51264 19 literature, APEX_matrix, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain, MS/MS Matrix Mitochondrial central dogma > Translation > Mitochondrial ribosome all 14

HSCB HscB mitochondrial iron-sulfur cluster cochaperone DNAJC20, HSC20, JAC1, Q8IWL3, gene:150274 19 APEX_matrix, GFP, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain, MS/MS Matrix Metabolism > Metals and cofactors > Fe-S cluster biosynthesis cerebrum, cerebellum, brainstem, spinalcord, skeletalmuscle, adipose, largeintestine, stomach, placenta, testis

MRPS10 mitochondrial ribosomal protein S10 MRP-S10, PNAS-122, P82664, gene:55173 19 literature, APEX_matrix, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain, MS/MS++ Matrix Mitochondrial central dogma > Translation > Mitochondrial ribosome all 14

AK4 adenylate kinase 4 AK 4, AK3, AK3L1, AK3L2, P27144, gene:205 19 APEX_matrix, yeast mito homolog++, Rickettsial homolog, mito protein domain, MS/MS++ Matrix Metabolism > Nucleotide metabolism > Nucleotide synthesis and processing all 14

ATAD3A ATPase family AAA domain containing 3A HAYOS, PHRINL, Q9NVI7, gene:55210 19 APEX_IMS, APEX_matrix, GFP, Rickettsial homolog, mito protein domain+, coexpression, MS/MS++ MIM Mitochondrial central dogma > mtDNA maintenance > mtDNA nucleoid;
Mitochondrial dynamics and surveillance > Organelle contact sites;
Mitochondrial dynamics and surveillance > Intramitochondrial membrane interactions all 14

MRPL37 mitochondrial ribosomal protein L37 L2mt, L37mt, MRP-L2, MRP-L37, MRPL2, RPML2, Q9BZE1, gene:51253 19 literature, APEX_matrix, targetP signal, mito protein domain, induction, coexpression+, MS/MS++ Matrix Mitochondrial central dogma > Translation > Mitochondrial ribosome kidney, liver, heart, adipose, smallintestine, largeintestine, stomach, placenta, testis

PTGES2 prostaglandin E synthase 2 C9orf15, GBF-1, GBF1, PGES2, mPGES-2, Q9H7Z7, gene:80142 19 literature, APEX_IMS, APEX_matrix, targetP signal, mito protein domain, induction, coexpression+, MS/MS++ MIM Metabolism > Lipid metabolism > Eicosanoid metabolism all 14

TXNRD2 thioredoxin reductase 2 GCCD5, SELZ, TR, TR-BETA, TR3, TRXR2, Q9NNW7, gene:10587 19 literature, APEX_matrix, targetP signal+, Rickettsial homolog, mito protein domain, coexpression, MS/MS++ Matrix Metabolism > Detoxification > ROS and glutathione metabolism;
Metabolism > Detoxification > Selenoproteins cerebrum, spinalcord, kidney, liver, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis

ACSM5 acyl-CoA synthetase medium chain family member 5 Q6NUN0, gene:54988 19 literature, targetP signal+, Rickettsial homolog, mito protein domain, coexpression, MS/MS++ Matrix Metabolism > Lipid metabolism > Fatty acid oxidation;
Metabolism > Detoxification > Xenobiotic metabolism kidney, liver, adipose, smallintestine, largeintestine

CISD1 CDGSH iron sulfur domain 1 C10orf70, MDS029, ZCD1, mitoNEET, Q9NZ45, gene:55847 19 literature, mito protein domain, induction, coexpression++, MS/MS++ MOM Metabolism > Metals and cofactors > Fe-S cluster biosynthesis;
Metabolism > Metals and cofactors > Fe-S-containing proteins all 14

MRPL41 mitochondrial ribosomal protein L41 BMRP, MRP-L27, MRPL27, PIG3, RPML27, Q8IXM3, gene:64975 19 literature, APEX_matrix, yeast mito homolog++, mito protein domain, induction, MS/MS++ Matrix Mitochondrial central dogma > Translation > Mitochondrial ribosome all 14

MTARC2 mitochondrial amidoxime reducing component 2 MARC2, MOSC2, Q969Z3, gene:54996 19 literature, targetP signal, mito protein domain+, induction, coexpression, MS/MS++ MOM Metabolism > Metals and cofactors > Molybdenum cofactor synthesis and proteins;
Metabolism > Detoxification > Xenobiotic metabolism;
Metabolism > Detoxification > Amidoxime reducing complex all 14

MRPS6 mitochondrial ribosomal protein S6 C21orf101, MRP-S6, RPMS6, S6mt, P82932, gene:64968 19 literature, APEX_matrix, yeast mito homolog++, Rickettsial homolog, mito protein domain, MS/MS++ Matrix Mitochondrial central dogma > Translation > Mitochondrial ribosome all 14

MRPL23 mitochondrial ribosomal protein L23 L23MRP, RPL23, RPL23L, Q16540, gene:6150 19 literature, APEX_matrix, targetP signal, yeast mito homolog++, mito protein domain, MS/MS++ Matrix Mitochondrial central dogma > Translation > Mitochondrial ribosome all 14

SQOR sulfide quinone oxidoreductase CGI-44, PRO1975, SQR, SQRDL, Q9Y6N5, gene:58472 19 literature, targetP signal, yeast mito homolog++, mito protein domain, MS/MS++ MIM Metabolism > Electron carriers > Q-linked reactions, other;
Metabolism > Sulfur metabolism spinalcord, kidney, liver, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis

SCO1 synthesis of cytochrome C oxidase 1 SCOD1, O75880, gene:6341 19 literature, APEX_IMS, APEX_matrix, targetP signal, yeast mito homolog+, Rickettsial homolog, mito protein domain, MS/MS++ MIM OXPHOS > Complex IV > CIV assembly factors;
Metabolism > Metals and cofactors > Copper metabolism;
OXPHOS > OXPHOS assembly factors cerebrum, cerebellum, brainstem, spinalcord, liver, heart, adipose, smallintestine, largeintestine, stomach, placenta, testis

GCAT glycine C-acetyltransferase KBL, O75600, gene:23464 19 literature, targetP signal, yeast mito homolog+, Rickettsial homolog, mito protein domain, MS/MS++ unknown Metabolism > Amino acid metabolism > Glycine metabolism brainstem, spinalcord, kidney, liver, adipose, smallintestine, largeintestine, stomach, placenta, testis

MTHFD1L methylenetetrahydrofolate dehydrogenase (NADP+ dependent) 1 like FTHFSDC1, MTC1THFS, dJ292B18.2, Q6UB35, gene:25902 19 literature, APEX_matrix, targetP signal, yeast mito homolog+, Rickettsial homolog, mito protein domain, MS/MS++ Matrix Metabolism > Vitamin metabolism > Folate and 1-C metabolism cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, adipose, smallintestine, largeintestine, stomach, placenta, testis

ECI2 enoyl-CoA delta isomerase 2 ACBD2, DRS-1, DRS1, HCA88, PECI, dJ1013A10.3, O75521, gene:10455 19 literature, APEX_matrix, targetP signal+, mito protein domain, coexpression+, MS/MS++ Matrix Metabolism > Lipid metabolism > Fatty acid oxidation all 14

UQCRB ubiquinol-cytochrome c reductase binding protein MC3DN3, QCR7, QP-C, QPC, UQBC, UQBP, UQCR6, UQPC, P14927, gene:7381 19 literature, APEX_matrix, yeast mito homolog++, mito protein domain+, MS/MS++ MIM OXPHOS > Complex III > CIII subunits;
OXPHOS > OXPHOS subunits all 14

ATP5PD ATP synthase peripheral stalk subunit d APT5H, ATP5H, ATPQ, O75947, gene:10476 19 literature, APEX_matrix, yeast mito homolog++, mito protein domain+, MS/MS++ MIM OXPHOS > Complex V > CV subunits;
OXPHOS > OXPHOS subunits all 14

ATP5MG ATP synthase membrane subunit g ATP5JG, ATP5L, O75964, gene:10632 19 literature, APEX_matrix, yeast mito homolog++, mito protein domain+, MS/MS++ MIM OXPHOS > Complex V > CV subunits;
OXPHOS > OXPHOS subunits;
Mitochondrial dynamics and surveillance > Cristae formation all 14

CCDC58 coiled-coil domain containing 58 Q4VC31, gene:131076 19 APEX_IMS, yeast mito homolog++, mito protein domain+, MS/MS++ IMS none all 14

MCCC2 methylcrotonoyl-CoA carboxylase 2 MCCB, Q9HCC0, gene:64087 19 literature, targetP signal, Rickettsial homolog, mito protein domain, coexpression+, MS/MS++ Matrix Metabolism > Amino acid metabolism > BCAA metabolism all 14

MCEE methylmalonyl-CoA epimerase GLOD2, MCE, Q96PE7, gene:84693 19 targetP signal, induction, coexpression++, MS/MS++ Matrix Metabolism > Carbohydrate metabolism > Propanoate metabolism;
Metabolism > Lipid metabolism > Fatty acid oxidation all 14

MOCS1 molybdenum cofactor synthesis 1 MIG11, MOCOD, MOCS1A, MOCS1B, Q9NZB8, gene:4337 19 APEX_matrix, targetP signal+, Rickettsial homolog, mito protein domain+, MS/MS+ Matrix Metabolism > Metals and cofactors > Molybdenum cofactor synthesis and proteins cerebrum, liver, adipose, smallintestine, largeintestine, stomach, placenta

MMUT methylmalonyl-CoA mutase MCM, MUT, P22033, gene:4594 19 literature, APEX_matrix, targetP signal, mito protein domain+, coexpression, MS/MS++ Matrix Metabolism > Carbohydrate metabolism > Propanoate metabolism;
Metabolism > Lipid metabolism > Fatty acid oxidation;
Metabolism > Vitamin metabolism > Vitamin B12 metabolism all 14

SLC25A51 solute carrier family 25 member 51 CG7943, MCART1, Q9H1U9, gene:92014 19 GFP, yeast mito homolog+, mito protein domain+, coexpression, MS/MS++ MIM Metabolism > Metals and cofactors > NAD biosynthesis and metabolism;
Small molecule transport > SLC25A family cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, heart, adipose, smallintestine, largeintestine, stomach, placenta, testis

SPG7 SPG7 matrix AAA peptidase subunit, paraplegin CAR, CMAR, PGN, SPG5C, Q9UQ90, gene:6687 19 literature, targetP signal, Rickettsial homolog, mito protein domain+, coexpression+, MS/MS MIM Protein import, sorting and homeostasis > Protein homeostasis > Proteases cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis

ATP5MC3 ATP synthase membrane subunit c locus 3 ATP5G3, P3, P48201, gene:518 19 literature, targetP signal, yeast mito homolog+, Rickettsial homolog, mito protein domain, coexpression++ MIM OXPHOS > Complex V > CV subunits;
OXPHOS > OXPHOS subunits none

ATAD1 ATPase family AAA domain containing 1 AFDC1, FNP001, HKPX4, Msp1, THORASE, Q8NBU5, gene:84896 19 yeast mito homolog++, Rickettsial homolog, mito protein domain, coexpression, MS/MS++ MOM Protein import, sorting and homeostasis > Protein homeostasis > Proteases cerebrum, cerebellum, brainstem, spinalcord, kidney, heart, skeletalmuscle, adipose, largeintestine, stomach, placenta, testis

OXNAD1 oxidoreductase NAD binding domain containing 1 Q96HP4, gene:92106 19 APEX_matrix, GFP, mito protein domain, coexpression++, MS/MS++ Matrix none cerebrum, spinalcord, liver, heart, adipose, smallintestine, largeintestine, stomach, placenta, testis

MTG2 mitochondrial ribosome associated GTPase 2 GTPBP5, ObgH1, dJ1005F21.2, Q9H4K7, gene:26164 19 literature, APEX_matrix, targetP signal+, yeast mito homolog+, Rickettsial homolog, mito protein domain, coexpression Matrix Mitochondrial central dogma > Translation > Mitochondrial ribosome assembly largeintestine, placenta

DARS2 aspartyl-tRNA synthetase 2, mitochondrial ASPRS, LBSL, MT-ASPRS, mtAspRS, Q6PI48, gene:55157 19 APEX_matrix, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain, coexpression, MS/MS Matrix Mitochondrial central dogma > Translation > mt-tRNA synthetases cerebrum, cerebellum, brainstem, spinalcord, kidney, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis

MRPL51 mitochondrial ribosomal protein L51 CDA09, HSPC241, MRP64, bMRP64, Q4U2R6, gene:51258 19 literature, APEX_matrix, targetP signal, mito protein domain+, coexpression, MS/MS++ Matrix Mitochondrial central dogma > Translation > Mitochondrial ribosome cerebrum, cerebellum, spinalcord, kidney, liver, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis

PC pyruvate carboxylase PCB, P11498, gene:5091 19 literature, targetP signal, Rickettsial homolog, mito protein domain, coexpression, MS/MS++ Matrix Metabolism > Carbohydrate metabolism > Gluconeogenesis;
Metabolism > Carbohydrate metabolism > TCA-associated;
Metabolism > Vitamin metabolism > Biotin utilizing proteins all 14

CLYBL citramalyl-CoA lyase CLB, Q8N0X4, gene:171425 19 APEX_matrix, targetP signal+, induction, coexpression+, MS/MS++ Matrix Metabolism > Carbohydrate metabolism > Itaconate metabolism all 14

PNPLA8 patatin like phospholipase domain containing 8 IPLA2-2, IPLA2G, MMLA, PNPLA-gamma, iPLA2gamma, Q9NP80, gene:50640 19 APEX_matrix, Rickettsial homolog, induction, coexpression++, MS/MS+ MIM Metabolism > Lipid metabolism > Cardiolipin synthesis;
Metabolism > Lipid metabolism > Phospholipid metabolism cerebrum, cerebellum, brainstem, spinalcord, liver, adipose, largeintestine, testis

MLYCD malonyl-CoA decarboxylase MCD, O95822, gene:23417 19 literature, APEX_matrix, targetP signal+, mito protein domain, coexpression++, MS/MS Matrix Metabolism > Lipid metabolism cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta

PYURF PIGY upstream reading frame PREY, Q96I23, gene:100996939 19 targetP signal+, induction, coexpression++, MS/MS unknown none cerebrum, cerebellum, brainstem, spinalcord, liver, heart, skeletalmuscle, adipose, largeintestine, stomach, placenta

TIMM17B translocase of inner mitochondrial membrane 17B DXS9822, JM3, TIM17B, O60830, gene:10245 19 literature, APEX_IMS, yeast mito homolog+, mito protein domain+, coexpression, MS/MS+ MIM Protein import, sorting and homeostasis > Protein import and sorting > TIM23 presequence pathway all 14

MRPL32 mitochondrial ribosomal protein L32 HSPC283, L32mt, MRP-L32, bMRP-59b, Q9BYC8, gene:64983 19 literature, APEX_matrix, yeast mito homolog++, Rickettsial homolog, mito protein domain, coexpression, MS/MS Matrix Mitochondrial central dogma > Translation > Mitochondrial ribosome cerebrum, brainstem, spinalcord, liver, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis

SLC25A26 solute carrier family 25 member 26 COXPD28, SAMC, Q70HW3, gene:115286 19 literature, yeast mito homolog++, mito protein domain+, coexpression, MS/MS MIM Small molecule transport > SLC25A family cerebrum, cerebellum, adipose, largeintestine, placenta, testis

PRODH2 proline dehydrogenase 2 HSPOX1, HYPDH, Q9UF12, gene:58510 19 targetP signal, yeast mito homolog+, mito protein domain+, MS/MS+ MIM Metabolism > Amino acid metabolism > Glyoxylate metabolism;
Metabolism > Electron carriers > Q-linked reactions, other kidney, liver

NIT2 nitrilase family member 2 HEL-S-8a, Q9NQR4, gene:56954 19 Rickettsial homolog, mito protein domain, coexpression, MS/MS++ unknown Metabolism > Detoxification cerebellum, spinalcord, kidney, liver, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis

FDX2 ferredoxin 2 FDX1L, MEOAL, Q6P4F2, gene:112812 18 APEX_matrix, targetP signal, yeast mito homolog+, Rickettsial homolog, mito protein domain, coexpression, MS/MS Matrix Metabolism > Metals and cofactors > Heme synthesis and processing;
Metabolism > Metals and cofactors > Fe-S cluster biosynthesis;
Metabolism > Metals and cofactors > Fe-S-containing proteins cerebrum, cerebellum, brainstem, spinalcord, liver, skeletalmuscle, adipose, largeintestine, stomach, placenta, testis

ME2 malic enzyme 2 ODS1, P23368, gene:4200 18 literature, APEX_matrix, targetP signal, yeast mito homolog+, Rickettsial homolog, mito protein domain, MS/MS++ Matrix Metabolism > Carbohydrate metabolism > TCA-associated cerebrum, cerebellum, brainstem, spinalcord, kidney, heart, adipose, smallintestine, largeintestine, stomach, placenta, testis

TUFM Tu translation elongation factor, mitochondrial COXPD4, EF-TuMT, EFTU, P43, P49411, gene:7284 18 literature, APEX_matrix, targetP signal, yeast mito homolog+, Rickettsial homolog, mito protein domain, MS/MS++ Matrix Mitochondrial central dogma > Translation > Translation factors all 14

COX6A2 cytochrome c oxidase subunit 6A2 COX6AH, COXVIAH, Q02221, gene:1339 18 literature, targetP signal, yeast mito homolog+, mito protein domain+, induction, coexpression MIM OXPHOS > Complex IV > CIV subunits;
OXPHOS > OXPHOS subunits heart, skeletalmuscle

SLC30A9 solute carrier family 30 member 9 BILAPES, C4orf1, GAC63, HUEL, ZNT9, Q6PML9, gene:10463 18 APEX_matrix, targetP signal+, yeast mito homolog++, mito protein domain, induction, MS/MS MIM Small molecule transport cerebrum, cerebellum, brainstem, spinalcord, adipose, largeintestine, stomach, placenta, testis

TAMM41 TAM41 mitochondrial translocator assembly and maintenance homolog C3orf31, RAM41, TAM41, Q96BW9, gene:132001 18 GFP, yeast mito homolog++, mito protein domain+, MS/MS++ MIM Metabolism > Lipid metabolism > Cardiolipin synthesis;
Metabolism > Lipid metabolism > Phospholipid metabolism cerebrum, cerebellum, brainstem, spinalcord, heart, skeletalmuscle, adipose, largeintestine, stomach, placenta, testis

TIMM23 translocase of inner mitochondrial membrane 23 TIM23, O14925, gene:100287932 18 literature, APEX_IMS, yeast mito homolog++, mito protein domain+, MS/MS++ MIM Protein import, sorting and homeostasis > Protein import and sorting > TIM23 presequence pathway all 14

RMND1 required for meiotic nuclear division 1 homolog C6orf96, COXPD11, RMD1, bA351K16, bA351K16.3, Q9NWS8, gene:55005 18 literature, GFP, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain, MS/MS MIM Mitochondrial central dogma > mtRNA metabolism > mtRNA granules;
Mitochondrial central dogma > Translation cerebrum, cerebellum, brainstem, spinalcord, adipose, smallintestine, largeintestine, stomach, placenta, testis

MFN2 mitofusin 2 CMT2A, CMT2A2, CMT2A2A, CMT2A2B, CPRP1, HMSN6A, HSG, MARF, O95140, gene:9927 18 literature, mito protein domain+, induction, coexpression, MS/MS+ MOM Mitochondrial dynamics and surveillance > Fusion;
Mitochondrial dynamics and surveillance > Organelle contact sites cerebrum, cerebellum, brainstem, spinalcord, liver, adipose, largeintestine, stomach, placenta, testis

DNLZ DNL-type zinc finger C9orf151, HEP, HEP1, TIMM15, ZIM17, bA413M3.2, Q5SXM8, gene:728489 18 APEX_matrix, targetP signal, yeast mito homolog++, mito protein domain+, MS/MS Matrix Protein import, sorting and homeostasis > Protein homeostasis > Chaperones cerebrum, cerebellum, brainstem, heart, adipose, smallintestine, largeintestine, stomach, placenta

CHCHD4 coiled-coil-helix-coiled-coil-helix domain containing 4 MIA40, TIMM40, Q8N4Q1, gene:131474 18 literature, APEX_IMS, yeast mito homolog++, mito protein domain, induction, coexpression, MS/MS+ IMS Protein import, sorting and homeostasis > Protein import and sorting > MIA40 cerebrum, cerebellum, brainstem, spinalcord, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis

NDUFAB1 NADH:ubiquinone oxidoreductase subunit AB1 ACP, ACP1, FASN2A, SDAP, O14561, gene:4706 18 literature, APEX_matrix, targetP signal+, yeast mito homolog, Rickettsial homolog, mito protein domain, MS/MS+ MIM OXPHOS > Complex I > CI subunits;
Metabolism > Lipid metabolism > Type II fatty acid synthesis;
Metabolism > Metals and cofactors > Fe-S cluster biosynthesis;
OXPHOS > OXPHOS subunits all 14

ACSM1 acyl-CoA synthetase medium chain family member 1 BUCS1, MACS1, Q08AH1, gene:116285 18 literature, targetP signal, Rickettsial homolog, mito protein domain, coexpression, MS/MS++ Matrix Metabolism > Lipid metabolism > Fatty acid oxidation spinalcord, kidney, liver, smallintestine

COA5 cytochrome c oxidase assembly factor 5 6330578E17Rik, C2orf64, CEMCOX3, Pet191, Q86WW8, gene:493753 18 literature, APEX_IMS, yeast mito homolog++, mito protein domain+, induction, MS/MS IMS OXPHOS > Complex IV > CIV assembly factors;
OXPHOS > OXPHOS assembly factors adipose, placenta, testis

COQ2 coenzyme Q2, polyprenyltransferase CL640, COQ10D1, MSA1, PHB:PPT, Q96H96, gene:27235 18 literature, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain, coexpression+ MIM Metabolism > Metals and cofactors > Coenzyme Q metabolism none

PDSS2 decaprenyl diphosphate synthase subunit 2 C6orf210, COQ10D3, COQ1B, DLP1, bA59I9.3, hDLP1, Q86YH6, gene:57107 18 literature, APEX_matrix, targetP signal, yeast mito homolog+, Rickettsial homolog, mito protein domain, coexpression+ MIM Metabolism > Metals and cofactors > Coenzyme Q metabolism smallintestine, largeintestine

EHHADH enoyl-CoA hydratase and 3-hydroxyacyl CoA dehydrogenase ECHD, FRTS3, L-PBE, LBFP, LBP, MFE1, PBFE, Q08426, gene:1962 18 yeast mito homolog+, Rickettsial homolog, mito protein domain, coexpression, MS/MS+ unknown Metabolism > Lipid metabolism spinalcord, kidney, liver, adipose, smallintestine, largeintestine, placenta

NDUFS3 NADH:ubiquinone oxidoreductase core subunit S3 CI-30, MC1DN8, O75489, gene:4722 18 literature, APEX_matrix, targetP signal+, Rickettsial homolog, mito protein domain, MS/MS++ MIM OXPHOS > Complex I > CI subunits;
OXPHOS > OXPHOS subunits all 14

ABCB10 ATP binding cassette subfamily B member 10 EST20237, M-ABC2, MTABC2, Q9NRK6, gene:23456 18 literature, APEX_matrix, yeast mito homolog+, Rickettsial homolog, mito protein domain, induction, coexpression, MS/MS+ MIM Metabolism > Metals and cofactors > Heme synthesis and processing;
Small molecule transport > ABC transporters cerebellum, spinalcord, liver, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis

CKMT2 creatine kinase, mitochondrial 2 SMTCK, P17540, gene:1160 18 literature, targetP signal, mito protein domain, induction, coexpression, MS/MS++ MIM Metabolism > Nucleotide metabolism > Creatine metabolism cerebrum, cerebellum, brainstem, spinalcord, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta

MRPS25 mitochondrial ribosomal protein S25 MRP-S25, RPMS25, P82663, gene:64432 18 literature, APEX_matrix, targetP signal, mito protein domain, induction, coexpression, MS/MS++ Matrix Mitochondrial central dogma > Translation > Mitochondrial ribosome all 14

ACAD9 acyl-CoA dehydrogenase family member 9 MC1DN20, NPD002, Q9H845, gene:28976 18 literature, APEX_matrix, targetP signal+, mito protein domain+, MS/MS++ MIM OXPHOS > Complex I > CI assembly factors;
OXPHOS > OXPHOS assembly factors all 14

COX16 cytochrome c oxidase assembly factor COX16 C14orf112, HSPC203, hCOX16, Q9P0S2, gene:51241 18 APEX_IMS, GFP, yeast mito homolog++, mito protein domain+, MS/MS+ MIM OXPHOS > Complex IV > CIV assembly factors;
OXPHOS > OXPHOS assembly factors kidney, liver, adipose, largeintestine, placenta, testis

FAM210A family with sequence similarity 210 member A C18orf19, HsT2329, Q96ND0, gene:125228 18 GFP, targetP signal, induction, coexpression++, MS/MS MIM none all 14

ACOT13 acyl-CoA thioesterase 13 HT012, PNAS-27, THEM2, Q9NPJ3, gene:55856 18 mito protein domain, induction, coexpression, MS/MS++ Matrix Metabolism > Lipid metabolism all 14

CPT1A carnitine palmitoyltransferase 1A CPT1, CPT1-L, L-CPT1, P50416, gene:1374 18 literature, yeast mito homolog+, mito protein domain, induction, coexpression, MS/MS++ MOM Metabolism > Lipid metabolism > Fatty acid oxidation;
Metabolism > Metals and cofactors > Carnitine synthesis and transport;
Metabolism > Metals and cofactors > Carnitine shuttle all 14

DHRS4 dehydrogenase/reductase 4 CR, NRDR, PHCR, PSCD, SCAD-SRL, SDR-SRL, SDR25C1, SDR25C2, Q9BTZ2, gene:10901 18 targetP signal, Rickettsial homolog, mito protein domain, coexpression, MS/MS++ Matrix Metabolism > Vitamin metabolism > Vitamin A metabolism all 14

PRELID2 PRELI domain containing 2 Q8N945, gene:153768 18 yeast mito homolog+, mito protein domain+, induction, coexpression, MS/MS IMS none largeintestine, stomach

CARS2 cysteinyl-tRNA synthetase 2, mitochondrial COXPD27, cysRS, Q9HA77, gene:79587 18 APEX_matrix, targetP signal, Rickettsial homolog, mito protein domain, coexpression++, MS/MS Matrix Mitochondrial central dogma > Translation > mt-tRNA synthetases cerebrum, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis

NIPSNAP2 nipsnap homolog 2 GBAS, O75323, gene:2631 18 literature, APEX_matrix, targetP signal+, induction, coexpression, MS/MS++ Matrix Mitochondrial dynamics and surveillance > Mitophagy;
Mitochondrial dynamics and surveillance > Autophagy all 14

NDUFB7 NADH:ubiquinone oxidoreductase subunit B7 B18, CI-B18, P17568, gene:4713 18 literature, APEX_IMS, APEX_matrix, induction, coexpression++, MS/MS++ MIM OXPHOS > Complex I > CI subunits;
OXPHOS > OXPHOS subunits all 14

MICOS13 mitochondrial contact site and cristae organizing system subunit 13 C19orf70, MIC13, P117, QIL1, Q5XKP0, gene:125988 18 induction, coexpression++, MS/MS++ MIM Mitochondrial dynamics and surveillance > Cristae formation > MICOS complex all 14

GLRX2 glutaredoxin 2 CGI-133, GRX2, Q9NS18, gene:51022 17 literature, targetP signal+, yeast mito homolog, Rickettsial homolog, mito protein domain, coexpression, MS/MS Matrix Metabolism > Metals and cofactors > Fe-S-containing proteins;
Metabolism > Vitamin metabolism > Vitamin C metabolism;
Metabolism > Detoxification > ROS and glutathione metabolism cerebrum, cerebellum, brainstem, spinalcord, skeletalmuscle, largeintestine, placenta, testis

MRPS30 mitochondrial ribosomal protein S30 MRP-S30, PAP, PDCD9, S30mt, Q9NP92, gene:10884 17 literature, APEX_matrix, targetP signal, mito protein domain, induction, coexpression, MS/MS++ Matrix Mitochondrial central dogma > Translation > Mitochondrial ribosome cerebrum, cerebellum, spinalcord, kidney, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis

SCO2 synthesis of cytochrome C oxidase 2 CEMCOX1, ECGF1, Gliostatin, MYP6, PD-ECGF, SCO1L, TP, TYMP, TdRPase, O43819, gene:9997 17 literature, APEX_IMS, targetP signal+, yeast mito homolog+, Rickettsial homolog, mito protein domain, MS/MS MIM OXPHOS > Complex IV > CIV assembly factors;
Metabolism > Metals and cofactors > Copper metabolism;
OXPHOS > OXPHOS assembly factors all 14

HSD17B10 hydroxysteroid 17-beta dehydrogenase 10 17b-HSD10, ABAD, CAMR, DUPXp11.22, ERAB, HADH2, HCD2, HSD10MD, MHBD, MRPP2, MRX17, MRX31, MRXS10, SCHAD, SDR5C1, Q99714, gene:3028 17 literature, APEX_matrix, Rickettsial homolog, mito protein domain, induction, coexpression, MS/MS++ Matrix 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 > BCAA metabolism all 14

NARS2 asparaginyl-tRNA synthetase 2, mitochondrial DFNB94, SLM5, asnRS, Q96I59, gene:79731 17 GFP, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain, coexpression Matrix Mitochondrial central dogma > Translation > mt-tRNA synthetases spinalcord, adipose, largeintestine, stomach, placenta

RPUSD3 RNA pseudouridine synthase D3 Q6P087, gene:285367 17 APEX_matrix, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain, coexpression Matrix Mitochondrial central dogma > mtRNA metabolism > mtRNA granules;
Mitochondrial central dogma > mtRNA metabolism > mt-rRNA modifications largeintestine

SUOX sulfite oxidase P51687, gene:6821 17 APEX_IMS, targetP signal, mito protein domain+, coexpression, MS/MS++ IMS Metabolism > Metals and cofactors > Molybdenum cofactor synthesis and proteins;
Metabolism > Sulfur metabolism cerebrum, brainstem, spinalcord, kidney, liver, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis

SARDH sarcosine dehydrogenase BPR-2, DMGDHL1, SAR, SARD, SDH, Q9UL12, gene:1757 17 literature, targetP signal+, mito protein domain, coexpression, MS/MS++ Matrix Metabolism > Amino acid metabolism > Glycine metabolism;
Metabolism > Vitamin metabolism > Choline and betaine metabolism;
Metabolism > Vitamin metabolism > Folate and 1-C metabolism cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, adipose, smallintestine, largeintestine, stomach, placenta, testis

RIDA reactive intermediate imine deaminase A homolog HRSP12, P14.5, PSP, UK114, hp14.5, P52758, gene:10247 17 APEX_matrix, yeast mito homolog++, mito protein domain, coexpression, MS/MS+ Matrix none cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, heart, adipose, smallintestine, largeintestine, stomach, placenta, testis

COX8A cytochrome c oxidase subunit 8A COX, COX8, COX8-2, COX8L, VIII, VIII-L, P10176, gene:1351 17 literature, targetP signal, mito protein domain+, coexpression+, MS/MS MIM OXPHOS > Complex IV > CIV subunits;
OXPHOS > OXPHOS subunits stomach, placenta, testis

SPR sepiapterin reductase SDR38C1, P35270, gene:6697 17 Rickettsial homolog, mito protein domain, induction, coexpression, MS/MS++ unknown Metabolism > Metals and cofactors > Tetrahydrobiopterin synthesis cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, heart, skeletalmuscle, adipose, largeintestine, stomach

MPV17 mitochondrial inner membrane protein MPV17 CMT2EE, MTDPS6, SYM1, P39210, gene:4358 17 literature, yeast mito homolog+, mito protein domain, coexpression, MS/MS++ MIM Small molecule transport all 14

ZADH2 zinc binding alcohol dehydrogenase domain containing 2 PRG-3, Q8N4Q0, gene:284273 17 mito protein domain, induction, coexpression+, MS/MS++ unknown Metabolism > Lipid metabolism > Eicosanoid metabolism all 14

MRPL18 mitochondrial ribosomal protein L18 HSPC071, L18mt, MRP-L18, Q9H0U6, gene:29074 17 literature, APEX_matrix, targetP signal+, Rickettsial homolog, mito protein domain, coexpression, MS/MS Matrix Mitochondrial central dogma > Translation > Mitochondrial ribosome all 14

ALDH1B1 aldehyde dehydrogenase 1 family member B1 ALDH5, ALDHX, P30837, gene:219 17 literature, APEX_matrix, targetP signal, yeast mito homolog+, mito protein domain, MS/MS++ Matrix Metabolism > Detoxification > Xenobiotic metabolism all 14

CHCHD1 coiled-coil-helix-coiled-coil-helix domain containing 1 C10orf34, C2360, MRP-S37, Q96BP2, gene:118487 17 GFP, targetP signal, yeast mito homolog+, mito protein domain, coexpression+, MS/MS MIM Mitochondrial central dogma > Translation > Mitochondrial ribosome cerebrum, cerebellum, spinalcord, kidney, liver, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis

TIMM8A translocase of inner mitochondrial membrane 8A DDP, DDP1, DFN1, MTS, TIM8, O60220, gene:1678 17 literature, yeast mito homolog+, mito protein domain+, MS/MS++ MIM Protein import, sorting and homeostasis > Protein homeostasis > Chaperones;
Protein import, sorting and homeostasis > Protein import and sorting all 14

VDAC3 voltage dependent anion channel 3 HD-VDAC3, VDAC-3, Q9Y277, gene:7419 17 literature, yeast mito homolog+, mito protein domain+, MS/MS++ MOM Signaling > Calcium homeostasis > Mitochondrial permeability transition pore;
Small molecule transport all 14

SLC25A24 solute carrier family 25 member 24 APC1, SCAMC-1, SCAMC1, Q6NUK1, gene:29957 17 literature, APEX_IMS, yeast mito homolog+, mito protein domain+, MS/MS++ MIM Metabolism > Nucleotide metabolism > Nucleotide import;
Signaling > Calcium homeostasis > EF hand proteins;
Small molecule transport > SLC25A family adipose, smallintestine, largeintestine, stomach, placenta, testis

MPC1 mitochondrial pyruvate carrier 1 BRP44L, CGI-129, MPYCD, SLC54A1, Q9Y5U8, gene:51660 17 GFP, yeast mito homolog+, mito protein domain+, MS/MS++ MIM Metabolism > Carbohydrate metabolism > Gluconeogenesis;
Metabolism > Carbohydrate metabolism > Pyruvate metabolism;
Metabolism > Carbohydrate metabolism > TCA-associated;
Small molecule transport all 14

SLC25A22 solute carrier family 25 member 22 EIEE3, GC-1, GC1, NET44, Q9H936, gene:79751 17 literature, yeast mito homolog+, mito protein domain+, MS/MS++ MIM Small molecule transport > SLC25A family cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, heart, adipose, smallintestine, largeintestine, stomach, placenta, testis

SLC25A31 solute carrier family 25 member 31 AAC4, ANT 4, ANT4, SFEC35kDa, Q9H0C2, gene:83447 17 literature, yeast mito homolog+, mito protein domain+, MS/MS++ MIM Metabolism > Nucleotide metabolism > Nucleotide import;
Signaling > Calcium homeostasis > Mitochondrial permeability transition pore;
Small molecule transport > SLC25A family all 14

SLC25A18 solute carrier family 25 member 18 GC2, Q9H1K4, gene:83733 17 literature, yeast mito homolog+, mito protein domain+, MS/MS++ MIM Small molecule transport > SLC25A family cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, adipose, smallintestine, largeintestine, stomach, placenta, testis

TRNT1 tRNA nucleotidyl transferase 1 CCA1, CGI-47, MtCCA, RPEM, SIFD, Q96Q11, gene:51095 17 literature, targetP signal, yeast mito homolog, Rickettsial homolog, mito protein domain, induction, MS/MS Matrix Mitochondrial central dogma > mtRNA metabolism > mt-tRNA modifications spinalcord, adipose, placenta, testis

IDE insulin degrading enzyme INSULYSIN, P14735, gene:3416 17 literature, APEX_matrix, targetP signal, Rickettsial homolog, mito protein domain+, coexpression, MS/MS+ Matrix Protein import, sorting and homeostasis > Protein homeostasis > Proteases cerebrum, cerebellum, brainstem, kidney, liver, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis

GSTK1 glutathione S-transferase kappa 1 GST, GST 13-13, GST13, GST13-13, GSTK1-1, hGSTK1, Q9Y2Q3, gene:373156 17 literature, mito protein domain+, coexpression+, MS/MS++ Matrix Metabolism > Detoxification > Xenobiotic metabolism all 14

VARS2 valyl-tRNA synthetase 2, mitochondrial COXPD20, VALRS, VARS2L, VARSL, Q5ST30, gene:57176 17 APEX_matrix, targetP signal, Rickettsial homolog, mito protein domain, coexpression, MS/MS++ Matrix Mitochondrial central dogma > Translation > mt-tRNA synthetases cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis

HSPE1 heat shock protein family E (Hsp10) member 1 CPN10, EPF, GROES, HSP10, P61604, gene:3336 17 literature, APEX_IMS, APEX_matrix, yeast mito homolog++, Rickettsial homolog, mito protein domain, MS/MS++ Matrix Protein import, sorting and homeostasis > Protein homeostasis > Chaperones all 14

MRPL54 mitochondrial ribosomal protein L54 L54mt, MRP-L54, Q6P161, gene:116541 17 literature, APEX_matrix, yeast mito homolog++, mito protein domain, coexpression, MS/MS+ Matrix Mitochondrial central dogma > Translation > Mitochondrial ribosome cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis

MRPL38 mitochondrial ribosomal protein L38 HSPC262, L38MT, MRP-L3, MRP-L38, RPML3, Q96DV4, gene:64978 17 APEX_matrix, targetP signal, mito protein domain, coexpression++, MS/MS+ Matrix Mitochondrial central dogma > Translation > Mitochondrial ribosome cerebrum, spinalcord, heart, smallintestine, largeintestine, stomach, placenta, testis

MRPL58 mitochondrial ribosomal protein L58 DS-1, DS1, ICT1, MRP-L58, Q14197, gene:3396 17 APEX_matrix, GFP, targetP signal+, mito protein domain, induction, coexpression, MS/MS Matrix Mitochondrial central dogma > Translation > Mitochondrial ribosome;
Mitochondrial central dogma > Translation > Translation factors all 14

MTCH1 mitochondrial carrier 1 CGI-64, PIG60, PSAP, SLC25A49, Q9NZJ7, gene:23787 17 literature, yeast mito homolog+, mito protein domain+, MS/MS++ MOM Small molecule transport > SLC25A family cerebrum, cerebellum, brainstem, spinalcord, kidney, heart, adipose, smallintestine, largeintestine, stomach, placenta, testis

TRIAP1 TP53 regulated inhibitor of apoptosis 1 HSPC132, MDM35, P53CSV, WF-1, O43715, gene:51499 17 literature, yeast mito homolog++, mito protein domain, induction, MS/MS++ IMS Metabolism > Lipid metabolism > Cardiolipin synthesis;
Metabolism > Lipid metabolism > Phospholipid metabolism adipose, stomach, placenta

PTCD3 pentatricopeptide repeat domain 3 MRP-S39, Q96EY7, gene:55037 17 literature, APEX_matrix, targetP signal+, mito protein domain+, MS/MS++ Matrix Mitochondrial central dogma > Translation > Mitochondrial ribosome;
Mitochondrial central dogma > Translation > Mitochondrial ribosome assembly all 14

COX10 cytochrome c oxidase assembly factor heme A:farnesyltransferase COX10 Q12887, gene:1352 17 literature, yeast mito homolog, Rickettsial homolog, mito protein domain, induction, coexpression++ MIM OXPHOS > Complex IV > CIV assembly factors;
Metabolism > Metals and cofactors > Heme synthesis and processing;
OXPHOS > OXPHOS assembly factors none

AKAP1 A-kinase anchoring protein 1 AKAP, AKAP121, AKAP149, AKAP84, D-AKAP1, PPP1R43, PRKA1, SAKAP84, TDRD17, Q92667, gene:8165 17 literature, mito protein domain, induction, coexpression++, MS/MS+ MOM Signaling > cAMP-PKA signaling cerebellum, skeletalmuscle, testis

MRM3 mitochondrial rRNA methyltransferase 3 RMTL1, RNMTL1, Q9HC36, gene:55178 17 APEX_matrix, GFP, targetP signal+, mito protein domain+, induction, coexpression Matrix Mitochondrial central dogma > mtRNA metabolism > mt-rRNA modifications;
Mitochondrial central dogma > Translation > Mitochondrial ribosome assembly none

AK2 adenylate kinase 2 ADK2, P54819, gene:204 17 literature, APEX_IMS, Rickettsial homolog, mito protein domain, coexpression+, MS/MS++ IMS Metabolism > Nucleotide metabolism > Nucleotide synthesis and processing all 14

TMEM70 transmembrane protein 70 MC5DN2, Q9BUB7, gene:54968 17 literature, APEX_matrix, mito protein domain+, coexpression++, MS/MS MIM OXPHOS > Complex I > CI assembly factors;
OXPHOS > Complex V > CV assembly factors;
OXPHOS > OXPHOS assembly factors cerebrum, cerebellum, brainstem, spinalcord, liver, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis

NOA1 nitric oxide associated 1 C4orf14, MTG3, hAtNOS1, hNOA1, mAtNOS1, Q8NC60, gene:84273 17 literature, APEX_matrix, targetP signal+, yeast mito homolog++, mito protein domain, coexpression MIM Mitochondrial central dogma > mtRNA metabolism > mtRNA granules;
Mitochondrial central dogma > Translation > Mitochondrial ribosome assembly adipose, largeintestine

MCUR1 mitochondrial calcium uniporter regulator 1 C6orf79, CCDC90A, FMP32, Q96AQ8, gene:63933 17 APEX_matrix, yeast mito homolog+, mito protein domain+, induction, MS/MS MIM none cerebrum, cerebellum, brainstem, spinalcord, kidney, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta

TMEM14C transmembrane protein 14C C6orf53, HSPC194, MSTP073, NET26, bA421M1.6, Q9P0S9, gene:51522 17 literature, mito protein domain+, induction, coexpression, MS/MS+ MIM none all 14

NDUFC1 NADH:ubiquinone oxidoreductase subunit C1 KFYI, O43677, gene:4717 17 literature, targetP signal+, mito protein domain+, coexpression++ MIM OXPHOS > Complex I > CI subunits;
OXPHOS > OXPHOS subunits none

MRPL35 mitochondrial ribosomal protein L35 L35mt, MRP-L35, Q9NZE8, gene:51318 17 literature, APEX_matrix, targetP signal, mito protein domain, induction, coexpression++ Matrix Mitochondrial central dogma > Translation > Mitochondrial ribosome largeintestine, testis

NDUFA3 NADH:ubiquinone oxidoreductase subunit A3 B9, CI-B9, O95167, gene:4696 17 literature, induction, coexpression++, MS/MS++ MIM OXPHOS > Complex I > CI subunits;
OXPHOS > OXPHOS subunits all 14

PDSS1 decaprenyl diphosphate synthase subunit 1 COQ1, COQ10D2, COQ1A, DPS, SPS, TPRT, TPT, TPT 1, hDPS1, Q5T2R2, gene:23590 17 APEX_matrix, targetP signal, yeast mito homolog+, Rickettsial homolog, mito protein domain, coexpression MIM Metabolism > Metals and cofactors > Coenzyme Q metabolism skeletalmuscle, largeintestine, placenta, testis

SLC25A16 solute carrier family 25 member 16 D10S105E, GDA, GDC, HGT.1, ML7, hML7, P16260, gene:8034 17 literature, yeast mito homolog+, mito protein domain+, coexpression, MS/MS MIM Small molecule transport > SLC25A family cerebrum, brainstem, spinalcord, liver, adipose, largeintestine, stomach, placenta, testis

FMC1 formation of mitochondrial complex V assembly factor 1 homolog C7orf55, HSPC268, 0, gene:154791 17 literature, APEX_matrix, targetP signal, induction, coexpression++, MS/MS Matrix OXPHOS > Complex V > CV assembly factors;
OXPHOS > OXPHOS assembly factors cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, heart, adipose, smallintestine, largeintestine, stomach, placenta, testis

ACSM3 acyl-CoA synthetase medium chain family member 3 SA, SAH, Q53FZ2, gene:6296 17 literature, targetP signal+, Rickettsial homolog, mito protein domain, MS/MS++ Matrix Metabolism > Lipid metabolism > Fatty acid oxidation kidney, liver, adipose, smallintestine, largeintestine, stomach

NDUFAF6 NADH:ubiquinone oxidoreductase complex assembly factor 6 C8orf38, FRTS5, MC1DN17, Q330K2, gene:137682 16 literature, targetP signal, mito protein domain, induction, coexpression++, MS/MS MIM OXPHOS > Complex I > CI assembly factors;
OXPHOS > OXPHOS assembly factors adipose, smallintestine, largeintestine, placenta, testis

UCP1 uncoupling protein 1 SLC25A7, UCP, P25874, gene:7350 16 literature, yeast mito homolog+, mito protein domain+, coexpression, MS/MS MIM Small molecule transport > SLC25A family adipose

SFXN1 sideroflexin 1 SLC56A1, TCC, Q9H9B4, gene:94081 16 literature, mito protein domain+, coexpression, MS/MS++ MIM Metabolism > Amino acid metabolism > Serine metabolism;
Metabolism > Vitamin metabolism > Folate and 1-C metabolism;
Small molecule transport > Sideroflexins cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis

GLRX5 glutaredoxin 5 C14orf87, FLB4739, GRX5, PR01238, PRO1238, PRSA, SIDBA3, SPAHGC, Q86SX6, gene:51218 16 literature, APEX_matrix, yeast mito homolog+, Rickettsial homolog, mito protein domain, coexpression, MS/MS+ Matrix Metabolism > Metals and cofactors > Fe-S cluster biosynthesis;
Metabolism > Metals and cofactors > Fe-S-containing proteins all 14

PTRH1 peptidyl-tRNA hydrolase 1 homolog C9orf115, PTH1, Q86Y79, gene:138428 16 APEX_matrix, GFP, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain, MS/MS Matrix none cerebrum, cerebellum, brainstem, largeintestine, placenta

MRPS11 mitochondrial ribosomal protein S11 HCC-2, MRP-S11, S11mt, P82912, gene:64963 16 literature, targetP signal, Rickettsial homolog, mito protein domain, induction, coexpression, MS/MS Matrix Mitochondrial central dogma > Translation > Mitochondrial ribosome all 14

XPNPEP3 X-prolyl aminopeptidase 3 APP3, ICP55, NPHPL1, Q9NQH7, gene:63929 16 literature, APEX_matrix, yeast mito homolog, Rickettsial homolog, mito protein domain, coexpression, MS/MS+ Matrix Protein import, sorting and homeostasis > Protein import and sorting > Preprotein cleavage;
Protein import, sorting and homeostasis > Protein homeostasis > Proteases cerebrum, liver, adipose, smallintestine, largeintestine, stomach, placenta, testis

PDP1 pyruvate dehyrogenase phosphatase catalytic subunit 1 PDH, PDP, PDPC, PPM2A, PPM2C, Q9P0J1, gene:54704 16 literature, APEX_matrix, targetP signal, yeast mito homolog+, mito protein domain, MS/MS++ MIM Metabolism > Carbohydrate metabolism > Pyruvate metabolism cerebrum, cerebellum, spinalcord, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis

ECI1 enoyl-CoA delta isomerase 1 DCI, P42126, gene:1632 16 literature, APEX_matrix, targetP signal, mito protein domain, coexpression+, MS/MS++ Matrix Metabolism > Lipid metabolism > Fatty acid oxidation all 14

SLC25A21 solute carrier family 25 member 21 MTDPS18, ODC, ODC1, Q9BQT8, gene:89874 16 literature, yeast mito homolog+, mito protein domain+, MS/MS++ MIM Metabolism > Amino acid metabolism;
Small molecule transport > SLC25A family spinalcord, kidney, liver, testis

SLC25A29 solute carrier family 25 member 29 C14orf69, CACL, ORNT3, Q8N8R3, gene:123096 16 literature, yeast mito homolog+, mito protein domain+, MS/MS++ MIM Metabolism > Amino acid metabolism > Lysine metabolism;
Small molecule transport > SLC25A family placenta, testis

SLC25A45 solute carrier family 25 member 45 Q8N413, gene:283130 16 yeast mito homolog+, mito protein domain+, MS/MS++ MIM Small molecule transport > SLC25A family liver, adipose, smallintestine, stomach, placenta, testis

SDHAF3 succinate dehydrogenase complex assembly factor 3 ACN9, DC11, LYRM10, Sdh7, Q9NRP4, gene:57001 16 targetP signal, yeast mito homolog++, coexpression+, MS/MS Matrix OXPHOS > Complex II > CII assembly factors;
OXPHOS > OXPHOS assembly factors adipose, testis

PRELID3B PRELI domain containing 3B C20orf45, SLMO2, dJ543J19.5, Q9Y3B1, gene:51012 16 GFP, yeast mito homolog+, mito protein domain+, induction, coexpression IMS Metabolism > Lipid metabolism > Phospholipid metabolism none

SLC25A39 solute carrier family 25 member 39 CGI-69, CGI69, Q9BZJ4, gene:51629 16 literature, yeast mito homolog+, mito protein domain+, induction, coexpression MIM Small molecule transport > SLC25A family none

NDUFB3 NADH:ubiquinone oxidoreductase subunit B3 B12, CI-B12, MC1DN25, O43676, gene:4709 16 literature, APEX_IMS, APEX_matrix, mito protein domain+, coexpression+, MS/MS++ MIM OXPHOS > Complex I > CI subunits;
OXPHOS > OXPHOS subunits all 14

REXO2 RNA exonuclease 2 CGI-114, REX2, RFN, SFN, Q9Y3B8, gene:25996 16 APEX_matrix, targetP signal, yeast mito homolog++, mito protein domain, MS/MS+ Matrix Mitochondrial central dogma > mtRNA metabolism > mtRNA stability and decay all 14

GATM glycine amidinotransferase AGAT, AT, CCDS3, FRTS1, P50440, gene:2628 16 literature, targetP signal, mito protein domain+, MS/MS++ Matrix Metabolism > Nucleotide metabolism > Creatine metabolism spinalcord, kidney, liver, stomach, placenta, testis

TIMM10B translocase of inner mitochondrial membrane 10B FXC1, TIM10B, Tim9b, Q9Y5J6, gene:26515 16 literature, APEX_IMS, yeast mito homolog+, mito protein domain+, MS/MS+ MIM Protein import, sorting and homeostasis > Protein homeostasis > Chaperones;
Protein import, sorting and homeostasis > Protein import and sorting cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, heart, adipose, largeintestine, stomach, placenta, testis

SARS2 seryl-tRNA synthetase 2, mitochondrial SARS, SARSM, SERS, SYS, SerRS, SerRSmt, mtSerRS, Q9NP81, gene:54938 16 literature, APEX_matrix, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain, MS/MS Matrix Mitochondrial central dogma > Translation > mt-tRNA synthetases skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis

CHDH choline dehydrogenase Q8NE62, gene:55349 16 APEX_matrix, targetP signal+, mito protein domain, coexpression, MS/MS++ MIM Metabolism > Amino acid metabolism > Glycine metabolism;
Metabolism > Vitamin metabolism > Choline and betaine metabolism;
Metabolism > Vitamin metabolism > Folate and 1-C metabolism;
Metabolism > Electron carriers > Q-linked reactions, other spinalcord, kidney, liver, adipose, smallintestine, largeintestine, stomach, placenta, testis

MRPL57 mitochondrial ribosomal protein L57 MRP63, bMRP63, Q9BQC6, gene:78988 16 literature, APEX_matrix, targetP signal, mito protein domain+, coexpression, MS/MS+ Matrix Mitochondrial central dogma > Translation > Mitochondrial ribosome cerebrum, spinalcord, kidney, liver, adipose, smallintestine, largeintestine, placenta, testis

C8orf82 chromosome 8 open reading frame 82 Q6P1X6, gene:414919 16 APEX_matrix, targetP signal, coexpression+, MS/MS++ Matrix none all 14

EARS2 glutamyl-tRNA synthetase 2, mitochondrial COXPD12, MSE1, gluRS, mtGlnRS, Q5JPH6, gene:124454 16 literature, APEX_matrix, GFP, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain, coexpression Matrix Mitochondrial central dogma > Translation > mt-tRNA synthetases adipose, largeintestine, placenta

GRHPR glyoxylate and hydroxypyruvate reductase GLXR, GLYD, PH2, Q9UBQ7, gene:9380 16 APEX_matrix, targetP signal, yeast mito homolog, mito protein domain, coexpression, MS/MS++ Matrix Metabolism > Amino acid metabolism > Glyoxylate metabolism cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, heart, skeletalmuscle, adipose, largeintestine, stomach, placenta, testis

MTFMT mitochondrial methionyl-tRNA formyltransferase COXPD15, FMT1, MC1DN27, Q96DP5, gene:123263 16 literature, APEX_matrix, targetP signal+, yeast mito homolog++, Rickettsial homolog, mito protein domain, coexpression Matrix Mitochondrial central dogma > Translation > fMet processing;
Metabolism > Vitamin metabolism > Folate and 1-C metabolism none

PTCD2 pentatricopeptide repeat domain 2 Q8WV60, gene:79810 16 literature, mito protein domain+, induction, coexpression, MS/MS Matrix Mitochondrial central dogma > mtRNA metabolism > mtRNA granules;
Mitochondrial central dogma > mtRNA metabolism > Polycistronic mtRNA processing smallintestine, largeintestine, placenta

ECHDC1 ethylmalonyl-CoA decarboxylase 1 HEL-S-76, MMCD, dJ351K20.2, Q9NTX5, gene:55862 16 yeast mito homolog+, Rickettsial homolog, mito protein domain, coexpression, MS/MS+ unknown Metabolism > Lipid metabolism brainstem, spinalcord, liver, adipose, largeintestine, placenta, testis

HARS2 histidyl-tRNA synthetase 2, mitochondrial HARSL, HARSR, HO3, HisRS, PRLTS2, P49590, gene:23438 16 literature, APEX_matrix, GFP, targetP signal, Rickettsial homolog, mito protein domain, coexpression, MS/MS+ Matrix Mitochondrial central dogma > Translation > mt-tRNA synthetases cerebellum, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis

NDUFA4 NDUFA4 mitochondrial complex associated CI-9k, CI-MLRQ, COXFA4, MLRQ, O00483, gene:4697 16 literature, mito protein domain, coexpression++, MS/MS++ MIM OXPHOS > Complex IV > CIV subunits;
OXPHOS > OXPHOS subunits all 14

NDUFA13 NADH:ubiquinone oxidoreductase subunit A13 B16.6, CDA016, CGI-39, GRIM-19, GRIM19, MC1DN28, Q9P0J0, gene:51079 16 literature, APEX_IMS, mito protein domain, coexpression++, MS/MS++ MIM OXPHOS > Complex I > CI subunits;
OXPHOS > OXPHOS subunits all 14

NAXE NAD(P)HX epimerase AIBP, APOA1BP, PEBEL, YJEFN1, Q8NCW5, gene:128240 16 APEX_matrix, targetP signal, mito protein domain, coexpression, MS/MS++ Matrix Metabolism > Metals and cofactors > NAD biosynthesis and metabolism all 14

TMEM11 transmembrane protein 11 C17orf35, PM1, PMI, P17152, gene:8834 16 GFP, targetP signal, mito protein domain+, induction, coexpression, MS/MS MIM Mitochondrial dynamics and surveillance all 14

SELENOO selenoprotein O SELO, Q9BVL4, gene:83642 15 targetP signal+, yeast mito homolog, mito protein domain+, coexpression Matrix Metabolism > Detoxification > Selenoproteins spinalcord, liver, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis

NAGS N-acetylglutamate synthase AGAS, ARGA, Q8N159, gene:162417 15 literature, targetP signal, yeast mito homolog++, mito protein domain+, MS/MS Matrix Metabolism > Amino acid metabolism > Urea cycle liver

HSD17B4 hydroxysteroid 17-beta dehydrogenase 4 DBP, MFE-2, MFP-2, MPF-2, PRLTS1, SDR8C1, P51659, gene:3295 15 Rickettsial homolog, mito protein domain+, induction, coexpression unknown Metabolism > Lipid metabolism kidney, liver, adipose, largeintestine, stomach, testis

NDUFAF7 NADH:ubiquinone oxidoreductase complex assembly factor 7 C2orf56, MidA, PRO1853, Q7L592, gene:55471 15 APEX_matrix, GFP, targetP signal, Rickettsial homolog, mito protein domain+, coexpression, MS/MS MIM OXPHOS > Complex I > CI assembly factors;
OXPHOS > OXPHOS assembly factors cerebrum, brainstem, spinalcord, kidney, adipose, largeintestine, stomach, placenta, testis

OAT ornithine aminotransferase GACR, HOGA, OATASE, OKT, P04181, gene:4942 15 literature, APEX_matrix, targetP signal+, mito protein domain, coexpression, MS/MS++ Matrix Metabolism > Amino acid metabolism > Proline metabolism all 14

MRPL50 mitochondrial ribosomal protein L50 MRP-L50, Q8N5N7, gene:54534 15 literature, APEX_matrix, targetP signal, mito protein domain+, coexpression+, MS/MS Matrix Mitochondrial central dogma > Translation > Mitochondrial ribosome cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis

COX7B cytochrome c oxidase subunit 7B APLCC, LSDMCA2, P24311, gene:1349 15 literature, targetP signal, mito protein domain+, induction, MS/MS+ MIM OXPHOS > Complex IV > CIV subunits;
OXPHOS > OXPHOS subunits cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, heart, adipose, smallintestine, largeintestine, stomach, placenta

RARS2 arginyl-tRNA synthetase 2, mitochondrial ArgRS, DALRD2, PCH6, PRO1992, RARSL, Q5T160, gene:57038 15 literature, APEX_matrix, GFP, Rickettsial homolog, mito protein domain, coexpression, MS/MS++ Matrix Mitochondrial central dogma > Translation > mt-tRNA synthetases all 14

OXLD1 oxidoreductase like domain containing 1 C17orf90, Q5BKU9, gene:339229 15 targetP signal, yeast mito homolog, mito protein domain+, coexpression+ unknown none adipose, largeintestine, stomach

LACTB2 lactamase beta 2 CGI-83, Q53H82, gene:51110 15 targetP signal, yeast mito homolog+, mito protein domain, induction, MS/MS+ Matrix Mitochondrial central dogma > mtRNA metabolism > mtRNA stability and decay kidney, liver, largeintestine, stomach

NDUFA11 NADH:ubiquinone oxidoreductase subunit A11 B14.7, CI-B14.7, MC1DN14, Q86Y39, gene:126328 15 literature, APEX_IMS, mito protein domain+, induction, MS/MS++ MIM OXPHOS > Complex I > CI subunits;
OXPHOS > OXPHOS subunits all 14

AURKAIP1 aurora kinase A interacting protein 1 AIP, AKIP, MRP-S38, Q9NWT8, gene:54998 15 GFP, targetP signal, yeast mito homolog++, mito protein domain, coexpression+ Matrix Mitochondrial central dogma > Translation > Mitochondrial ribosome none

CHCHD7 coiled-coil-helix-coiled-coil-helix domain containing 7 COX23, Q9BUK0, gene:79145 15 yeast mito homolog, induction, coexpression, MS/MS+ IMS Metabolism > Metals and cofactors > Copper metabolism cerebrum, smallintestine, placenta, testis

SHMT2 serine hydroxymethyltransferase 2 GLYA, HEL-S-51e, SHMT, P34897, gene:6472 15 literature, APEX_matrix, targetP signal, Rickettsial homolog, mito protein domain, coexpression, MS/MS++ Matrix Metabolism > Amino acid metabolism > Serine metabolism;
Metabolism > Amino acid metabolism > Glycine metabolism;
Metabolism > Vitamin metabolism > Folate and 1-C metabolism cerebrum, brainstem, spinalcord, kidney, liver, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis

MTHFD2 methylenetetrahydrofolate dehydrogenase (NADP+ dependent) 2, methenyltetrahydrofolate cyclohydrolase NMDMC, P13995, gene:10797 15 literature, APEX_matrix, targetP signal, yeast mito homolog+, Rickettsial homolog, mito protein domain, MS/MS Matrix Metabolism > Vitamin metabolism > Folate and 1-C metabolism spinalcord, placenta, testis

RMDN1 regulator of microtubule dynamics 1 CGI-90, FAM82B, RMD-1, RMD1, Q96DB5, gene:51115 15 APEX_IMS, targetP signal+, induction, coexpression, MS/MS MOM none all 14

CPS1 carbamoyl-phosphate synthase 1 CPSASE1, PHN, P31327, gene:1373 15 literature, APEX_matrix, targetP signal, Rickettsial homolog, mito protein domain, MS/MS++ Matrix Metabolism > Amino acid metabolism > Urea cycle cerebellum, brainstem, spinalcord, liver, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta

MRPL14 mitochondrial ribosomal protein L14 L14mt, L32mt, MRP-L14, MRP-L32, MRPL32, RMPL32, RPML32, Q6P1L8, gene:64928 15 literature, APEX_matrix, targetP signal, Rickettsial homolog, mito protein domain, coexpression+, MS/MS Matrix Mitochondrial central dogma > Translation > Mitochondrial ribosome all 14

MTRES1 mitochondrial transcription rescue factor 1 C6orf203, HSPC230, PRED31, Q9P0P8, gene:51250 15 GFP, induction, coexpression++, MS/MS+ Matrix Mitochondrial central dogma > mtRNA metabolism > Transcription;
Mitochondrial central dogma > Translation all 14

RDH13 retinol dehydrogenase 13 SDR7C3, Q8NBN7, gene:112724 15 yeast mito homolog+, Rickettsial homolog, mito protein domain, induction, MS/MS MIM Metabolism > Vitamin metabolism > Vitamin A metabolism cerebrum, cerebellum, brainstem, spinalcord, adipose, largeintestine, stomach, placenta, testis

TRMT2B tRNA methyltransferase 2 homolog B CXorf34, dJ341D10.3, Q96GJ1, gene:79979 15 GFP, targetP signal, Rickettsial homolog, mito protein domain+, induction, coexpression Matrix Mitochondrial central dogma > mtRNA metabolism > mt-tRNA modifications;
Mitochondrial central dogma > mtRNA metabolism > mt-rRNA modifications none

MRPS24 mitochondrial ribosomal protein S24 HSPC335, MRP-S24, S24mt, bMRP-47, bMRP47, Q96EL2, gene:64951 15 literature, APEX_matrix, induction, coexpression++, MS/MS Matrix Mitochondrial central dogma > Translation > Mitochondrial ribosome cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis

COA3 cytochrome c oxidase assembly factor 3 CCDC56, COX25, HSPC009, MITRAC12, Q9Y2R0, gene:28958 15 APEX_IMS, APEX_matrix, mito protein domain+, coexpression, MS/MS++ MIM Mitochondrial central dogma > Translation > Translation factors;
OXPHOS > Complex IV > CIV assembly factors;
OXPHOS > OXPHOS assembly factors all 14

GSR glutathione-disulfide reductase GR, GSRD, HEL-75, HEL-S-122m, P00390, gene:2936 15 literature, APEX_matrix, targetP signal, Rickettsial homolog, mito protein domain, MS/MS++ Matrix Metabolism > Detoxification > ROS and glutathione metabolism spinalcord, kidney, liver, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis

ENDOG endonuclease G Q14249, gene:2021 15 literature, APEX_IMS, mito protein domain, coexpression++, MS/MS IMS Mitochondrial central dogma > mtDNA maintenance > mtDNA stability and decay;
Mitochondrial central dogma > mtRNA metabolism > mtRNA stability and decay;
Mitochondrial dynamics and surveillance > Apoptosis all 14

BCAT2 branched chain amino acid transaminase 2 BCAM, BCATM, BCT2, HVLI, PP18, O15382, gene:587 15 literature, APEX_matrix, targetP signal, Rickettsial homolog, mito protein domain, coexpression, MS/MS++ Matrix Metabolism > Amino acid metabolism > BCAA metabolism cerebrum, cerebellum, brainstem, kidney, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis

TEFM transcription elongation factor, mitochondrial C17orf42, Q96QE5, gene:79736 15 APEX_matrix, targetP signal, induction, coexpression++, MS/MS Matrix Mitochondrial central dogma > mtRNA metabolism > Transcription liver, heart, skeletalmuscle, adipose, largeintestine, stomach, placenta, testis

ACSF2 acyl-CoA synthetase family member 2 ACSMW, AVYV493, Q96CM8, gene:80221 15 APEX_matrix, targetP signal+, Rickettsial homolog, mito protein domain, MS/MS++ Matrix Metabolism > Lipid metabolism > Fatty acid oxidation cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, heart, adipose, smallintestine, largeintestine, stomach, placenta, testis

TRMU tRNA mitochondrial 2-thiouridylase LCAL3, MTO2, MTU1, TRMT, TRMT1, O75648, gene:55687 15 literature, APEX_matrix, yeast mito homolog++, Rickettsial homolog, mito protein domain, coexpression Matrix Mitochondrial central dogma > mtRNA metabolism > mt-tRNA modifications adipose, largeintestine, placenta

MRPS22 mitochondrial ribosomal protein S22 C3orf5, COXPD5, GIBT, GK002, MRP-S22, ODG7, RPMS22, P82650, gene:56945 15 literature, APEX_matrix, mito protein domain+, induction, coexpression, MS/MS Matrix Mitochondrial central dogma > Translation > Mitochondrial ribosome all 14

LAP3 leucine aminopeptidase 3 HEL-S-106, LAP, LAPEP, PEPS, P28838, gene:51056 15 APEX_matrix, targetP signal+, Rickettsial homolog, coexpression, MS/MS++ Matrix Protein import, sorting and homeostasis > Protein homeostasis > Proteases all 14

GLUD1 glutamate dehydrogenase 1 GDH, GDH1, GLUD, P00367, gene:2746 15 literature, APEX_matrix, Rickettsial homolog, mito protein domain, coexpression, MS/MS++ Matrix Metabolism > Amino acid metabolism > Glutamate metabolism;
Metabolism > Amino acid metabolism > GABA metabolism all 14

HMGCL 3-hydroxy-3-methylglutaryl-CoA lyase HL, P35914, gene:3155 15 literature, APEX_matrix, targetP signal+, mito protein domain, coexpression, MS/MS++ Matrix Metabolism > Carbohydrate metabolism > Ketone metabolism;
Metabolism > Amino acid metabolism > BCAA metabolism all 14

FHIT fragile histidine triad diadenosine triphosphatase AP3Aase, FRA3B, P49789, gene:2272 15 yeast mito homolog, Rickettsial homolog, mito protein domain, MS/MS++ Matrix Metabolism > Nucleotide metabolism > Nucleotide synthesis and processing cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, adipose, smallintestine, largeintestine, stomach, placenta, testis

MRPS33 mitochondrial ribosomal protein S33 CGI-139, MRP-S33, PTD003, S33mt, Q9Y291, gene:51650 15 literature, targetP signal, mito protein domain+, MS/MS++ Matrix Mitochondrial central dogma > Translation > Mitochondrial ribosome cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis

NUBPL nucleotide binding protein like C14orf127, IND1, MC1DN21, huInd1, Q8TB37, gene:80224 14 literature, targetP signal, Rickettsial homolog, mito protein domain, coexpression+, MS/MS+ Matrix 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 spinalcord, adipose, largeintestine, stomach, placenta, testis

LYRM2 LYR motif containing 2 DJ122O8.2, Q9NU23, gene:57226 14 APEX_matrix, GFP, targetP signal+, mito protein domain+, coexpression, MS/MS Matrix OXPHOS > Complex I > CI assembly factors;
OXPHOS > OXPHOS assembly factors heart, adipose, largeintestine, placenta

MPV17L MPV17 mitochondrial inner membrane protein like M-LPH, MLPH1, MLPH2, MPV17L1, Q2QL34, gene:255027 14 targetP signal, yeast mito homolog+, mito protein domain, MS/MS++ MIM Small molecule transport cerebrum, cerebellum, brainstem, spinalcord, kidney, liver

GADD45GIP1 GADD45G interacting protein 1 CKBBP2, CKbetaBP2, CRIF1, MRP-L59, PLINP, PLINP-1, PRG6, Plinp1, Q8TAE8, gene:90480 14 APEX_matrix, GFP, targetP signal, coexpression+, MS/MS++ Matrix Mitochondrial central dogma > Translation > Mitochondrial ribosome all 14

TFAM transcription factor A, mitochondrial MTDPS15, MTTF1, MTTFA, TCF6, TCF6L1, TCF6L2, TCF6L3, Q00059, gene:7019 14 literature, APEX_matrix, targetP signal, mito protein domain, induction, MS/MS++ Matrix Mitochondrial central dogma > mtDNA maintenance > mtDNA replication;
Mitochondrial central dogma > mtDNA maintenance > mtDNA nucleoid;
Mitochondrial central dogma > mtRNA metabolism > Transcription all 14

ALDH18A1 aldehyde dehydrogenase 18 family member A1 ADCL3, ARCL3A, GSAS, P5CS, PYCS, SPG9, SPG9A, SPG9B, P54886, gene:5832 14 literature, Rickettsial homolog, mito protein domain, coexpression, MS/MS++ Matrix Metabolism > Amino acid metabolism > Proline metabolism cerebrum, cerebellum, brainstem, spinalcord, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis

OXSM 3-oxoacyl-ACP synthase, mitochondrial CEM1, FASN2D, KASI, KS, Q9NWU1, gene:54995 14 literature, APEX_matrix, targetP signal, Rickettsial homolog, mito protein domain, MS/MS++ Matrix Metabolism > Lipid metabolism > Type II fatty acid synthesis liver, heart, adipose, smallintestine, largeintestine, placenta, testis

KYAT3 kynurenine aminotransferase 3 CCBL2, KAT3, KATIII, Q6YP21, gene:56267 14 APEX_matrix, targetP signal, Rickettsial homolog, mito protein domain, MS/MS++ Matrix Metabolism > Amino acid metabolism > Kynurenine metabolism;
Metabolism > Metals and cofactors > NAD biosynthesis and metabolism spinalcord, kidney, liver, heart, adipose, smallintestine, largeintestine, stomach, placenta, testis

TARS2 threonyl-tRNA synthetase 2, mitochondrial COXPD21, TARSL1, thrRS, Q9BW92, gene:80222 14 APEX_matrix, targetP signal, Rickettsial homolog, mito protein domain, MS/MS++ Matrix Mitochondrial central dogma > Translation > mt-tRNA synthetases cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis

TBRG4 transforming growth factor beta regulator 4 CPR2, FASTKD4, Q969Z0, gene:9238 14 literature, APEX_matrix, targetP signal+, coexpression, MS/MS++ Matrix Mitochondrial central dogma > mtRNA metabolism > Polycistronic mtRNA processing;
Mitochondrial central dogma > mtRNA metabolism > mtRNA stability and decay all 14

TMEM65 transmembrane protein 65 Q6PI78, gene:157378 14 literature, APEX_matrix, targetP signal+, coexpression, MS/MS++ MIM none cerebrum, cerebellum, brainstem, spinalcord, kidney, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis

ROMO1 reactive oxygen species modulator 1 C20orf52, MTGM, MTGMP, bA353C18.2, P60602, gene:140823 14 literature, APEX_IMS, yeast mito homolog++, mito protein domain+, MS/MS MIM Protein import, sorting and homeostasis > Protein import and sorting > TIM23 presequence pathway;
Small molecule transport cerebrum, cerebellum, brainstem, spinalcord, liver, skeletalmuscle, adipose, smallintestine, stomach, placenta, testis

PRDX5 peroxiredoxin 5 ACR1, AOEB166, B166, HEL-S-55, PLP, PMP20, PRDX6, PRXV, SBBI10, prx-V, P30044, gene:25824 14 literature, APEX_matrix, targetP signal, mito protein domain, induction, MS/MS++ Matrix Metabolism > Detoxification > ROS and glutathione metabolism all 14

DNAJC15 DnaJ heat shock protein family (Hsp40) member C15 DNAJD1, HSD18, MCJ, Q9Y5T4, gene:29103 14 GFP, mito protein domain, induction, coexpression, MS/MS+ MIM Protein import, sorting and homeostasis > Protein import and sorting > Import motor cerebrum, spinalcord, liver, adipose, smallintestine, largeintestine, placenta, testis

MAIP1 matrix AAA peptidase interacting protein 1 C2orf47, Q8WWC4, gene:79568 14 GFP, targetP signal, induction, coexpression, MS/MS+ Matrix Protein import, sorting and homeostasis > Protein homeostasis > Chaperones all 14

HIGD1A HIG1 hypoxia inducible domain family member 1A HIG1, RCF1a, Q9Y241, gene:25994 14 literature, APEX_IMS, mito protein domain+, induction, MS/MS++ MIM OXPHOS > Complex IV > CIV assembly factors;
OXPHOS > OXPHOS assembly factors;
OXPHOS > Respirasome assembly all 14

C16orf91 chromosome 16 open reading frame 91 CCSMST1, URLC5, gs103, Q4G0I0, gene:283951 14 APEX_IMS, targetP signal+, induction, coexpression, MS/MS IMS none brainstem, spinalcord, adipose, largeintestine, placenta, testis

RHOT2 ras homolog family member T2 ARHT2, C16orf39, MIRO-2, MIRO2, RASL, Q8IXI1, gene:89941 14 literature, mito protein domain+, induction, coexpression MOM Signaling > Calcium homeostasis > EF hand proteins;
Mitochondrial dynamics and surveillance > Trafficking cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis

SLC25A44 solute carrier family 25 member 44 Q96H78, gene:9673 14 yeast mito homolog+, mito protein domain+, induction, MS/MS MIM Metabolism > Amino acid metabolism > BCAA metabolism;
Small molecule transport > SLC25A family placenta

HAO2 hydroxyacid oxidase 2 GIG16, HAOX2, Q9NYQ3, gene:51179 14 yeast mito homolog+, Rickettsial homolog, mito protein domain, coexpression, MS/MS unknown Metabolism > Lipid metabolism kidney, largeintestine, testis

OXA1L OXA1L mitochondrial inner membrane protein OXA1, Q15070, gene:5018 14 literature, APEX_matrix, targetP signal, yeast mito homolog+, Rickettsial homolog, mito protein domain, coexpression MIM Protein import, sorting and homeostasis > Protein import and sorting > OXA;
Mitochondrial central dogma > Translation cerebrum, brainstem, spinalcord, kidney, heart, adipose, smallintestine, largeintestine, placenta, testis

SLC25A46 solute carrier family 25 member 46 HMSN6B, Q96AG3, gene:91137 14 yeast mito homolog++, mito protein domain+, induction MOM Small molecule transport > SLC25A family;
Mitochondrial dynamics and surveillance > Fission;
Mitochondrial dynamics and surveillance > Intramitochondrial membrane interactions cerebrum, spinalcord, adipose, placenta

RDH14 retinol dehydrogenase 14 PAN2, SDR7C4, Q9HBH5, gene:57665 14 yeast mito homolog+, Rickettsial homolog, mito protein domain, coexpression, MS/MS Membrane Metabolism > Vitamin metabolism > Vitamin A metabolism cerebrum, cerebellum, brainstem, spinalcord, smallintestine, largeintestine, stomach, placenta, testis

ATP5MPL ATP synthase membrane subunit 6.8PL 6.8PL, C14orf2, MLQ, MP68, PLPM, P56378, gene:9556 14 induction, coexpression++, MS/MS+ MIM OXPHOS > Complex V > CV subunits;
OXPHOS > OXPHOS subunits cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, heart, adipose, smallintestine, largeintestine, stomach, placenta

CA5B carbonic anhydrase 5B CA-VB, CAVB, Q9Y2D0, gene:11238 14 literature, targetP signal, mito protein domain, coexpression, MS/MS++ Matrix Metabolism > Amino acid metabolism > Urea cycle cerebellum, spinalcord, kidney, adipose, stomach, placenta, testis

COASY Coenzyme A synthase DPCK, NBIA6, NBP, PCH12, PPAT, UKR1, pOV-2, Q13057, gene:80347 14 APEX_matrix, targetP signal, mito protein domain, coexpression, MS/MS++ Matrix Metabolism > Metals and cofactors > Coenzyme A metabolism cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, adipose, smallintestine, largeintestine, stomach, placenta, testis

CISD3 CDGSH iron sulfur domain 3 MiNT, Miner2, P0C7P0, gene:284106 14 APEX_matrix, targetP signal, mito protein domain, coexpression, MS/MS++ Matrix Metabolism > Metals and cofactors > Fe-S cluster biosynthesis;
Metabolism > Metals and cofactors > Fe-S-containing proteins all 14

GSTZ1 glutathione S-transferase zeta 1 GSTZ1-1, MAAI, MAAID, MAI, O43708, gene:2954 14 APEX_matrix, mito protein domain, coexpression, MS/MS++ Matrix Metabolism > Detoxification > Xenobiotic metabolism all 14

COX6B2 cytochrome c oxidase subunit 6B2 COXVIB2, CT59, Q6YFQ2, gene:125965 14 literature, yeast mito homolog+, mito protein domain+, MS/MS+ MIM OXPHOS > Complex IV > CIV subunits;
OXPHOS > OXPHOS subunits testis

GLOD4 glyoxalase domain containing 4 C17orf25, CGI-150, HC71, Q9HC38, gene:51031 14 APEX_matrix, targetP signal+, coexpression, MS/MS++ Matrix none cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, heart, adipose, smallintestine, largeintestine, stomach, placenta, testis

ACAA1 acetyl-CoA acyltransferase 1 ACAA, PTHIO, THIO, P09110, gene:30 14 yeast mito homolog++, Rickettsial homolog, mito protein domain, coexpression unknown Metabolism > Lipid metabolism kidney, liver, adipose, smallintestine, largeintestine, stomach, placenta, testis

CROT carnitine O-octanoyltransferase COT, Q9UKG9, gene:54677 14 yeast mito homolog+, mito protein domain, induction, coexpression, MS/MS unknown Metabolism > Lipid metabolism > Fatty acid oxidation kidney, liver

PDF peptide deformylase, mitochondrial Q9HBH1, gene:64146 14 APEX_matrix, Rickettsial homolog, mito protein domain+, coexpression, MS/MS Matrix Mitochondrial central dogma > Translation > fMet processing;
Protein import, sorting and homeostasis > Protein homeostasis cerebrum, heart, skeletalmuscle, adipose, largeintestine, placenta, testis

COX19 cytochrome c oxidase assembly factor COX19 Q49B96, gene:90639 14 yeast mito homolog, mito protein domain, induction, coexpression+, MS/MS IMS OXPHOS > Complex IV > CIV assembly factors;
Metabolism > Metals and cofactors > Copper metabolism;
OXPHOS > OXPHOS assembly factors liver, placenta

AMT aminomethyltransferase GCE, GCST, GCVT, NKH, P48728, gene:275 14 targetP signal+, mito protein domain, MS/MS++ Matrix Metabolism > Amino acid metabolism > Glycine metabolism;
Metabolism > Amino acid metabolism > Glycine cleavage system;
Metabolism > Vitamin metabolism > Folate and 1-C metabolism cerebrum, brainstem, spinalcord, kidney, liver, largeintestine, stomach, placenta

PDPR pyruvate dehydrogenase phosphatase regulatory subunit PDP3, Q8NCN5, gene:55066 14 APEX_matrix, targetP signal+, mito protein domain, MS/MS++ Matrix Metabolism > Carbohydrate metabolism > Pyruvate metabolism cerebrum, cerebellum, brainstem, spinalcord, heart, skeletalmuscle, adipose, largeintestine, stomach, placenta, testis

NLN neurolysin AGTBP, EP24.16, MEP, MOP, Q9BYT8, gene:57486 14 literature, APEX_matrix, targetP signal+, mito protein domain, MS/MS++ Matrix Protein import, sorting and homeostasis > Protein homeostasis > Proteases cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis

BOLA1 bolA family member 1 CGI-143, Q9Y3E2, gene:51027 13 GFP, Rickettsial homolog, mito protein domain, coexpression, MS/MS++ Matrix Metabolism > Metals and cofactors > Fe-S cluster biosynthesis;
Metabolism > Metals and cofactors > Fe-S-containing proteins cerebrum, brainstem, spinalcord, kidney, liver, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis

ALAS2 5'-aminolevulinate synthase 2 ALAS-E, ALASE, ANH1, ASB, SIDBA1, XLDPP, XLEPP, XLSA, P22557, gene:212 13 literature, yeast mito homolog+, Rickettsial homolog, mito protein domain+, coexpression Matrix Metabolism > Metals and cofactors > Heme synthesis and processing none

GRPEL2 GrpE like 2, mitochondrial Mt-GrpE#2, Q8TAA5, gene:134266 13 literature, APEX_matrix, targetP signal+, Rickettsial homolog, mito protein domain+ Matrix Protein import, sorting and homeostasis > Protein import and sorting > Import motor;
Metabolism > Metals and cofactors > Fe-S cluster biosynthesis placenta

ALDH7A1 aldehyde dehydrogenase 7 family member A1 ATQ1, EPD, PDE, P49419, gene:501 13 literature, APEX_matrix, targetP signal, yeast mito homolog, mito protein domain, MS/MS++ Matrix Metabolism > Amino acid metabolism > Lysine metabolism;
Metabolism > Amino acid metabolism > Glycine metabolism;
Metabolism > Vitamin metabolism > Choline and betaine metabolism;
Metabolism > Vitamin metabolism > Folate and 1-C metabolism;
Metabolism > Detoxification > ROS and glutathione metabolism cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, adipose, smallintestine, largeintestine, stomach, placenta, testis

HDHD5 haloacid dehalogenase like hydrolase domain containing 5 CECR5, Q9BXW7, gene:27440 13 APEX_matrix, targetP signal, yeast mito homolog, mito protein domain, MS/MS++ Matrix none cerebellum, kidney, liver, skeletalmuscle, adipose, largeintestine, stomach, placenta, testis

GFER growth factor, augmenter of liver regeneration ALR, ERV1, HERV1, HPO, HPO1, HPO2, HSS, MMCHD, MPMCD, P55789, gene:2671 13 literature, GFP, yeast mito homolog++, mito protein domain, coexpression, MS/MS IMS Protein import, sorting and homeostasis > Protein import and sorting > MIA40 cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, adipose, smallintestine, largeintestine, stomach, placenta, testis

IMMP2L inner mitochondrial membrane peptidase subunit 2 IMMP2L-IT1, IMP2, IMP2-LIKE, Q96T52, gene:83943 13 literature, yeast mito homolog++, mito protein domain, coexpression, MS/MS MIM Protein import, sorting and homeostasis > Protein import and sorting > Preprotein cleavage;
Protein import, sorting and homeostasis > Protein homeostasis > Proteases cerebellum, brainstem, liver, skeletalmuscle, adipose, largeintestine, testis

COX4I2 cytochrome c oxidase subunit 4I2 COX4, COX4-2, COX4B, COX4L2, COXIV-2, dJ857M17.2, Q96KJ9, gene:84701 13 literature, targetP signal, yeast mito homolog++, mito protein domain+ MIM OXPHOS > Complex IV > CIV subunits;
OXPHOS > OXPHOS subunits cerebrum, spinalcord, heart, adipose, largeintestine, stomach, placenta

MRPL39 mitochondrial ribosomal protein L39 C21orf92, L39mt, L5mt, MRP-L5, MRPL5, MSTP003, PRED22, PRED66, RPML5, Q9NYK5, gene:54148 13 literature, APEX_matrix, targetP signal, Rickettsial homolog, induction, coexpression, MS/MS Matrix Mitochondrial central dogma > Translation > Mitochondrial ribosome cerebrum, cerebellum, brainstem, kidney, liver, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis

AADAT aminoadipate aminotransferase KAT2, KATII, KYAT2, Q8N5Z0, gene:51166 13 targetP signal, Rickettsial homolog, mito protein domain, coexpression, MS/MS++ Matrix Metabolism > Amino acid metabolism > Lysine metabolism kidney, liver

FUNDC2 FUN14 domain containing 2 DC44, HCBP6, HCC3, PD03104, Q9BWH2, gene:65991 13 mito protein domain+, MS/MS++ MOM Mitochondrial dynamics and surveillance > Organelle contact sites;
Signaling cerebrum, cerebellum, brainstem, spinalcord, kidney, heart, skeletalmuscle, adipose, largeintestine, stomach, placenta, testis

LYPLA1 lysophospholipase 1 APT-1, APT1, LPL-I, LPL1, hAPT1, O75608, gene:10434 13 Rickettsial homolog, mito protein domain, coexpression, MS/MS++ unknown Metabolism > Lipid metabolism;
Signaling all 14

PDHA2 pyruvate dehydrogenase E1 subunit alpha 2 PDHAL, P29803, gene:5161 13 targetP signal+, yeast mito homolog+, Rickettsial homolog, mito protein domain+ Matrix Metabolism > Carbohydrate metabolism > Pyruvate metabolism none

MTRF1L mitochondrial translational release factor 1 like HMRF1L, MRF1L, mtRF1a, Q9UGC7, gene:54516 13 literature, targetP signal+, yeast mito homolog+, Rickettsial homolog, mito protein domain Matrix Mitochondrial central dogma > Translation > Translation factors testis

GTPBP3 GTP binding protein 3, mitochondrial COXPD23, GTPBG3, MSS1, MTGP1, THDF1, Q969Y2, gene:84705 13 literature, APEX_matrix, targetP signal+, yeast mito homolog+, Rickettsial homolog, mito protein domain Matrix Mitochondrial central dogma > mtRNA metabolism > mt-tRNA modifications;
Metabolism > Vitamin metabolism > Folate and 1-C metabolism largeintestine

MTHFD2L methylenetetrahydrofolate dehydrogenase (NADP+ dependent) 2 like Q9H903, gene:441024 13 literature, targetP signal+, yeast mito homolog+, Rickettsial homolog, mito protein domain Matrix Metabolism > Vitamin metabolism > Folate and 1-C metabolism spinalcord, placenta

YME1L1 YME1 like 1 ATPase FTSH, MEG4, OPA11, PAMP, YME1L, Q96TA2, gene:10730 13 literature, APEX_IMS, Rickettsial homolog, mito protein domain+, MS/MS+ MIM Protein import, sorting and homeostasis > Protein homeostasis > Proteases cerebrum, cerebellum, brainstem, spinalcord, kidney, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis

ACOT2 acyl-CoA thioesterase 2 CTE-IA, CTE1A, MTE1, PTE2, PTE2A, ZAP128, P49753, gene:10965 13 literature, mito protein domain, induction, coexpression, MS/MS++ Matrix Metabolism > Lipid metabolism all 14

COA8 cytochrome c oxidase assembly factor 8 APOP, APOP1, APOPT1, C14orf153, Q96IL0, gene:84334 13 literature, targetP signal, mito protein domain+, coexpression+ MIM OXPHOS > Complex IV > CIV assembly factors;
OXPHOS > OXPHOS assembly factors largeintestine, placenta

MMAB metabolism of cobalamin associated B ATR, CFAP23, cblB, cob, Q96EY8, gene:326625 13 APEX_matrix, GFP, targetP signal, coexpression, MS/MS++ Matrix Metabolism > Vitamin metabolism > Vitamin B12 metabolism cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, heart, skeletalmuscle, adipose, largeintestine, stomach, placenta, testis

PPOX protoporphyrinogen oxidase PPO, V290M, VP, P50336, gene:5498 13 literature, APEX_matrix, yeast mito homolog++, mito protein domain, coexpression, MS/MS MIM Metabolism > Metals and cofactors > Heme synthesis and processing cerebrum, spinalcord, liver, adipose, smallintestine, largeintestine, stomach, placenta, testis

ABCD3 ATP binding cassette subfamily D member 3 ABC43, CBAS5, PMP70, PXMP1, ZWS2, P28288, gene:5825 13 GFP, targetP signal, Rickettsial homolog, mito protein domain, induction, coexpression Membrane Small molecule transport > ABC transporters kidney, liver, adipose, placenta, testis

SIRT3 sirtuin 3 SIR2L3, Q9NTG7, gene:23410 13 literature, APEX_matrix, mito protein domain, induction, coexpression, MS/MS+ Matrix Metabolism > Metals and cofactors > NAD biosynthesis and metabolism;
Signaling cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, heart, adipose, smallintestine, largeintestine, stomach, placenta, testis

LDHD lactate dehydrogenase D DLACD, DLD, Q86WU2, gene:197257 13 literature, yeast mito homolog+, mito protein domain, MS/MS++ MIM Metabolism > Carbohydrate metabolism kidney, liver, heart, adipose, stomach, placenta, testis

MRPL45 mitochondrial ribosomal protein L45 L45mt, MRP-L45, B4DEF8, gene:84311 13 literature, APEX_matrix, mito protein domain, induction, coexpression, MS/MS+ Matrix Mitochondrial central dogma > Translation > Mitochondrial ribosome cerebrum, brainstem, spinalcord, adipose, smallintestine, largeintestine, stomach, placenta, testis

C12orf65 chromosome 12 open reading frame 65 COXPD7, SPG55, Q9H3J6, gene:91574 13 literature, yeast mito homolog++, Rickettsial homolog, mito protein domain, MS/MS Matrix Mitochondrial central dogma > Translation > Translation factors cerebrum, brainstem, spinalcord, adipose, largeintestine, stomach, placenta

IMMP1L inner mitochondrial membrane peptidase subunit 1 IMMP1, IMP1, IMP1-LIKE, Q96LU5, gene:196294 13 literature, yeast mito homolog++, Rickettsial homolog, mito protein domain, MS/MS MIM Protein import, sorting and homeostasis > Protein import and sorting > Preprotein cleavage;
Protein import, sorting and homeostasis > Protein homeostasis > Proteases testis

POLG DNA polymerase gamma, catalytic subunit MDP1, MIRAS, MTDPS4A, MTDPS4B, PEO, POLG1, POLGA, SANDO, SCAE, P54098, gene:5428 13 literature, APEX_matrix, targetP signal, yeast mito homolog++, mito protein domain, MS/MS Matrix Mitochondrial central dogma > mtDNA maintenance > mtDNA replication;
Mitochondrial central dogma > mtDNA maintenance > mtDNA nucleoid;
Mitochondrial central dogma > mtDNA maintenance > mtDNA repair cerebrum, cerebellum, spinalcord, liver, adipose, largeintestine, placenta, testis

ATPAF1 ATP synthase mitochondrial F1 complex assembly factor 1 ATP11, ATP11p, Q5TC12, gene:64756 13 literature, APEX_matrix, yeast mito homolog++, mito protein domain+, MS/MS Matrix OXPHOS > Complex V > CV assembly factors;
OXPHOS > OXPHOS assembly factors cerebrum, brainstem, spinalcord, adipose, smallintestine, largeintestine, placenta, testis

BLOC1S1 biogenesis of lysosomal organelles complex 1 subunit 1 BLOS1, BORCS1, GCN5L1, MICoA, RT14, P78537, gene:2647 13 mito protein domain, coexpression++, MS/MS unknown Signaling placenta, testis

ALDH1L2 aldehyde dehydrogenase 1 family member L2 mtFDH, Q3SY69, gene:160428 13 literature, APEX_matrix, targetP signal, Rickettsial homolog, mito protein domain, coexpression, MS/MS Matrix Metabolism > Vitamin metabolism > Folate and 1-C metabolism brainstem, spinalcord, liver, skeletalmuscle, smallintestine, largeintestine, stomach, placenta

COX18 cytochrome c oxidase assembly factor COX18 COX18HS, Q8N8Q8, gene:285521 13 literature, targetP signal, yeast mito homolog+, Rickettsial homolog, mito protein domain, coexpression MIM OXPHOS > Complex IV > CIV assembly factors;
OXPHOS > OXPHOS assembly factors none

CPOX coproporphyrinogen oxidase COX, CPO, CPX, HARPO, HCP, P36551, gene:1371 13 literature, APEX_IMS, APEX_matrix, Rickettsial homolog, mito protein domain, coexpression, MS/MS++ MIM Metabolism > Metals and cofactors > Heme synthesis and processing cerebellum, brainstem, spinalcord, kidney, liver, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis

NNT nicotinamide nucleotide transhydrogenase GCCD4, Q13423, gene:23530 13 literature, APEX_matrix, Rickettsial homolog, mito protein domain, induction, coexpression, MS/MS+ MIM Metabolism > Metals and cofactors > NAD biosynthesis and metabolism cerebrum, spinalcord, skeletalmuscle, adipose, placenta

NDUFAF8 NADH:ubiquinone oxidoreductase complex assembly factor 8 C17orf89, MC1DN34, A1L188, gene:284184 13 targetP signal, induction, coexpression+, MS/MS IMS OXPHOS > Complex I > CI assembly factors;
OXPHOS > OXPHOS assembly factors adipose, stomach

ATP23 ATP23 metallopeptidase and ATP synthase assembly factor homolog KUB3, XRCC6BP1, Q9Y6H3, gene:91419 13 yeast mito homolog++, mito protein domain+, coexpression IMS Protein import, sorting and homeostasis > Protein homeostasis > Proteases cerebellum

DCAKD dephospho-CoA kinase domain containing Q8WVC6, gene:79877 13 GFP, yeast mito homolog, Rickettsial homolog, mito protein domain, coexpression, MS/MS Membrane none all 14

ATP5MF ATP synthase membrane subunit f ATP5J2, ATP5JL, P56134, gene:9551 13 literature, coexpression++, MS/MS++ MIM OXPHOS > Complex V > CV subunits;
OXPHOS > OXPHOS subunits all 14

SFXN3 sideroflexin 3 BA108L7.2, SFX3, SLC56A3, Q9BWM7, gene:81855 13 mito protein domain+, MS/MS++ MIM Metabolism > Amino acid metabolism > Serine metabolism;
Metabolism > Vitamin metabolism > Folate and 1-C metabolism;
Small molecule transport > Sideroflexins cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis

AASS aminoadipate-semialdehyde synthase LKR/SDH, LKRSDH, LORSDH, Q9UDR5, gene:10157 13 APEX_matrix, targetP signal, mito protein domain, coexpression, MS/MS++ Matrix Metabolism > Amino acid metabolism > Lysine metabolism cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, adipose, largeintestine, stomach, placenta, testis

DIABLO diablo IAP-binding mitochondrial protein DFNA64, SMAC, Q9NR28, gene:56616 13 literature, APEX_IMS, targetP signal+, mito protein domain+, MS/MS IMS Mitochondrial dynamics and surveillance > Apoptosis cerebrum, cerebellum, brainstem, spinalcord, liver, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis

COX8C cytochrome c oxidase subunit 8C COX8-3, Q7Z4L0, gene:341947 13 literature, targetP signal+, mito protein domain+, MS/MS MIM OXPHOS > Complex IV > CIV subunits;
OXPHOS > OXPHOS subunits testis

PXMP2 peroxisomal membrane protein 2 MPV17L3, PMP22, Q9NR77, gene:5827 13 GFP, targetP signal, yeast mito homolog+, mito protein domain, induction MIM none cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, heart, skeletalmuscle, adipose, smallintestine, largeintestine, placenta, testis

AGXT2 alanine--glyoxylate aminotransferase 2 AGT2, BAIBA, DAIBAT, Q9BYV1, gene:64902 13 literature, yeast mito homolog++, mito protein domain, MS/MS+ Matrix Metabolism > Amino acid metabolism > Glyoxylate metabolism kidney, liver

GLS2 glutaminase 2 GA, GLS, LGA, hLGA, Q9UI32, gene:27165 13 targetP signal, mito protein domain+, MS/MS++ Matrix Metabolism > Amino acid metabolism > Glutamate metabolism cerebrum, cerebellum, brainstem, spinalcord, liver, skeletalmuscle, smallintestine, largeintestine

APOO apolipoprotein O FAM121B, MIC26, MICOS26, Mic23, My025, Q9BUR5, gene:79135 13 literature, APEX_IMS, mito protein domain+, MS/MS++ MIM Mitochondrial dynamics and surveillance > Cristae formation > MICOS complex all 14

ACAD11 acyl-CoA dehydrogenase family member 11 ACAD-11, Q709F0, gene:84129 13 mito protein domain+, induction, coexpression MIM Metabolism > Lipid metabolism > Fatty acid oxidation spinalcord, kidney, liver, adipose

C5orf63 chromosome 5 open reading frame 63 YDR286C, A6NC05, gene:401207 13 targetP signal+, coexpression+, MS/MS Matrix none cerebrum, cerebellum, spinalcord, liver, heart, skeletalmuscle, adipose, placenta, testis

HOGA1 4-hydroxy-2-oxoglutarate aldolase 1 C10orf65, DHDPS2, DHDPSL, HP3, NPL2, Q86XE5, gene:112817 13 GFP, targetP signal, Rickettsial homolog, mito protein domain, coexpression, MS/MS Matrix Metabolism > Amino acid metabolism > Glyoxylate metabolism kidney, liver, adipose, largeintestine, placenta, testis

TTC19 tetratricopeptide repeat domain 19 2010204O13Rik, MC3DN2, Q6DKK2, gene:54902 13 literature, APEX_IMS, targetP signal+, mito protein domain, induction, MS/MS+ MIM OXPHOS > Complex III > CIII assembly factors;
OXPHOS > OXPHOS assembly factors cerebrum, cerebellum, spinalcord, adipose, largeintestine, stomach, testis

NGRN neugrin, neurite outgrowth associated DSC92, Q9NPE2, gene:51335 12 APEX_matrix, yeast mito homolog++, mito protein domain+, coexpression Matrix Mitochondrial central dogma > mtRNA metabolism > mt-rRNA modifications;
Mitochondrial central dogma > Translation > Mitochondrial ribosome assembly none

TCAIM T cell activation inhibitor, mitochondrial C3orf23, TOAG-1, TOAG1, Q8N3R3, gene:285343 12 literature, APEX_matrix, targetP signal, mito protein domain+, coexpression++ Matrix none none

GCSH glycine cleavage system protein H GCE, NKH, P23434, gene:2653 12 literature, APEX_matrix, targetP signal, yeast mito homolog, mito protein domain+, MS/MS Matrix Metabolism > Lipid metabolism > Lipoate insertion;
Metabolism > Amino acid metabolism > Glycine metabolism;
Metabolism > Amino acid metabolism > Glycine cleavage system spinalcord, liver, adipose, smallintestine, largeintestine, placenta, testis

SIRT5 sirtuin 5 SIR2L5, Q9NXA8, gene:23408 12 literature, APEX_matrix, Rickettsial homolog, mito protein domain, induction, coexpression, MS/MS Matrix Metabolism > Metals and cofactors > NAD biosynthesis and metabolism cerebrum, brainstem, spinalcord, liver, heart, adipose, largeintestine, stomach

GPT2 glutamic--pyruvic transaminase 2 ALT2, GPT 2, MRT49, Q8TD30, gene:84706 12 APEX_matrix, targetP signal, Rickettsial homolog, mito protein domain, MS/MS++ Matrix Metabolism > Amino acid metabolism > Glutamate metabolism cerebrum, brainstem, liver, skeletalmuscle, adipose, stomach, placenta, testis

TOMM6 translocase of outer mitochondrial membrane 6 OBTP, TOM6, Q96B49, gene:100188893 12 GFP, mito protein domain+, coexpression, MS/MS+ MOM Protein import, sorting and homeostasis > Protein import and sorting > TOM cerebrum, cerebellum, brainstem, spinalcord, liver, heart, adipose, smallintestine, largeintestine, stomach, placenta, testis

FAM162A family with sequence similarity 162 member A C3orf28, E2IG5, HGTD-P, Q96A26, gene:26355 12 APEX_IMS, APEX_matrix, targetP signal, induction, coexpression, MS/MS++ MIM none all 14

ABCB6 ATP binding cassette subfamily B member 6 (Langereis blood group) ABC, LAN, MTABC3, PRP, umat, Q9NP58, gene:10058 12 literature, yeast mito homolog+, Rickettsial homolog, mito protein domain, coexpression, MS/MS MOM Metabolism > Metals and cofactors > Heme synthesis and processing;
Small molecule transport > ABC transporters cerebrum, brainstem, liver, largeintestine, stomach, placenta, testis

MACROD1 mono-ADP ribosylhydrolase 1 LRP16, Q9BQ69, gene:28992 12 APEX_matrix, targetP signal+, coexpression, MS/MS++ Matrix Signaling cerebrum, cerebellum, brainstem, spinalcord, liver, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis

TST thiosulfate sulfurtransferase RDS, Q16762, gene:7263 12 literature, APEX_matrix, Rickettsial homolog, mito protein domain, coexpression, MS/MS++ Matrix Metabolism > Detoxification > Xenobiotic metabolism;
Metabolism > Sulfur metabolism all 14

MT-CO1 cytochrome c oxidase subunit I COI, MTCO1, P00395, gene:4512 12 literature, APEX_IMS, yeast mito homolog++, Rickettsial homolog, mito protein domain, MS/MS MIM OXPHOS > Complex IV > CIV subunits;
Metabolism > Metals and cofactors > Heme-containing proteins;
OXPHOS > OXPHOS subunits all 14

MTFR1L mitochondrial fission regulator 1 like FAM54B, HYST1888, MST116, MSTP116, Q9H019, gene:56181 12 mito protein domain+, induction, coexpression, MS/MS unknown none cerebrum, cerebellum, brainstem, spinalcord, placenta, testis

SDR39U1 short chain dehydrogenase/reductase family 39U member 1 C14orf124, HCDI, Q9NRG7, gene:56948 12 mito protein domain, coexpression, MS/MS++ unknown none cerebrum, cerebellum, brainstem, spinalcord, kidney, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis

DMGDH dimethylglycine dehydrogenase DMGDHD, ME2GLYDH, Q9UI17, gene:29958 12 literature, targetP signal, mito protein domain, MS/MS++ Matrix Metabolism > Amino acid metabolism > Glycine metabolism;
Metabolism > Vitamin metabolism > Choline and betaine metabolism;
Metabolism > Vitamin metabolism > Folate and 1-C metabolism spinalcord, kidney, liver, smallintestine, stomach, placenta

SLC25A14 solute carrier family 25 member 14 BMCP1, UCP5, O95258, gene:9016 12 literature, yeast mito homolog+, mito protein domain+, MS/MS MIM Metabolism > Sulfur metabolism;
Small molecule transport > SLC25A family cerebrum, cerebellum, brainstem, placenta, testis

PRELID3A PRELI domain containing 3A C18orf43, HFL-EDDG1, SLMO1, Q96N28, gene:10650 12 yeast mito homolog+, mito protein domain+, MS/MS IMS Metabolism > Lipid metabolism > Cardiolipin synthesis;
Metabolism > Lipid metabolism > Phospholipid metabolism cerebrum, cerebellum, spinalcord

CHCHD5 coiled-coil-helix-coiled-coil-helix domain containing 5 C2orf9, CHTM1, MIC14, MIX14, Q9BSY4, gene:84269 12 APEX_IMS, yeast mito homolog++, mito protein domain, coexpression, MS/MS IMS none adipose, placenta, testis

TOMM70 translocase of outer mitochondrial membrane 70 TOMM70A, Tom70, O94826, gene:9868 12 literature, yeast mito homolog+, mito protein domain, induction, coexpression MOM Protein import, sorting and homeostasis > Protein import and sorting > TOM cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, skeletalmuscle, adipose, largeintestine, stomach, placenta, testis

NIT1 nitrilase 1 Q86X76, gene:4817 12 APEX_matrix, targetP signal, mito protein domain, coexpression, MS/MS++ Matrix Metabolism > Detoxification > ROS and glutathione metabolism cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, adipose, smallintestine, largeintestine, stomach, placenta, testis

FAHD1 fumarylacetoacetate hydrolase domain containing 1 C16orf36, YISKL, Q6P587, gene:81889 12 literature, APEX_matrix, mito protein domain, coexpression, MS/MS++ Matrix Metabolism > Carbohydrate metabolism > TCA-associated all 14

MCU mitochondrial calcium uniporter C10orf42, CCDC109A, HsMCU, Q8NE86, gene:90550 12 literature, GFP, mito protein domain+, MS/MS++ MIM Signaling > Calcium homeostasis > Calcium cycle;
Small molecule transport > Calcium uniporter cerebrum, cerebellum, brainstem, spinalcord, liver, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta

CYP27A1 cytochrome P450 family 27 subfamily A member 1 CP27, CTX, CYP27, Q02318, gene:1593 12 literature, targetP signal+, mito protein domain, MS/MS++ MIM Metabolism > Lipid metabolism > Cholesterol, bile acid, steroid synthesis;
Metabolism > Metals and cofactors > Heme-containing proteins kidney, liver, skeletalmuscle, adipose, smallintestine, placenta, testis

MT-ND2 MTND2 MTND2, P03891, gene:4536 12 literature, Rickettsial homolog, mito protein domain+, MS/MS+ MIM OXPHOS > Complex I > CI subunits;
OXPHOS > OXPHOS subunits cerebrum, cerebellum, brainstem, spinalcord, liver, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis

MT-ND4 NADH dehydrogenase, subunit 4 (complex I) MTND4, P03905, gene:4538 12 literature, APEX_IMS, APEX_matrix, Rickettsial homolog, mito protein domain+, MS/MS+ MIM OXPHOS > Complex I > CI subunits;
OXPHOS > OXPHOS subunits all 14

MT-ND5 NADH dehydrogenase, subunit 5 (complex I) MTND5, P03915, gene:4540 12 literature, APEX_IMS, APEX_matrix, Rickettsial homolog, mito protein domain+, MS/MS+ MIM OXPHOS > Complex I > CI subunits;
OXPHOS > OXPHOS subunits all 14

DELE1 DAP3 binding cell death enhancer 1 DELE, DELE1(L), KIAA0141, Q14154, gene:9812 12 targetP signal+, mito protein domain, induction, coexpression MIM Signaling none

FASTKD1 FAST kinase domains 1 Q53R41, gene:79675 12 APEX_matrix, induction, coexpression++ Matrix Mitochondrial central dogma > mtRNA metabolism > mtRNA granules;
Mitochondrial central dogma > mtRNA metabolism > Polycistronic mtRNA processing;
Mitochondrial central dogma > mtRNA metabolism > mtRNA stability and decay liver, largeintestine

MRPS31 mitochondrial ribosomal protein S31 IMOGN38, MRP-S31, S31mt, Q92665, gene:10240 12 literature, APEX_matrix, targetP signal, induction, coexpression, MS/MS Matrix Mitochondrial central dogma > Translation > Mitochondrial ribosome all 14

ALDH1L1 aldehyde dehydrogenase 1 family member L1 10-FTHFDH, 10-fTHF, FDH, FTHFD, O75891, gene:10840 12 APEX_matrix, Rickettsial homolog, mito protein domain, induction, coexpression, MS/MS Matrix Metabolism > Vitamin metabolism > Folate and 1-C metabolism cerebrum, liver, smallintestine, largeintestine, stomach, placenta, testis

PTCD1 pentatricopeptide repeat domain 1 O75127, gene:26024 11 targetP signal+, mito protein domain+, coexpression Matrix Mitochondrial central dogma > mtRNA metabolism > mtRNA granules cerebellum, largeintestine, placenta

HADHB hydroxyacyl-CoA dehydrogenase trifunctional multienzyme complex subunit beta ECHB, MSTP029, MTPB, TP-BETA, P55084, gene:3032 11 literature, targetP signal, Rickettsial homolog, mito protein domain, MS/MS++ MIM Metabolism > Carbohydrate metabolism > Ketone metabolism;
Metabolism > Lipid metabolism > Fatty acid oxidation;
Metabolism > Amino acid metabolism > Lysine metabolism all 14

TRIT1 tRNA isopentenyltransferase 1 COXPD35, GRO1, IPPT, IPT, IPTase, MOD5, hGRO1, Q9H3H1, gene:54802 11 literature, APEX_matrix, targetP signal, yeast mito homolog, Rickettsial homolog, mito protein domain, coexpression Matrix Mitochondrial central dogma > mtRNA metabolism > mt-tRNA modifications;
Mitochondrial central dogma > mtRNA metabolism > mt-rRNA modifications cerebellum

CRLS1 cardiolipin synthase 1 C20orf155, CLS, CLS1, GCD10, dJ967N21.6, Q9UJA2, gene:54675 11 literature, targetP signal, yeast mito homolog, Rickettsial homolog, mito protein domain, coexpression MIM Metabolism > Lipid metabolism > Cardiolipin synthesis none

GLYCTK glycerate kinase HBEBP2, HBEBP4, HBeAgBP4A, Q8IVS8, gene:132158 11 targetP signal+, coexpression, MS/MS+ Matrix Metabolism > Carbohydrate metabolism kidney, liver, placenta

SLC25A32 solute carrier family 25 member 32 MFT, MFTC, RREI, Q9H2D1, gene:81034 11 literature, targetP signal, yeast mito homolog+, mito protein domain+ MIM Metabolism > Vitamin metabolism > Vitamin B2 metabolism;
Metabolism > Vitamin metabolism > Folate and 1-C metabolism;
Small molecule transport > SLC25A family adipose, placenta

AARS2 alanyl-tRNA synthetase 2, mitochondrial AARSL, COXPD8, LKENP, MT-ALARS, MTALARS, Q5JTZ9, gene:57505 11 literature, APEX_matrix, targetP signal+, Rickettsial homolog, mito protein domain, MS/MS Matrix Mitochondrial central dogma > Translation > mt-tRNA synthetases cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis

EXOG exo/endonuclease G ENDOGL1, ENDOGL2, ENGL, ENGL-a, ENGL-b, ENGLA, ENGLB, Q9Y2C4, gene:9941 11 APEX_IMS, targetP signal, mito protein domain, MS/MS++ MIM Mitochondrial central dogma > mtDNA maintenance > mtDNA replication;
Mitochondrial central dogma > mtDNA maintenance > mtDNA repair;
Mitochondrial central dogma > mtDNA maintenance > mtDNA stability and decay cerebrum, cerebellum, brainstem, spinalcord, adipose, largeintestine, placenta

CHCHD2 coiled-coil-helix-coiled-coil-helix domain containing 2 C7orf17, MIX17B, MNRR1, NS2TP, PARK22, Q9Y6H1, gene:51142 11 APEX_IMS, targetP signal, yeast mito homolog+, mito protein domain, coexpression IMS Mitochondrial dynamics and surveillance > Apoptosis none

KMO kynurenine 3-monooxygenase dJ317G22.1, O15229, gene:8564 11 yeast mito homolog, Rickettsial homolog, mito protein domain, MS/MS++ MOM Metabolism > Amino acid metabolism > Kynurenine metabolism;
Metabolism > Metals and cofactors > NAD biosynthesis and metabolism kidney, liver

SFXN5 sideroflexin 5 BBG-TCC, SLC56A5, Q8TD22, gene:94097 11 literature, mito protein domain+, MS/MS++ MIM Metabolism > Carbohydrate metabolism > TCA-associated;
Small molecule transport > Sideroflexins cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, adipose, largeintestine

ELAC2 elaC ribonuclease Z 2 COXPD17, ELC2, HPC2, Q9BQ52, gene:60528 11 APEX_matrix, GFP, targetP signal, Rickettsial homolog, mito protein domain, MS/MS Matrix Mitochondrial central dogma > mtRNA metabolism > Polycistronic mtRNA processing cerebellum, skeletalmuscle, adipose, largeintestine, stomach

GPX1 glutathione peroxidase 1 GPXD, GSHPX1, P07203, gene:2876 11 literature, APEX_matrix, targetP signal, mito protein domain, coexpression, MS/MS+ Matrix Metabolism > Detoxification > ROS and glutathione metabolism;
Metabolism > Detoxification > Selenoproteins cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, adipose, smallintestine, largeintestine, stomach, placenta, testis

CPT1B carnitine palmitoyltransferase 1B CPT1-M, CPT1M, CPTI, CPTI-M, M-CPT1, MCCPT1, MCPT1, Q92523, gene:1375 11 literature, yeast mito homolog+, mito protein domain, MS/MS++ MOM Metabolism > Lipid metabolism > Fatty acid oxidation;
Metabolism > Metals and cofactors > Carnitine synthesis and transport;
Metabolism > Metals and cofactors > Carnitine shuttle cerebellum, brainstem, spinalcord, kidney, liver, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis

FIS1 fission, mitochondrial 1 CGI-135, TTC11, Q9Y3D6, gene:51024 11 literature, yeast mito homolog++, mito protein domain+ MOM Mitochondrial dynamics and surveillance > Fission all 14

PRELID1 PRELI domain containing 1 CGI-106, PRELI, PX19, SBBI12, Q9Y255, gene:27166 11 literature, APEX_IMS, GFP, targetP signal, yeast mito homolog++, mito protein domain+ IMS Metabolism > Lipid metabolism > Cardiolipin synthesis;
Metabolism > Lipid metabolism > Phospholipid metabolism none

UCP3 uncoupling protein 3 SLC25A9, P55916, gene:7352 11 literature, yeast mito homolog+, mito protein domain+, MS/MS MIM Small molecule transport > SLC25A family spinalcord, heart, skeletalmuscle, adipose

SLC25A27 solute carrier family 25 member 27 UCP4, O95847, gene:9481 11 literature, yeast mito homolog+, mito protein domain+, MS/MS MIM Small molecule transport > SLC25A family cerebrum, cerebellum, brainstem, spinalcord

SLC25A40 solute carrier family 25 member 40 MCFP, Q8TBP6, gene:55972 11 literature, yeast mito homolog+, mito protein domain+, MS/MS MIM Small molecule transport > SLC25A family cerebrum, cerebellum, brainstem, spinalcord, adipose, largeintestine, stomach, placenta, testis

SLC25A23 solute carrier family 25 member 23 APC2, MCSC2, SCaMC-3, Q9BV35, gene:79085 11 literature, yeast mito homolog+, mito protein domain+, MS/MS MIM Metabolism > Nucleotide metabolism > Nucleotide import;
Signaling > Calcium homeostasis > EF hand proteins;
Small molecule transport > SLC25A family cerebrum, brainstem, spinalcord, liver, adipose, smallintestine, largeintestine, stomach, placenta

HEMK1 HemK methyltransferase family member 1 HEMK, MPRMC, MTQ1, Q9Y5R4, gene:51409 11 GFP, targetP signal, yeast mito homolog, Rickettsial homolog, mito protein domain+ Matrix Mitochondrial central dogma > Translation > Translation factors none

SLC25A36 solute carrier family 25 member 36 PNC2, Q96CQ1, gene:55186 11 GFP, yeast mito homolog+, mito protein domain+, coexpression MIM Metabolism > Nucleotide metabolism > Nucleotide import;
Small molecule transport > SLC25A family brainstem, spinalcord, placenta, testis

OMA1 OMA1 zinc metallopeptidase 2010001O09Rik, DAB1, MPRP-1, MPRP1, YKR087C, ZMPOMA1, peptidase, Q96E52, gene:115209 11 APEX_IMS, GFP, targetP signal+, yeast mito homolog++, coexpression MIM Protein import, sorting and homeostasis > Protein homeostasis > Proteases;
Mitochondrial dynamics and surveillance > Fusion;
Mitochondrial dynamics and surveillance > Fission skeletalmuscle, adipose, largeintestine

LETM2 leucine zipper and EF-hand containing transmembrane protein 2 SLC55A2, Q2VYF4, gene:137994 11 targetP signal, yeast mito homolog+, mito protein domain+ MIM none none

DHRS1 dehydrogenase/reductase 1 SDR19C1, Q96LJ7, gene:115817 11 Rickettsial homolog, mito protein domain, MS/MS++ MIM Metabolism > Lipid metabolism > Cholesterol, bile acid, steroid synthesis cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, adipose, smallintestine, largeintestine, stomach, placenta, testis

STOM stomatin BND7, EPB7, EPB72, P27105, gene:2040 11 yeast mito homolog+, Rickettsial homolog, mito protein domain, MS/MS Membrane none adipose, placenta, testis

METTL5 methyltransferase like 5 HSPC133, MRT72, Q9NRN9, gene:29081 10 Rickettsial homolog, mito protein domain+, induction, coexpression Matrix Mitochondrial central dogma > mtRNA metabolism > mt-rRNA modifications none

MSRA methionine sulfoxide reductase A PMSR, Q9UJ68, gene:4482 10 mito protein domain, coexpression, MS/MS++ Matrix Metabolism > Detoxification > ROS and glutathione metabolism;
Metabolism > Sulfur metabolism cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta

NUDT8 nudix hydrolase 8 Q8WV74, gene:254552 10 APEX_matrix, mito protein domain, coexpression, MS/MS++ Matrix Metabolism > Metals and cofactors > Coenzyme A metabolism cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, heart, skeletalmuscle, adipose, largeintestine, stomach, placenta, testis

NMNAT3 nicotinamide nucleotide adenylyltransferase 3 FKSG76, PNAT-3, PNAT3, Q96T66, gene:349565 10 literature, mito protein domain, coexpression, MS/MS++ Matrix Metabolism > Metals and cofactors > NAD biosynthesis and metabolism cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, heart, skeletalmuscle, adipose, smallintestine, largeintestine, placenta, testis

SLC25A38 solute carrier family 25 member 38 SIDBA2, Q96DW6, gene:54977 10 yeast mito homolog++, mito protein domain+, coexpression MIM 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 none

MPV17L2 MPV17 mitochondrial inner membrane protein like 2 FKSG24, Q567V2, gene:84769 10 GFP, targetP signal, yeast mito homolog+, mito protein domain, coexpression MIM Mitochondrial central dogma > Translation > Mitochondrial ribosome assembly largeintestine, placenta

ACLY ATP citrate lyase ACL, ATPCL, CLATP, P53396, gene:47 10 APEX_matrix, yeast mito homolog+, Rickettsial homolog, mito protein domain, coexpression Matrix Metabolism > Carbohydrate metabolism > TCA-associated cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, adipose, largeintestine, stomach, placenta, testis

ABCD2 ATP binding cassette subfamily D member 2 ABC39, ALDL1, ALDR, ALDRP, hALDR, Q9UBJ2, gene:225 10 targetP signal, Rickettsial homolog, mito protein domain, coexpression, MS/MS+ Membrane Small molecule transport > ABC transporters adipose

FLAD1 flavin adenine dinucleotide synthetase 1 FAD1, FADS, LSMFLAD, PP591, Q8NFF5, gene:80308 10 APEX_matrix, targetP signal, mito protein domain, induction, coexpression Matrix Metabolism > Vitamin metabolism > Vitamin B2 metabolism spinalcord, adipose, stomach

LIPT1 lipoyltransferase 1 LIPT1D, Q9Y234, gene:51601 10 GFP, yeast mito homolog++, mito protein domain, coexpression Matrix Metabolism > Lipid metabolism > Lipoate insertion adipose, placenta

DGLUCY D-glutamate cyclase C14orf159, Q7Z3D6, gene:80017 10 literature, APEX_matrix, targetP signal, coexpression, MS/MS++ Matrix Metabolism > Amino acid metabolism all 14

PCK2 phosphoenolpyruvate carboxykinase 2, mitochondrial PEPCK, PEPCK-M, PEPCK2, Q16822, gene:5106 10 literature, targetP signal, mito protein domain, coexpression, MS/MS++ Matrix Metabolism > Carbohydrate metabolism > Gluconeogenesis;
Metabolism > Carbohydrate metabolism > TCA-associated cerebrum, cerebellum, brainstem, spinalcord, adipose, smallintestine, largeintestine, stomach, placenta, testis

NADK2 NAD kinase 2, mitochondrial C5orf33, DECRD, MNADK, NADKD1, Q4G0N4, gene:133686 10 APEX_matrix, targetP signal, mito protein domain, coexpression, MS/MS++ Matrix Metabolism > Metals and cofactors > NAD biosynthesis and metabolism cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis

MPST mercaptopyruvate sulfurtransferase MST, TST2, TUM1, P25325, gene:4357 10 APEX_matrix, mito protein domain, coexpression, MS/MS++ MIM Metabolism > Detoxification > Xenobiotic metabolism;
Metabolism > Sulfur metabolism cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis

NUDT13 nudix hydrolase 13 Q86X67, gene:25961 10 GFP, targetP signal, mito protein domain, induction, coexpression, MS/MS Matrix Metabolism > Metals and cofactors > NAD biosynthesis and metabolism cerebellum, brainstem, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta

ACACA acetyl-CoA carboxylase alpha ACAC, ACACAD, ACC, ACC1, ACCA, Q13085, gene:31 10 targetP signal, Rickettsial homolog, mito protein domain, coexpression Matrix Metabolism > Lipid metabolism > Type II fatty acid synthesis;
Metabolism > Vitamin metabolism > Biotin utilizing proteins brainstem, spinalcord, liver, adipose, largeintestine, stomach, placenta, testis

ABAT 4-aminobutyrate aminotransferase GABA-AT, GABAT, NPD009, P80404, gene:18 10 literature, APEX_matrix, targetP signal, mito protein domain, MS/MS++ Matrix Metabolism > Amino acid metabolism > GABA metabolism;
Metabolism > Metals and cofactors > Fe-S-containing proteins cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, heart, adipose, smallintestine, largeintestine, stomach, placenta, testis

HMGCS2 3-hydroxy-3-methylglutaryl-CoA synthase 2 P54868, gene:3158 10 literature, targetP signal, mito protein domain, MS/MS++ Matrix Metabolism > Carbohydrate metabolism > Ketone metabolism cerebellum, spinalcord, liver, adipose, smallintestine, largeintestine, stomach, placenta, testis

ALDH5A1 aldehyde dehydrogenase 5 family member A1 SSADH, SSDH, P51649, gene:7915 10 APEX_matrix, targetP signal, mito protein domain, MS/MS++ Matrix Metabolism > Amino acid metabolism > GABA metabolism all 14

CKMT1A creatine kinase, mitochondrial 1A CKMT1, U-MtCK, mia-CK, 0, gene:548596 10 literature, targetP signal, mito protein domain, MS/MS++ MIM Metabolism > Nucleotide metabolism > Creatine metabolism cerebrum, cerebellum, brainstem, spinalcord, kidney, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta

PISD phosphatidylserine decarboxylase DJ858B16, LIBF, PSD, PSDC, PSSC, dJ858B16.2, Q9UG56, gene:23761 10 yeast mito homolog++, mito protein domain, MS/MS MIM Metabolism > Lipid metabolism > Phospholipid metabolism cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, adipose, smallintestine, largeintestine, stomach, placenta, testis

NDUFB4 NADH:ubiquinone oxidoreductase subunit B4 B15, CI-B15, O95168, gene:4710 10 literature, mito protein domain+, MS/MS++ MIM OXPHOS > Complex I > CI subunits;
OXPHOS > OXPHOS subunits all 14

UQCR11 ubiquinol-cytochrome c reductase, complex III subunit XI 0710008D09Rik, QCR10, UQCR, O14957, gene:10975 10 literature, mito protein domain+, MS/MS++ MIM OXPHOS > Complex III > CIII subunits;
OXPHOS > OXPHOS subunits cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, heart, adipose, smallintestine, stomach, placenta, testis

FAM136A family with sequence similarity 136 member A Q96C01, gene:84908 10 APEX_IMS, mito protein domain+, MS/MS++ IMS none all 14

TOMM5 translocase of outer mitochondrial membrane 5 C9orf105, Tom5, bA613M10.3, Q8N4H5, gene:401505 10 literature, mito protein domain+, MS/MS++ MOM Protein import, sorting and homeostasis > Protein import and sorting > TOM cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis

HTRA2 HtrA serine peptidase 2 MGCA8, OMI, PARK13, PRSS25, O43464, gene:27429 10 literature, APEX_IMS, targetP signal, Rickettsial homolog, mito protein domain, MS/MS+ MIM Protein import, sorting and homeostasis > Protein homeostasis > Proteases;
Mitochondrial dynamics and surveillance > Apoptosis cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis

NDUFA1 NADH:ubiquinone oxidoreductase subunit A1 CI-MWFE, MC1DN12, MWFE, ZNF183, O15239, gene:4694 10 literature, coexpression+, MS/MS++ MIM OXPHOS > Complex I > CI subunits;
OXPHOS > OXPHOS subunits all 14

MT-CYB cytochrome b MTCYB, P00156, gene:4519 10 literature, yeast mito homolog++, Rickettsial homolog, mito protein domain MIM OXPHOS > Complex III > CIII subunits;
Metabolism > Metals and cofactors > Heme-containing proteins;
Metabolism > Electron carriers > Cytochromes;
OXPHOS > OXPHOS subunits all 14

GUK1 guanylate kinase 1 GMK, Q16774, gene:2987 10 APEX_matrix, targetP signal, Rickettsial homolog, induction, coexpression, MS/MS Matrix Metabolism > Nucleotide metabolism > Nucleotide synthesis and processing cerebrum, cerebellum, brainstem, spinalcord, adipose, largeintestine, stomach

D2HGDH D-2-hydroxyglutarate dehydrogenase D2HGD, Q8N465, gene:728294 10 literature, APEX_matrix, targetP signal, yeast mito homolog++, mito protein domain Matrix Metabolism > Carbohydrate metabolism > TCA-associated adipose, largeintestine

OPA3 outer mitochondrial membrane lipid metabolism regulator OPA3 MGA3, Q9H6K4, gene:80207 10 literature, targetP signal, coexpression, MS/MS++ MOM none all 14

AKR1B10 aldo-keto reductase family 1 member B10 AKR1B11, AKR1B12, ALDRLn, ARL-1, ARL1, HIS, HSI, O60218, gene:57016 10 induction, coexpression, MS/MS++ unknown Metabolism > Vitamin metabolism > Vitamin A metabolism;
Metabolism > Detoxification > Xenobiotic metabolism cerebrum, brainstem, spinalcord, kidney, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis

PHYH phytanoyl-CoA 2-hydroxylase LN1, LNAP1, PAHX, PHYH1, RD, O14832, gene:5264 10 targetP signal, induction, MS/MS++ Matrix Metabolism > Lipid metabolism kidney, liver, smallintestine

NDUFAF2 NADH:ubiquinone oxidoreductase complex assembly factor 2 B17.2L, MC1DN10, MMTN, NDUFA12L, mimitin, Q8N183, gene:91942 10 literature, APEX_matrix, induction, coexpression, MS/MS++ MIM OXPHOS > Complex I > CI assembly factors;
OXPHOS > OXPHOS assembly factors all 14

ECSIT ECSIT signaling integrator SITPEC, Q9BQ95, gene:51295 10 literature, APEX_matrix, targetP signal, mito protein domain, induction, coexpression+ MIM OXPHOS > Complex I > CI assembly factors;
OXPHOS > OXPHOS assembly factors brainstem, spinalcord, kidney, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis

PARL presenilin associated rhomboid like PRO2207, PSARL, PSARL1, PSENIP2, RHBDS1, Q9H300, gene:55486 10 literature, yeast mito homolog++, mito protein domain, MS/MS MIM Protein import, sorting and homeostasis > Protein homeostasis > Proteases;
Mitochondrial dynamics and surveillance > Mitophagy;
Mitochondrial dynamics and surveillance > Autophagy cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, heart, adipose, smallintestine, largeintestine, stomach, placenta, testis

MRPL42 mitochondrial ribosomal protein L42 HSPC204, L31MT, L42MT, MRP-L31, MRP-L42, MRP-S32, MRPL31, MRPS32, PTD007, RPML31, S32MT, Q9Y6G3, gene:28977 10 APEX_matrix, GFP, mito protein domain+, induction, MS/MS Matrix Mitochondrial central dogma > Translation > Mitochondrial ribosome liver, adipose, largeintestine, testis

DNAJC4 DnaJ heat shock protein family (Hsp40) member C4 DANJC4, HSPF2, MCG18, Q9NNZ3, gene:3338 10 GFP, targetP signal+, Rickettsial homolog, mito protein domain, coexpression Membrane Protein import, sorting and homeostasis > Protein homeostasis largeintestine

PAM16 presequence translocase associated motor 16 CGI-136, MAGMAS, SMDMDM, TIM16, TIMM16, Q9Y3D7, gene:51025 10 literature, APEX_matrix, yeast mito homolog, MS/MS++ MIM Protein import, sorting and homeostasis > Protein import and sorting > Import motor all 14

ALDH3A2 aldehyde dehydrogenase 3 family member A2 ALDH10, FALDH, SLS, P51648, gene:224 10 targetP signal+, mito protein domain, coexpression Membrane Metabolism > Detoxification > ROS and glutathione metabolism spinalcord, kidney, liver, smallintestine, largeintestine, stomach, placenta, testis

ACSL6 acyl-CoA synthetase long chain family member 6 ACS2, FACL6, LACS 6, LACS2, LACS5, Q9UKU0, gene:23305 10 Rickettsial homolog, mito protein domain, MS/MS++ MOM Metabolism > Lipid metabolism > Fatty acid oxidation cerebrum, cerebellum, brainstem, spinalcord, liver, heart, skeletalmuscle, adipose, largeintestine, stomach, testis

MRS2 magnesium transporter MRS2 HPT, MRS2L, Q9HD23, gene:57380 10 APEX_matrix, GFP, targetP signal, yeast mito homolog++, coexpression MIM Small molecule transport;
Signaling cerebrum, cerebellum, brainstem, spinalcord, skeletalmuscle, smallintestine, largeintestine, stomach, placenta, testis

ABHD10 abhydrolase domain containing 10, depalmitoylase Q9NUJ1, gene:55347 9 literature, APEX_IMS, APEX_matrix, Rickettsial homolog, mito protein domain, MS/MS++ Matrix Metabolism > Detoxification > Xenobiotic metabolism cerebrum, cerebellum, brainstem, spinalcord, liver, adipose, smallintestine, largeintestine, stomach, placenta, testis

HINT3 histidine triad nucleotide binding protein 3 HINT4, Q9NQE9, gene:135114 9 Rickettsial homolog, mito protein domain, induction, coexpression+ unknown none brainstem, heart, skeletalmuscle

PXMP4 peroxisomal membrane protein 4 PMP24, Q9Y6I8, gene:11264 9 targetP signal, mito protein domain+, coexpression unknown none kidney, liver, adipose

OSGEPL1 O-sialoglycoprotein endopeptidase like 1 OSGEPL, Qri7, Q9H4B0, gene:64172 9 APEX_matrix, targetP signal, yeast mito homolog, Rickettsial homolog, mito protein domain, coexpression Matrix Mitochondrial central dogma > mtRNA metabolism > mt-tRNA modifications none

NUDT2 nudix hydrolase 2 APAH1, P50583, gene:318 9 mito protein domain, coexpression, MS/MS++ Matrix Metabolism > Nucleotide metabolism > Nucleotide synthesis and processing all 14

CYB5B cytochrome b5 type B CYB5-M, CYPB5M, OMB5, O43169, gene:80777 9 literature, mito protein domain, coexpression, MS/MS++ MOM Metabolism > Metals and cofactors > Heme-containing proteins;
Metabolism > Detoxification > Xenobiotic metabolism;
Metabolism > Detoxification > Amidoxime reducing complex;
Metabolism > Electron carriers > Cytochromes all 14

GRSF1 G-rich RNA sequence binding factor 1 Q12849, gene:2926 9 APEX_matrix, mito protein domain, induction, coexpression, MS/MS Matrix 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 adipose, smallintestine, largeintestine, stomach, placenta, testis

MRPS18B mitochondrial ribosomal protein S18B C6orf14, HSPC183, HumanS18a, MRP-S18-2, MRPS18-2, PTD017, S18amt, Q9Y676, gene:28973 9 literature, APEX_matrix, targetP signal+, Rickettsial homolog, mito protein domain, MS/MS Matrix Mitochondrial central dogma > Translation > Mitochondrial ribosome cerebrum, cerebellum, brainstem, spinalcord, liver, heart, adipose, smallintestine, largeintestine, placenta, testis

DNAJC30 DnaJ heat shock protein family (Hsp40) member C30 WBSCR18, Q96LL9, gene:84277 9 APEX_matrix, GFP, targetP signal+, Rickettsial homolog, mito protein domain, MS/MS MIM none cerebrum, cerebellum, brainstem, spinalcord, heart, skeletalmuscle, adipose, largeintestine, stomach

CAT catalase P04040, gene:847 9 yeast mito homolog++, mito protein domain, coexpression IMS Metabolism > Detoxification > ROS and glutathione metabolism spinalcord, kidney, liver, heart, adipose, smallintestine, largeintestine, stomach, placenta, testis

DGUOK deoxyguanosine kinase MTDPS3, NCPH, PEOB4, dGK, Q16854, gene:1716 9 literature, APEX_matrix, targetP signal, mito protein domain, MS/MS++ Matrix Metabolism > Nucleotide metabolism > Nucleotide synthesis and processing cerebrum, cerebellum, brainstem, spinalcord, liver, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis

ACOT9 acyl-CoA thioesterase 9 ACATE2, CGI-16, MT-ACT48, MTACT48, Q9Y305, gene:23597 9 literature, targetP signal, mito protein domain, MS/MS++ MIM Metabolism > Lipid metabolism cerebrum, cerebellum, brainstem, spinalcord, kidney, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis

ACSS1 acyl-CoA synthetase short chain family member 1 ACAS2L, ACECS1, AceCS2L, Q9NUB1, gene:84532 9 literature, APEX_matrix, targetP signal, mito protein domain, MS/MS++ MIM Metabolism > Lipid metabolism > Fatty acid oxidation cerebrum, cerebellum, brainstem, spinalcord, kidney, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis

RFK riboflavin kinase RIFK, Q969G6, gene:55312 9 targetP signal, yeast mito homolog++, MS/MS Matrix Metabolism > Vitamin metabolism > Vitamin B2 metabolism brainstem, spinalcord, kidney, adipose, smallintestine, largeintestine

STARD7 StAR related lipid transfer domain containing 7 FAME2, GTT1, Q9NQZ5, gene:56910 9 APEX_IMS, targetP signal, mito protein domain, induction, coexpression+ IMS Metabolism > Lipid metabolism > Phospholipid metabolism;
Small molecule transport none

CHPT1 choline phosphotransferase 1 CPT, CPT1, Q8WUD6, gene:56994 9 targetP signal, mito protein domain, induction, coexpression+ MIM Metabolism > Lipid metabolism > Phospholipid metabolism none

MT-ATP8 ATP synthase F0 subunit 8 ATPase8, MTATP8, P03928, gene:4509 9 literature, mito protein domain+, MS/MS++ MIM OXPHOS > Complex V > CV subunits;
OXPHOS > OXPHOS subunits all 14

TIMMDC1 translocase of inner mitochondrial membrane domain containing 1 C3orf1, MC1DN31, Q9NPL8, gene:51300 9 literature, APEX_IMS, APEX_matrix, mito protein domain+, MS/MS++ MIM OXPHOS > Complex I > CI assembly factors;
OXPHOS > OXPHOS assembly factors cerebrum, cerebellum, brainstem, spinalcord, liver, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis

SPATA19 spermatogenesis associated 19 CT132, SPAS1, spergen1, Q7Z5L4, gene:219938 9 mito protein domain+, MS/MS++ unknown none testis

HTATIP2 HIV-1 Tat interactive protein 2 CC3, SDR44U1, TIP30, Q9BUP3, gene:10553 9 yeast mito homolog, mito protein domain+, MS/MS Membrane none cerebrum, cerebellum, brainstem, liver, adipose, placenta, testis

AKR7A2 aldo-keto reductase family 7 member A2 AFAR, AFAR1, AFB1-AR1, AKR7, O43488, gene:8574 9 APEX_matrix, targetP signal, coexpression, MS/MS++ Matrix Metabolism > Amino acid metabolism > GABA metabolism;
Metabolism > Detoxification > Xenobiotic metabolism all 14

DUT deoxyuridine triphosphatase dUTPase, P33316, gene:1854 9 literature, targetP signal, Rickettsial homolog, MS/MS++ Matrix Metabolism > Nucleotide metabolism > Nucleotide synthesis and processing cerebrum, spinalcord, liver, heart, adipose, largeintestine, stomach, placenta

MT-CO3 cytochrome c oxidase III COIII, MTCO3, P00414, gene:4514 9 literature, yeast mito homolog++, Rickettsial homolog, mito protein domain MIM OXPHOS > Complex IV > CIV subunits;
OXPHOS > OXPHOS subunits all 14

DHRS7B dehydrogenase/reductase 7B CGI-93, SDR32C1, Q6IAN0, gene:25979 9 Rickettsial homolog, mito protein domain, induction, coexpression Membrane none cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, adipose, smallintestine, largeintestine, stomach, placenta, testis

MTRF1 mitochondrial translation release factor 1 MRF1, MTTRF1, RF1, O75570, gene:9617 9 literature, APEX_matrix, targetP signal, yeast mito homolog+, Rickettsial homolog, mito protein domain Matrix Mitochondrial central dogma > Translation > Translation factors adipose

TMEM143 transmembrane protein 143 Q96AN5, gene:55260 9 targetP signal, coexpression+, MS/MS MIM none cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, heart, skeletalmuscle, adipose, largeintestine, stomach, placenta

NT5M 5',3'-nucleotidase, mitochondrial dNT-2, dNT2, mdN, Q9NPB1, gene:56953 9 literature, targetP signal, mito protein domain, induction, MS/MS Matrix Metabolism > Nucleotide metabolism > Nucleotide synthesis and processing cerebrum, cerebellum, brainstem, spinalcord, skeletalmuscle, adipose, testis

RHOT1 ras homolog family member T1 ARHT1, MIRO-1, MIRO1, Q8IXI2, gene:55288 9 literature, mito protein domain+, MS/MS+ MOM Signaling > Calcium homeostasis > EF hand proteins;
Mitochondrial dynamics and surveillance > Trafficking all 14

NME4 NME/NM23 nucleoside diphosphate kinase 4 NDPK-D, NM23H4, nm23-H4, O00746, gene:4833 9 APEX_matrix, GFP, targetP signal, Rickettsial homolog, mito protein domain, MS/MS MIM Metabolism > Nucleotide metabolism > Nucleotide synthesis and processing adipose, largeintestine, placenta, testis

MTHFS methenyltetrahydrofolate synthetase HsT19268, NEDMEHM, P49914, gene:10588 9 targetP signal, Rickettsial homolog, mito protein domain, MS/MS Matrix Metabolism > Vitamin metabolism > Folate and 1-C metabolism cerebrum, kidney, liver, adipose, smallintestine, largeintestine, stomach, placenta

TIMM29 translocase of inner mitochondrial membrane 29 C19orf52, TIM29, Q9BSF4, gene:90580 9 targetP signal+, coexpression, MS/MS MIM Protein import, sorting and homeostasis > Protein import and sorting > TIM22 carrier pathway cerebrum, cerebellum, brainstem, spinalcord, liver, heart, adipose, largeintestine, placenta, testis

PNPO pyridoxamine 5'-phosphate oxidase HEL-S-302, PDXPO, Q9NVS9, gene:55163 9 APEX_matrix, yeast mito homolog++, coexpression, MS/MS Matrix Metabolism > Vitamin metabolism > Vitamin B6 metabolism brainstem, liver

MICU2 mitochondrial calcium uptake 2 1110008L20Rik, EFHA1, Q8IYU8, gene:221154 9 literature, APEX_IMS, GFP, targetP signal+, mito protein domain, MS/MS+ MIM Signaling > Calcium homeostasis > Calcium cycle;
Signaling > Calcium homeostasis > EF hand proteins;
Small molecule transport > Calcium uniporter smallintestine, largeintestine, placenta, testis

PCBD2 pterin-4 alpha-carbinolamine dehydratase 2 DCOH2, DCOHM, PHS2, Q9H0N5, gene:84105 9 targetP signal+, induction, MS/MS unknown Metabolism > Metals and cofactors > Tetrahydrobiopterin synthesis cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, adipose, smallintestine, largeintestine, stomach, placenta, testis

QRSL1 glutaminyl-tRNA amidotransferase subunit QRSL1 COXPD40, GatA, Q9H0R6, gene:55278 9 literature, APEX_matrix, GFP, yeast mito homolog, Rickettsial homolog, mito protein domain, coexpression, MS/MS Matrix Mitochondrial central dogma > Translation > mt-tRNA synthetases brainstem, heart, skeletalmuscle, adipose, largeintestine, stomach, placenta, testis

ADCK1 aarF domain containing kinase 1 Q86TW2, gene:57143 9 yeast mito homolog+, Rickettsial homolog, mito protein domain, MS/MS unknown none cerebrum, spinalcord, kidney, heart, skeletalmuscle, adipose, stomach, placenta

PRDX6 peroxiredoxin 6 1-Cys, AOP2, HEL-S-128m, NSGPx, PRX, aiPLA2, p29, P30041, gene:9588 9 yeast mito homolog++, Rickettsial homolog, mito protein domain unknown Metabolism > Lipid metabolism > Phospholipid metabolism;
Metabolism > Detoxification > ROS and glutathione metabolism cerebrum, cerebellum, brainstem, spinalcord, liver, adipose, smallintestine, largeintestine, placenta, testis

NIPSNAP1 nipsnap homolog 1 Q9BPW8, gene:8508 9 APEX_matrix, targetP signal, coexpression, MS/MS++ Matrix Mitochondrial dynamics and surveillance > Mitophagy;
Mitochondrial dynamics and surveillance > Autophagy all 14

GARS1 glycyl-tRNA synthetase 1 CMT2D, DSMAV, GARS, GlyRS, HMN5, SMAD1, P41250, gene:2617 9 targetP signal, yeast mito homolog, mito protein domain, induction Matrix Mitochondrial central dogma > Translation > mt-tRNA synthetases cerebrum, cerebellum, brainstem, spinalcord, liver, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis

ACSM2A acyl-CoA synthetase medium chain family member 2A A-923A4.1, ACSM2, Q08AH3, gene:123876 9 targetP signal, mito protein domain, MS/MS+ Matrix Metabolism > Lipid metabolism > Fatty acid oxidation;
Metabolism > Detoxification > Xenobiotic metabolism kidney, liver, heart

SLC25A33 solute carrier family 25 member 33 BMSC-MCP, PNC1, Q9BSK2, gene:84275 9 literature, yeast mito homolog+, mito protein domain+ MIM Metabolism > Nucleotide metabolism > Nucleotide import;
Small molecule transport > SLC25A family brainstem, spinalcord, placenta, testis

LYRM9 LYR motif containing 9 C17orf108, HSD24, A8MSI8, gene:201229 9 targetP signal, mito protein domain+, MS/MS unknown none cerebellum, heart, adipose, testis

RAB24 RAB24, member RAS oncogene family Q969Q5, gene:53917 9 mito protein domain, induction, coexpression, MS/MS MOM Mitochondrial dynamics and surveillance > Fission;
Mitochondrial dynamics and surveillance > Autophagy cerebrum, cerebellum, brainstem, spinalcord, skeletalmuscle, adipose, stomach, testis

PARS2 prolyl-tRNA synthetase 2, mitochondrial EIEE75, MT-PRORS, proRS, Q7L3T8, gene:25973 8 APEX_matrix, GFP, targetP signal, yeast mito homolog, Rickettsial homolog, mito protein domain Matrix Mitochondrial central dogma > Translation > mt-tRNA synthetases smallintestine, largeintestine, stomach

MRPL52 mitochondrial ribosomal protein L52 Q86TS9, gene:122704 8 literature, APEX_matrix, targetP signal, coexpression, MS/MS Matrix Mitochondrial central dogma > Translation > Mitochondrial ribosome cerebrum, spinalcord, liver, heart, adipose, smallintestine, largeintestine, stomach, placenta, testis

PUS1 pseudouridine synthase 1 MLASA1, Q9Y606, gene:80324 8 APEX_matrix, targetP signal, yeast mito homolog+, mito protein domain, coexpression Matrix Mitochondrial central dogma > mtRNA metabolism > mtRNA granules;
Mitochondrial central dogma > mtRNA metabolism > mt-tRNA modifications none

GOT2 glutamic-oxaloacetic transaminase 2 KAT4, KATIV, KYAT4, mitAAT, P00505, gene:2806 8 literature, APEX_IMS, APEX_matrix, targetP signal, mito protein domain, MS/MS++ MIM Metabolism > Carbohydrate metabolism > Malate-aspartate shuttle;
Metabolism > Amino acid metabolism > Glutamate metabolism;
Metabolism > Amino acid metabolism > Glyoxylate metabolism;
Metabolism > Amino acid metabolism > Kynurenine metabolism;
Metabolism > Sulfur metabolism all 14

NAXD NAD(P)HX dehydratase CARKD, LP3298, PEBEL2, Q8IW45, gene:55739 8 GFP, targetP signal, mito protein domain, MS/MS++ MIM Metabolism > Metals and cofactors > NAD biosynthesis and metabolism all 14

MT-ND3 NADH dehydrogenase, subunit 3 (complex I) MTND3, P03897, gene:4537 8 literature, Rickettsial homolog, mito protein domain+, MS/MS MIM OXPHOS > Complex I > CI subunits;
OXPHOS > OXPHOS subunits cerebrum, cerebellum, brainstem, spinalcord, kidney, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta

ACCS 1-aminocyclopropane-1-carboxylate synthase homolog (inactive) ACS, PHACS, Q96QU6, gene:84680 8 yeast mito homolog++, Rickettsial homolog, mito protein domain, coexpression unknown none none

PTRH2 peptidyl-tRNA hydrolase 2 BIT1, CFAP37, CGI-147, IMNEPD, PTH, PTH 2, PTH2, Q9Y3E5, gene:51651 8 literature, yeast mito homolog, mito protein domain, MS/MS++ MOM none all 14

PGS1 phosphatidylglycerophosphate synthase 1 Q32NB8, gene:9489 8 targetP signal, yeast mito homolog++, mito protein domain MIM Metabolism > Lipid metabolism > Cardiolipin synthesis;
Metabolism > Lipid metabolism > Phospholipid metabolism cerebrum, spinalcord, placenta

OXR1 oxidation resistance 1 CHEGDD, Nbla00307, TLDC3, Q8N573, gene:55074 8 literature, yeast mito homolog+, mito protein domain, MS/MS MOM Metabolism > Detoxification > ROS and glutathione metabolism cerebrum, cerebellum, brainstem

LYPLAL1 lysophospholipase like 1 Q96AV0, Q5VWZ2, gene:127018 8 mito protein domain, MS/MS++ unknown Metabolism > Lipid metabolism cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, adipose, largeintestine, placenta, testis

DNAJC28 DnaJ heat shock protein family (Hsp40) member C28 C21orf55, C21orf78, Q9NX36, gene:54943 8 Rickettsial homolog, mito protein domain, coexpression+ unknown none stomach

FPGS folylpolyglutamate synthase Q05932, gene:2356 8 APEX_matrix, GFP, targetP signal, yeast mito homolog, Rickettsial homolog, mito protein domain Matrix Metabolism > Vitamin metabolism > Folate and 1-C metabolism none

TMEM205 transmembrane protein 205 UNQ501, Q6UW68, gene:374882 8 yeast mito homolog, mito protein domain+, coexpression Membrane none liver, adipose

SLC25A53 solute carrier family 25 member 53 MCART6, Q5H9E4, gene:401612 8 yeast mito homolog+, mito protein domain+ MIM Small molecule transport > SLC25A family testis

AIFM3 apoptosis inducing factor mitochondria associated 3 AIFL, Q96NN9, gene:150209 8 literature, yeast mito homolog, Rickettsial homolog, mito protein domain+ MIM Metabolism > Metals and cofactors > Fe-S-containing proteins;
Mitochondrial dynamics and surveillance > Apoptosis none

MGST1 microsomal glutathione S-transferase 1 GST12, MGST, MGST-I, P10620, gene:4257 8 mito protein domain, coexpression, MS/MS++ Membrane Metabolism > Detoxification > Xenobiotic metabolism;
Metabolism > Detoxification > ROS and glutathione metabolism cerebrum, brainstem, spinalcord, liver, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis

MTFP1 mitochondrial fission process 1 HSPC242, MTP18, Q9UDX5, gene:51537 8 literature, induction, coexpression, MS/MS+ Membrane Mitochondrial dynamics and surveillance > Fission all 14

COA4 cytochrome c oxidase assembly factor 4 homolog CHCHD8, CMC3, E2IG2, Q9NYJ1, gene:51287 8 yeast mito homolog++, mito protein domain, MS/MS IMS OXPHOS > Complex IV > CIV assembly factors;
OXPHOS > OXPHOS assembly factors placenta

NIF3L1 NGG1 interacting factor 3 like 1 ALS2CR1, CALS-7, MDS015, Q9GZT8, gene:60491 8 APEX_matrix, targetP signal, yeast mito homolog, coexpression, MS/MS+ Matrix none cerebrum, cerebellum, brainstem, spinalcord, skeletalmuscle, adipose, largeintestine, placenta, testis

PUSL1 pseudouridine synthase like 1 Q8N0Z8, gene:126789 8 APEX_matrix, yeast mito homolog+, Rickettsial homolog, mito protein domain Matrix Mitochondrial central dogma > Translation cerebrum, spinalcord, largeintestine, placenta

SDSL serine dehydratase like SDH 2, SDS-RS1, TDH, cSDH, Q96GA7, gene:113675 8 yeast mito homolog+, Rickettsial homolog, mito protein domain, coexpression unknown Metabolism > Amino acid metabolism > Serine metabolism none

BAX BCL2 associated X, apoptosis regulator BCL2L4, Q07812, gene:581 8 literature, mito protein domain, coexpression, MS/MS++ MOM Mitochondrial dynamics and surveillance > Apoptosis cerebrum, cerebellum, brainstem, adipose, largeintestine, placenta, testis

MAVS mitochondrial antiviral signaling protein CARDIF, IPS-1, IPS1, VISA, Q7Z434, gene:57506 8 literature, induction, coexpression+, MS/MS MOM Signaling > Immune response brainstem, skeletalmuscle, adipose, placenta, testis

METTL17 methyltransferase like 17 METT11D1, Q9H7H0, gene:64745 8 GFP, yeast mito homolog++, mito protein domain+ Matrix Mitochondrial central dogma > mtRNA metabolism > mt-rRNA modifications;
Mitochondrial central dogma > Translation > Mitochondrial ribosome assembly none

ACACB acetyl-CoA carboxylase beta ACC2, ACCB, HACC275, O00763, gene:32 8 Rickettsial homolog, mito protein domain, coexpression+ MOM Metabolism > Lipid metabolism > Fatty acid oxidation;
Metabolism > Vitamin metabolism > Biotin utilizing proteins cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, adipose, largeintestine, stomach, placenta, testis

ARG2 arginase 2 P78540, gene:384 8 literature, APEX_matrix, targetP signal+, mito protein domain, MS/MS Matrix Metabolism > Amino acid metabolism kidney, adipose, smallintestine, largeintestine, placenta

MTPAP mitochondrial poly(A) polymerase PAPD1, SPAX4, TENT6, Q9NVV4, gene:55149 7 literature, APEX_matrix, targetP signal+, mito protein domain, induction Matrix 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 cerebrum, cerebellum, spinalcord, liver, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis

CCDC51 coiled-coil domain containing 51 MITOK, Q96ER9, gene:79714 7 APEX_matrix, targetP signal, MS/MS++ MIM Small molecule transport all 14

SUGCT succinyl-CoA:glutarate-CoA transferase C7orf10, DERP13, GA3, ORF19, Q9HAC7, gene:79783 7 APEX_matrix, targetP signal, mito protein domain, coexpression, MS/MS+ Matrix Metabolism > Amino acid metabolism kidney, liver, testis

DNM1L dynamin 1 like DLP1, DRP1, DVLP, DYMPLE, EMPF, EMPF1, HDYNIV, OPA5, O00429, gene:10059 7 literature, yeast mito homolog+, mito protein domain, induction MOM Mitochondrial dynamics and surveillance > Fission cerebrum, cerebellum, brainstem, spinalcord

NRDC nardilysin convertase NRD1, hNRD1, hNRD2, O43847, gene:4898 7 targetP signal, Rickettsial homolog, mito protein domain+ Matrix Protein import, sorting and homeostasis > Protein homeostasis > Chaperones;
Protein import, sorting and homeostasis > Protein import and sorting testis

TMEM126B transmembrane protein 126B HT007, MC1DN29, Q8IUX1, gene:55863 7 targetP signal, mito protein domain+, MS/MS MIM OXPHOS > Complex I > CI assembly factors;
OXPHOS > OXPHOS assembly factors cerebrum, cerebellum, brainstem, spinalcord, liver, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis

NSUN4 NOP2/Sun RNA methyltransferase 4 SHTAP, Q96CB9, gene:387338 7 APEX_matrix, targetP signal, mito protein domain, induction, coexpression Matrix Mitochondrial central dogma > mtRNA metabolism > mt-rRNA modifications;
Mitochondrial central dogma > Translation > Mitochondrial ribosome assembly placenta

ACSS3 acyl-CoA synthetase short chain family member 3 Q9H6R3, gene:79611 7 Rickettsial homolog, mito protein domain, MS/MS Matrix Metabolism > Lipid metabolism kidney, liver, adipose, placenta, testis

FAM210B family with sequence similarity 210 member B 5A3, C20orf108, dJ1167H4.1, Q96KR6, gene:116151 7 targetP signal, yeast mito homolog, coexpression, MS/MS MIM Metabolism > Metals and cofactors > Iron homeostasis cerebrum, cerebellum, brainstem, spinalcord, liver, adipose, smallintestine, largeintestine, stomach, placenta, testis

RBFA ribosome binding factor A C18orf22, HsT169, Q8N0V3, gene:79863 7 APEX_matrix, targetP signal+, Rickettsial homolog, coexpression Matrix Mitochondrial central dogma > Translation > Mitochondrial ribosome assembly none

METAP1D methionyl aminopeptidase type 1D, mitochondrial MAP 1D, MAP1D, MetAP 1D, Metap1l, Q6UB28, gene:254042 7 literature, APEX_matrix, targetP signal, Rickettsial homolog, mito protein domain, coexpression Matrix Mitochondrial central dogma > Translation > fMet processing;
Protein import, sorting and homeostasis > Protein homeostasis > Proteases none

AHCYL1 adenosylhomocysteinase like 1 DCAL, IRBIT, PPP1R78, PRO0233, XPVKONA, O43865, gene:10768 7 targetP signal, mito protein domain+, induction MOM Mitochondrial dynamics and surveillance > Organelle contact sites brainstem, spinalcord, smallintestine, stomach

NIPSNAP3A nipsnap homolog 3A HSPC299, NIPSNAP4, TASSC, Q9UFN0, gene:25934 7 APEX_matrix, targetP signal+, MS/MS++ Matrix none cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, adipose, smallintestine, largeintestine, stomach, placenta, testis

MTARC1 mitochondrial amidoxime reducing component 1 MARC1, MOSC1, Q5VT66, gene:64757 7 mito protein domain+, MS/MS MOM Metabolism > Metals and cofactors > Molybdenum cofactor synthesis and proteins;
Metabolism > Detoxification > Xenobiotic metabolism;
Metabolism > Detoxification > Amidoxime reducing complex cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis

FUNDC1 FUN14 domain containing 1 Q8IVP5, gene:139341 7 yeast mito homolog, mito protein domain+, MS/MS MOM Mitochondrial dynamics and surveillance > Mitophagy;
Mitochondrial dynamics and surveillance > Autophagy cerebrum, cerebellum, brainstem, spinalcord, kidney, skeletalmuscle, adipose, largeintestine, stomach, testis

ACSL1 acyl-CoA synthetase long chain family member 1 ACS1, FACL1, FACL2, LACS, LACS1, LACS2, P33121, gene:2180 7 Rickettsial homolog, mito protein domain, coexpression MOM Metabolism > Lipid metabolism > Fatty acid oxidation all 14

COX20 cytochrome c oxidase assembly factor COX20 FAM36A, Q5RI15, gene:116228 7 literature, APEX_IMS, GFP, yeast mito homolog++, MS/MS MIM OXPHOS > Complex IV > CIV assembly factors;
OXPHOS > OXPHOS assembly factors all 14

HSDL1 hydroxysteroid dehydrogenase like 1 SDR12C3, Q3SXM5, gene:83693 7 GFP, Rickettsial homolog, mito protein domain, MS/MS++ unknown none cerebrum, cerebellum, brainstem, spinalcord, adipose, smallintestine, largeintestine, stomach, placenta, testis

CBR3 carbonyl reductase 3 HEL-S-25, SDR21C2, hCBR3, O75828, gene:874 7 targetP signal, Rickettsial homolog, mito protein domain, MS/MS Matrix Metabolism > Detoxification > Xenobiotic metabolism cerebrum, spinalcord, adipose, smallintestine, largeintestine, stomach

MTERF4 mitochondrial transcription termination factor 4 MTERFD2, Q7Z6M4, gene:130916 7 APEX_matrix, targetP signal, mito protein domain+, coexpression Matrix Mitochondrial central dogma > Translation > Mitochondrial ribosome assembly none

NUDT19 nudix hydrolase 19 RP2, A8MXV4, gene:390916 7 APEX_matrix, targetP signal, mito protein domain, induction Matrix Metabolism > Metals and cofactors > Coenzyme A metabolism kidney, liver, largeintestine, placenta

HEBP1 heme binding protein 1 HBP, HEBP, Q9NRV9, gene:50865 7 APEX_IMS, mito protein domain, coexpression, MS/MS++ IMS none cerebrum, cerebellum, liver, adipose, placenta

PTPMT1 protein tyrosine phosphatase mitochondrial 1 DUSP23, MOSP, PLIP, PNAS-129, Q8WUK0, gene:114971 7 literature, APEX_matrix, mito protein domain, coexpression, MS/MS++ MIM Metabolism > Lipid metabolism > Cardiolipin synthesis;
Metabolism > Lipid metabolism > Phospholipid metabolism all 14

PGAM5 PGAM family member 5, mitochondrial serine/threonine protein phosphatase BXLBV68, Q96HS1, gene:192111 7 APEX_IMS, mito protein domain, coexpression, MS/MS++ MOM Mitochondrial dynamics and surveillance > Mitophagy;
Mitochondrial dynamics and surveillance > Autophagy cerebrum, cerebellum, brainstem, spinalcord, skeletalmuscle, adipose, largeintestine, placenta, testis

DTYMK deoxythymidylate kinase CDC8, PP3731, TMPK, TYMK, P23919, gene:1841 7 Rickettsial homolog, MS/MS+ Matrix Metabolism > Nucleotide metabolism > Nucleotide synthesis and processing cerebrum, cerebellum, brainstem, spinalcord, liver, heart, adipose, smallintestine, largeintestine, stomach, placenta, testis

KARS1 lysyl-tRNA synthetase 1 CMTRIB, DFNB89, KARS, KARS2, KRS, Q15046, gene:3735 7 literature, GFP, targetP signal, Rickettsial homolog, mito protein domain, coexpression Matrix Mitochondrial central dogma > Translation > mt-tRNA synthetases cerebrum, cerebellum, brainstem, spinalcord, skeletalmuscle, adipose, largeintestine, stomach, placenta, testis

STAR steroidogenic acute regulatory protein STARD1, P49675, gene:6770 7 literature, targetP signal, mito protein domain, coexpression, MS/MS MOM, IMS Metabolism > Lipid metabolism > Cholesterol, bile acid, steroid synthesis testis

DNAJC19 DnaJ heat shock protein family (Hsp40) member C19 PAM18, TIM14, TIMM14, Q96DA6, gene:131118 7 literature, APEX_matrix, mito protein domain, MS/MS++ MIM Protein import, sorting and homeostasis > Protein import and sorting > Import motor all 14

OTC ornithine carbamoyltransferase OCTD, P00480, gene:5009 7 literature, targetP signal, mito protein domain, MS/MS+ Matrix Metabolism > Amino acid metabolism > Urea cycle cerebellum, spinalcord, kidney, liver, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta

COX7A2L cytochrome c oxidase subunit 7A2 like COX7AR, COX7RP, EB1, SIG81, O14548, gene:9167 7 literature, APEX_IMS, APEX_matrix, mito protein domain+, MS/MS+ MIM OXPHOS > Complex IV > CIV subunits;
OXPHOS > OXPHOS subunits;
OXPHOS > OXPHOS assembly factors;
OXPHOS > Respirasome assembly all 14

MTERF3 mitochondrial transcription termination factor 3 CGI-12, MTERFD1, Q96E29, gene:51001 7 literature, APEX_matrix, GFP, targetP signal, mito protein domain+, coexpression Matrix Mitochondrial central dogma > Translation > Mitochondrial ribosome assembly none

SFXN2 sideroflexin 2 SLC56A2, Q96NB2, gene:118980 7 mito protein domain+, coexpression MIM Small molecule transport > Sideroflexins kidney, liver, adipose, largeintestine, stomach, placenta, testis

FASN fatty acid synthase FAS, OA-519, SDR27X1, P49327, gene:2194 7 Rickettsial homolog, mito protein domain, coexpression IMS Metabolism > Lipid metabolism cerebrum, cerebellum, brainstem, spinalcord, liver, skeletalmuscle, adipose, smallintestine, stomach, placenta, testis

PINK1 PTEN induced kinase 1 BRPK, PARK6, Q9BXM7, gene:65018 7 literature, targetP signal+, mito protein domain, coexpression MOM Mitochondrial dynamics and surveillance > Mitophagy;
Mitochondrial dynamics and surveillance > Autophagy;
Signaling none

TMEM177 transmembrane protein 177 Q53S58, gene:80775 7 targetP signal, coexpression, MS/MS MIM OXPHOS > Complex IV > CIV assembly factors;
OXPHOS > OXPHOS assembly factors cerebrum, adipose, stomach, placenta, testis

TXNRD1 thioredoxin reductase 1 GRIM-12, TR, TR1, TRXR1, TXNR, Q16881, gene:7296 6 GFP, Rickettsial homolog, mito protein domain, coexpression, MS/MS unknown Metabolism > Detoxification > ROS and glutathione metabolism;
Metabolism > Detoxification > Selenoproteins skeletalmuscle, smallintestine, placenta, testis

THNSL1 threonine synthase like 1 TSH1, Q8IYQ7, gene:79896 6 APEX_matrix, mito protein domain, MS/MS++ Matrix none cerebrum, cerebellum, brainstem, spinalcord, skeletalmuscle, adipose, largeintestine, stomach, testis

FDPS farnesyl diphosphate synthase FPPS, FPS, POROK9, P14324, gene:2224 6 targetP signal, mito protein domain, MS/MS+ MIM Metabolism > Lipid metabolism > Cholesterol-associated liver, adipose, smallintestine, largeintestine, placenta

IDI1 isopentenyl-diphosphate delta isomerase 1 IPP1, IPPI1, Q13907, gene:3422 6 targetP signal, mito protein domain, MS/MS+ Matrix Metabolism > Lipid metabolism > Cholesterol-associated cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, adipose, smallintestine, largeintestine, stomach, placenta, testis

TOP3A DNA topoisomerase III alpha MGRISCE2, PEOB5, TOP3, ZGRF7, Q13472, gene:7156 6 APEX_matrix, targetP signal+, Rickettsial homolog, mito protein domain Matrix Mitochondrial central dogma > mtDNA maintenance > mtDNA replication testis

FTMT ferritin mitochondrial MTF, Q8N4E7, gene:94033 6 literature, targetP signal+, mito protein domain, MS/MS Matrix Metabolism > Metals and cofactors > Iron homeostasis testis

LDHAL6B lactate dehydrogenase A like 6B LDH6B, LDHAL6, LDHL, Q9BYZ2, gene:92483 6 targetP signal, Rickettsial homolog, mito protein domain, MS/MS Matrix Metabolism > Carbohydrate metabolism cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, adipose, smallintestine, largeintestine, stomach, placenta, testis

MTFR1 mitochondrial fission regulator 1 CHPPR, FAM54A2, Q15390, gene:9650 6 literature, targetP signal, mito protein domain+, coexpression Membrane Mitochondrial dynamics and surveillance > Fission none

BCL2L13 BCL2 like 13 BCL-RAMBO, Bcl2-L-13, MIL1, Q9BXK5, gene:23786 6 literature, mito protein domain, coexpression, MS/MS+ MOM Mitochondrial dynamics and surveillance > Mitophagy;
Mitochondrial dynamics and surveillance > Autophagy;
Mitochondrial dynamics and surveillance > Apoptosis cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, heart, skeletalmuscle, adipose, largeintestine, stomach, placenta, testis

TRMT10C tRNA methyltransferase 10C, mitochondrial RNase P subunit COXPD30, HNYA, MRPP1, RG9MTD1, Q7L0Y3, gene:54931 6 literature, APEX_matrix, GFP, targetP signal+, mito protein domain, coexpression Matrix Mitochondrial central dogma > mtRNA metabolism > mtRNA granules;
Mitochondrial central dogma > mtRNA metabolism > Polycistronic mtRNA processing;
Mitochondrial central dogma > mtRNA metabolism > mt-tRNA modifications largeintestine, placenta

ACOT7 acyl-CoA thioesterase 7 ACH1, ACT, BACH, CTE-II, LACH, LACH1, hBACH, O00154, gene:11332 6 targetP signal+, mito protein domain, induction Matrix Metabolism > Lipid metabolism brainstem, spinalcord, adipose, largeintestine

TFB1M transcription factor B1, mitochondrial CGI-75, CGI75, mtTFB, mtTFB1, Q8WVM0, gene:51106 6 literature, APEX_matrix, Rickettsial homolog, mito protein domain, MS/MS+ Matrix Mitochondrial central dogma > mtRNA metabolism > mtRNA granules;
Mitochondrial central dogma > mtRNA metabolism > mt-rRNA modifications;
Mitochondrial central dogma > Translation > Mitochondrial ribosome assembly brainstem, largeintestine, placenta, testis

ARF5 ADP ribosylation factor 5 P84085, gene:381 6 targetP signal, mito protein domain, coexpression, MS/MS unknown none cerebrum, cerebellum, brainstem, spinalcord, liver, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis

MCUB mitochondrial calcium uniporter dominant negative subunit beta CCDC109B, Q9NWR8, gene:55013 6 literature, targetP signal, mito protein domain+ MIM Signaling > Calcium homeostasis > Calcium cycle;
Small molecule transport > Calcium uniporter placenta

TWNK twinkle mtDNA helicase ATXN8, C10orf2, IOSCA, MTDPS7, PEO, PEO1, PEOA3, PRLTS5, SANDO, SCA8, TWINL, Q96RR1, gene:56652 6 literature, APEX_matrix, targetP signal, induction, coexpression Matrix Mitochondrial central dogma > mtDNA maintenance > mtDNA replication;
Mitochondrial central dogma > mtDNA maintenance > mtDNA nucleoid none

MMADHC metabolism of cobalamin associated D C2orf25, CL25022, cblD, Q9H3L0, gene:27249 6 APEX_matrix, targetP signal, induction, coexpression Matrix Metabolism > Vitamin metabolism > Vitamin B12 metabolism none

PYCR2 pyrroline-5-carboxylate reductase 2 HLD10, P5CR2, Q96C36, gene:29920 6 APEX_matrix, mito protein domain, MS/MS++ Matrix Metabolism > Amino acid metabolism > Proline metabolism cerebrum, cerebellum, brainstem, spinalcord, adipose, largeintestine, stomach, placenta, testis

TSTD1 thiosulfate sulfurtransferase like domain containing 1 KAT, TST, Q8NFU3, gene:100131187 6 Rickettsial homolog, mito protein domain, MS/MS unknown Metabolism > Sulfur metabolism kidney, smallintestine, largeintestine

LYRM1 LYR motif containing 1 A211C6.1, O43325, gene:57149 6 GFP, mito protein domain+, MS/MS unknown none brainstem, testis

OSBPL1A oxysterol binding protein like 1A ORP-1, ORP1, OSBPL1B, Q9BXW6, gene:114876 6 Rickettsial homolog, mito protein domain, induction, MS/MS unknown Metabolism > Lipid metabolism > Cholesterol, bile acid, steroid synthesis;
Metabolism > Lipid metabolism > Phospholipid metabolism cerebrum, liver, largeintestine, placenta

RECQL4 RecQ like helicase 4 RECQ4, O94761, gene:9401 6 targetP signal, Rickettsial homolog, mito protein domain, coexpression Matrix Mitochondrial central dogma > mtDNA maintenance > mtDNA repair none

PET100 PET100 cytochrome c oxidase chaperone C19orf79, P0DJ07, gene:100131801 6 literature, yeast mito homolog++, MS/MS MIM OXPHOS > Complex IV > CIV assembly factors;
OXPHOS > OXPHOS assembly factors cerebrum, spinalcord, kidney, liver, heart, smallintestine, largeintestine, placenta

GDAP1 ganglioside induced differentiation associated protein 1 CMT4, CMT4A, CMTRIA, Q8TB36, gene:54332 6 APEX_IMS, mito protein domain, MS/MS++ MOM none cerebrum, cerebellum, brainstem, spinalcord

MTIF3 mitochondrial translational initiation factor 3 IF3mt, Q9H2K0, gene:219402 6 literature, APEX_matrix, Rickettsial homolog, mito protein domain, coexpression Matrix Mitochondrial central dogma > Translation > Translation factors largeintestine

UNG uracil DNA glycosylase DGU, HIGM4, HIGM5, UDG, UNG1, UNG15, UNG2, P13051, gene:7374 6 literature, targetP signal, yeast mito homolog, mito protein domain, induction Matrix Mitochondrial central dogma > mtDNA maintenance > mtDNA repair none

GLYAT glycine-N-acyltransferase ACGNAT, CAT, GAT, Q6IB77, gene:10249 6 literature, mito protein domain, coexpression, MS/MS+ Matrix Metabolism > Amino acid metabolism > Glycine metabolism;
Metabolism > Detoxification > Xenobiotic metabolism spinalcord, kidney, liver, placenta

ISOC2 isochorismatase domain containing 2 Q96AB3, gene:79763 6 APEX_matrix, targetP signal, MS/MS++ Matrix none all 14

ATP5MC1 ATP synthase membrane subunit c locus 1 ATP5A, ATP5G, ATP5G1, P05496, gene:516 6 literature, yeast mito homolog+, Rickettsial homolog, mito protein domain MIM OXPHOS > Complex V > CV subunits;
OXPHOS > OXPHOS subunits none

QDPR quinoid dihydropteridine reductase DHPR, HDHPR, PKU2, SDR33C1, P09417, gene:5860 6 APEX_matrix, targetP signal, mito protein domain, induction Matrix Metabolism > Metals and cofactors > Tetrahydrobiopterin synthesis;
Metabolism > Vitamin metabolism > Folate and 1-C metabolism cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, adipose, stomach, placenta, testis

COQ8B coenzyme Q8B ADCK4, NPHS9, Q96D53, gene:79934 6 APEX_matrix, GFP, yeast mito homolog+, Rickettsial homolog, mito protein domain MIM Metabolism > Metals and cofactors > Coenzyme Q metabolism adipose, smallintestine, largeintestine, placenta

GTPBP10 GTP binding protein 10 ObgH2, UG0751c10, A4D1E9, gene:85865 6 APEX_matrix, yeast mito homolog+, Rickettsial homolog, mito protein domain Matrix Mitochondrial central dogma > Translation > Mitochondrial ribosome assembly largeintestine, placenta

TSPO translocator protein BPBS, BZRP, DBI, IBP, MBR, PBR, PBS, PKBS, PTBR, mDRC, pk18, P30536, gene:706 6 literature, mito protein domain, MS/MS++ MOM Metabolism > Lipid metabolism > Cholesterol, bile acid, steroid synthesis spinalcord, kidney, liver, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis

FTH1 ferritin heavy chain 1 FHC, FTH, FTHL6, HFE5, PIG15, PLIF, P02794, gene:2495 6 mito protein domain, MS/MS++ unknown Metabolism > Metals and cofactors > Iron homeostasis cerebrum, cerebellum, brainstem, spinalcord, liver, adipose, smallintestine, largeintestine, stomach, placenta

TRMT1 tRNA methyltransferase 1 MRT68, TRM1, Q9NXH9, gene:55621 6 targetP signal+, mito protein domain, coexpression Matrix Mitochondrial central dogma > mtRNA metabolism > mt-tRNA modifications none

ADCK2 aarF domain containing kinase 2 AARF, Q7Z695, gene:90956 6 GFP, targetP signal, yeast mito homolog, Rickettsial homolog, mito protein domain MIM none none

TSTD3 thiosulfate sulfurtransferase like domain containing 3 H0UI37, gene:100130890 6 mito protein domain, induction, MS/MS unknown none cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, adipose, smallintestine, largeintestine, stomach, placenta, testis

MRPS36 mitochondrial ribosomal protein S36 DC47, MRP-S36, P82909, gene:92259 6 literature, APEX_matrix, MS/MS++ Matrix Mitochondrial central dogma > Translation > Mitochondrial ribosome;
Metabolism > Carbohydrate metabolism > TCA cycle all 14

PDP2 pyruvate dehyrogenase phosphatase catalytic subunit 2 PDPC 2, PPM2B, PPM2C2, Q9P2J9, gene:57546 6 APEX_matrix, yeast mito homolog+, mito protein domain, coexpression Matrix Metabolism > Carbohydrate metabolism > Pyruvate metabolism adipose

AGPAT5 1-acylglycerol-3-phosphate O-acyltransferase 5 1AGPAT5, LPAATE, Q9NUQ2, gene:55326 6 literature, Rickettsial homolog, mito protein domain, induction, MS/MS MOM Metabolism > Lipid metabolism > Cardiolipin synthesis;
Metabolism > Lipid metabolism > Phospholipid metabolism cerebellum, brainstem, spinalcord, adipose, smallintestine, largeintestine, placenta, testis

MCRIP2 MAPK regulated corepressor interacting protein 2 C16orf14, FAM195A, c349E10.1, Q9BUT9, gene:84331 5 induction, coexpression+ unknown Signaling none

UCP2 uncoupling protein 2 BMIQ4, SLC25A8, UCPH, P55851, gene:7351 5 literature, yeast mito homolog+, mito protein domain+ MIM Small molecule transport > SLC25A family none

SLC25A37 solute carrier family 25 member 37 HT015, MFRN, MFRN1, MSC, MSCP, PRO1278, PRO1584, PRO2217, Q9NYZ2, gene:51312 5 literature, yeast mito homolog+, mito protein domain+ MIM Metabolism > Metals and cofactors > Iron homeostasis;
Small molecule transport > SLC25A family none

SLC25A28 solute carrier family 25 member 28 MFRN2, MRS3/4, MRS4L, NPD016, Q96A46, gene:81894 5 literature, yeast mito homolog+, mito protein domain+ MIM Metabolism > Metals and cofactors > Iron homeostasis;
Small molecule transport > SLC25A family none

SLC25A48 solute carrier family 25 member 48 Q6ZT89, gene:153328 5 yeast mito homolog+, mito protein domain+ MIM Small molecule transport > SLC25A family none

SLC25A43 solute carrier family 25 member 43 Q8WUT9, gene:203427 5 yeast mito homolog+, mito protein domain+ MIM Small molecule transport > SLC25A family none

SLC25A47 solute carrier family 25 member 47 C14orf68, HDMCP, HMFN1655, Q6Q0C1, gene:283600 5 literature, yeast mito homolog+, mito protein domain+ MIM Small molecule transport > SLC25A family none

SLC25A41 solute carrier family 25 member 41 APC4, Q8N5S1, gene:284427 5 yeast mito homolog+, mito protein domain+ MIM Metabolism > Nucleotide metabolism > Nucleotide import;
Small molecule transport > SLC25A family none

SLC25A34 solute carrier family 25 member 34 Q6PIV7, gene:284723 5 yeast mito homolog+, mito protein domain+ MIM Small molecule transport > SLC25A family none

EPHX2 epoxide hydrolase 2 ABHD20, CEH, SEH, P34913, gene:2053 5 Rickettsial homolog, mito protein domain, coexpression unknown Metabolism > Detoxification > Xenobiotic metabolism;
Metabolism > Lipid metabolism kidney, liver, heart, skeletalmuscle, adipose, smallintestine, stomach

NUDT5 nudix hydrolase 5 YSA1, YSA1H, YSAH1, hNUDT5, Q9UKK9, gene:11164 5 yeast mito homolog++, mito protein domain, coexpression unknown Metabolism > Nucleotide metabolism > Nucleotide synthesis and processing none

MICU1 mitochondrial calcium uptake 1 CALC, CBARA1, EFHA3, MPXPS, ara CALC, Q9BPX6, gene:10367 5 literature, APEX_IMS, GFP, targetP signal, mito protein domain, MS/MS+ MIM Signaling > Calcium homeostasis > Calcium cycle;
Signaling > Calcium homeostasis > EF hand proteins;
Small molecule transport > Calcium uniporter cerebrum, cerebellum, brainstem, spinalcord, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis

SOD1 superoxide dismutase 1 ALS, ALS1, HEL-S-44, IPOA, SOD, STAHP, hSod1, homodimer, P00441, gene:6647 5 literature, mito protein domain, induction, coexpression IMS Metabolism > Detoxification > ROS and glutathione metabolism all 14

SERHL2 serine hydrolase like 2 dJ222E13.1, Q9H4I8, gene:253190 5 Rickettsial homolog, mito protein domain, MS/MS unknown none kidney, adipose, smallintestine, largeintestine, stomach, placenta, testis

NDUFAF3 NADH:ubiquinone oxidoreductase complex assembly factor 3 2P1, C3orf60, E3-3, MC1DN18, Q9BU61, gene:25915 5 literature, targetP signal, mito protein domain, MS/MS MIM OXPHOS > Complex I > CI assembly factors;
OXPHOS > OXPHOS assembly factors all 14

BOLA3 bolA family member 3 MMDS2, Q53S33, gene:388962 5 targetP signal, mito protein domain, MS/MS Matrix Metabolism > Metals and cofactors > Fe-S cluster biosynthesis;
Metabolism > Metals and cofactors > Fe-S-containing proteins cerebrum, spinalcord, liver, heart, adipose, smallintestine, largeintestine, stomach, placenta, testis

OCIAD2 OCIA domain containing 2 Q56VL3, gene:132299 5 MS/MS++ unknown Signaling all 14

RSAD1 radical S-adenosyl methionine domain containing 1 HemW, Q9HA92, gene:55316 5 APEX_matrix, targetP signal, Rickettsial homolog, mito protein domain, coexpression MIM Metabolism > Metals and cofactors > Fe-S-containing proteins none

CHCHD6 coiled-coil-helix-coiled-coil-helix domain containing 6 CHCM1, MICOS25, Mic25, PPP1R23, Q9BRQ6, gene:84303 5 literature, APEX_IMS, APEX_matrix, coexpression, MS/MS++ MIM Mitochondrial dynamics and surveillance > Cristae formation > MICOS complex cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, heart, adipose, smallintestine, largeintestine, stomach, placenta, testis

FASTKD2 FAST kinase domains 2 COXPD44, KIAA0971, Q9NYY8, gene:22868 5 literature, APEX_matrix, induction, coexpression, MS/MS Matrix 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 skeletalmuscle, adipose, smallintestine, largeintestine, placenta, testis

PEX11B peroxisomal biogenesis factor 11 beta PEX11-BETA, PEX14B, O96011, gene:8799 5 targetP signal, coexpression, MS/MS Membrane none cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, adipose, smallintestine, largeintestine, placenta, testis

TMEM186 transmembrane protein 186 C16orf51, Q96B77, gene:25880 5 targetP signal, coexpression, MS/MS MIM OXPHOS > Complex I > CI assembly factors;
OXPHOS > OXPHOS assembly factors cerebrum, cerebellum, brainstem, spinalcord, liver, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis

PIF1 PIF1 5'-to-3' DNA helicase C15orf20, PIF, Q9H611, gene:80119 5 literature, targetP signal+, yeast mito homolog, mito protein domain Matrix Mitochondrial central dogma > mtDNA maintenance none

FABP1 fatty acid binding protein 1 FABPL, L-FABP, P07148, gene:2168 5 mito protein domain, MS/MS++ unknown Metabolism > Metals and cofactors > Heme synthesis and processing;
Metabolism > Lipid metabolism liver, smallintestine

ABCD1 ATP binding cassette subfamily D member 1 ABC42, ALD, ALDP, AMN, P33897, gene:215 5 Rickettsial homolog, mito protein domain, induction Membrane Small molecule transport > ABC transporters adipose

PLPBP pyridoxal phosphate binding protein EPVB6D, PROSC, O94903, gene:11212 5 APEX_matrix, coexpression, MS/MS++ Matrix Metabolism > Vitamin metabolism > Vitamin B6 metabolism all 14

DBI diazepam binding inhibitor, acyl-CoA binding protein ACBD1, ACBP, CCK-RP, EP, P07108, gene:1622 5 mito protein domain, induction, coexpression unknown Metabolism > Lipid metabolism cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, adipose, smallintestine, largeintestine, stomach, placenta, testis

PNPT1 polyribonucleotide nucleotidyltransferase 1 COXPD13, DFNB70, OLD35, PNPASE, old-35, Q8TCS8, gene:87178 5 literature, APEX_matrix, targetP signal, Rickettsial homolog, mito protein domain Matrix, IMS Mitochondrial central dogma > mtRNA metabolism > mtRNA stability and decay all 14

TOP1MT DNA topoisomerase I mitochondrial Q969P6, gene:116447 5 literature, APEX_matrix, targetP signal, mito protein domain, coexpression Matrix Mitochondrial central dogma > mtDNA maintenance > mtDNA replication;
Mitochondrial central dogma > mtRNA metabolism > Transcription none

RNASEH1 ribonuclease H1 H1RNA, PEOB2, RNH1, O60930, gene:246243 5 literature, APEX_matrix, targetP signal, Rickettsial homolog, coexpression Matrix Mitochondrial central dogma > mtDNA maintenance > mtDNA replication;
Mitochondrial central dogma > mtRNA metabolism > mtRNA stability and decay none

ABCA9 ATP binding cassette subfamily A member 9 EST640918, Q8IUA7, gene:10350 5 GFP, yeast mito homolog, Rickettsial homolog, mito protein domain Membrane Small molecule transport > ABC transporters cerebrum, placenta

SCP2 sterol carrier protein 2 NLTP, NSL-TP, SCP-2, SCP-CHI, SCP-X, SCPX, P22307, gene:6342 5 Rickettsial homolog, mito protein domain+ MIM Metabolism > Lipid metabolism > Cholesterol, bile acid, steroid synthesis brainstem, spinalcord, kidney, liver, heart, adipose, smallintestine, placenta, testis

NT5DC3 5'-nucleotidase domain containing 3 TU12B1-TY, TU12B1TY, Q86UY8, gene:51559 5 APEX_matrix, targetP signal, MS/MS++ Matrix none cerebrum, cerebellum, brainstem, spinalcord, skeletalmuscle, stomach, placenta, testis

CMC4 C-X9-C motif containing 4 C6.1B, MTCP1, MTCP1B, MTCP1NB, p8, p8MTCP1, P56277, gene:100272147 5 yeast mito homolog++, mito protein domain+ IMS none none

LDHB lactate dehydrogenase B HEL-S-281, LDH-B, LDH-H, LDHBD, TRG-5, P07195, gene:3945 5 Rickettsial homolog, mito protein domain, induction unknown Metabolism > Amino acid metabolism > Glyoxylate metabolism cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, adipose, smallintestine, largeintestine, stomach, placenta, testis

YBEY ybeY metalloendoribonuclease C21orf57, P58557, gene:54059 5 APEX_matrix, targetP signal, Rickettsial homolog, MS/MS Matrix Mitochondrial central dogma > mtRNA metabolism > Polycistronic mtRNA processing;
Mitochondrial central dogma > Translation > Mitochondrial ribosome assembly largeintestine, placenta, testis

HINT1 histidine triad nucleotide binding protein 1 HINT, NMAN, PKCI-1, PRKCNH1, P49773, gene:3094 5 Rickettsial homolog, mito protein domain, coexpression unknown Metabolism > Nucleotide metabolism > Nucleotide synthesis and processing cerebrum, cerebellum, brainstem, spinalcord, kidney, smallintestine, placenta, testis

MUL1 mitochondrial E3 ubiquitin protein ligase 1 C1orf166, GIDE, MAPL, MULAN, RNF218, Q969V5, gene:79594 5 literature, mito protein domain+, induction MOM Protein import, sorting and homeostasis > Protein homeostasis;
Mitochondrial dynamics and surveillance > Fission;
Signaling skeletalmuscle, largeintestine

TK2 thymidine kinase 2 MTDPS2, MTTK, PEOB3, SCA31, O00142, gene:7084 5 literature, targetP signal, mito protein domain, MS/MS Matrix Metabolism > Nucleotide metabolism > Nucleotide synthesis and processing cerebrum, cerebellum, brainstem, adipose

AGMAT agmatinase Q9BSE5, gene:79814 5 literature, targetP signal, mito protein domain, MS/MS+ Matrix Metabolism > Amino acid metabolism liver, smallintestine

CMPK2 cytidine/uridine monophosphate kinase 2 NDK, TMPK2, TYKi, UMP-CMPK2, Q5EBM0, gene:129607 4 literature, targetP signal+, MS/MS Matrix Metabolism > Nucleotide metabolism > Nucleotide synthesis and processing spinalcord, smallintestine

BID BH3 interacting domain death agonist FP497, P55957, gene:637 4 literature, targetP signal+, mito protein domain, coexpression MOM Mitochondrial dynamics and surveillance > Apoptosis none

ARL2 ADP ribosylation factor like GTPase 2 ARFL2, P36404, gene:402 4 literature, mito protein domain, coexpression, MS/MS+ IMS Mitochondrial dynamics and surveillance > Fusion cerebrum, brainstem, spinalcord, adipose, largeintestine

SYNJ2BP synaptojanin 2 binding protein ARIP2, OMP25, P57105, gene:55333 4 APEX_IMS, mito protein domain, coexpression, MS/MS+ MOM Mitochondrial dynamics and surveillance > Organelle contact sites cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, heart, skeletalmuscle, adipose, largeintestine, stomach, placenta, testis

MFF mitochondrial fission factor C2orf33, EMPF2, GL004, Q9GZY8, gene:56947 4 literature, induction, MS/MS MOM Mitochondrial dynamics and surveillance > Fission cerebrum, cerebellum, brainstem, spinalcord, adipose, largeintestine, placenta, testis

DCXR dicarbonyl and L-xylulose reductase DCR, HCR2, HCRII, KIDCR, P34H, PNTSU, SDR20C1, XR, Q7Z4W1, gene:51181 4 APEX_matrix, targetP signal, Rickettsial homolog, mito protein domain Matrix Metabolism > Carbohydrate metabolism cerebrum, cerebellum, spinalcord, heart, adipose, largeintestine, stomach, placenta, testis

NUDT9 nudix hydrolase 9 NUDT10, Q9BW91, gene:53343 4 APEX_matrix, mito protein domain, coexpression, MS/MS Matrix Metabolism > Nucleotide metabolism > Nucleotide synthesis and processing cerebrum, cerebellum, placenta, testis

AMACR alpha-methylacyl-CoA racemase AMACRD, CBAS4, P504S, RACE, RM, Q9UHK6, gene:23600 4 literature, mito protein domain, coexpression, MS/MS Matrix Metabolism > Lipid metabolism > Fatty acid oxidation kidney, liver, adipose, smallintestine, testis

VPS13D vacuolar protein sorting 13 homolog D SCAR4, Q5THJ4, gene:55187 4 manual, mito protein domain, induction, coexpression MOM Mitochondrial dynamics and surveillance > Mitophagy;
Mitochondrial dynamics and surveillance > Autophagy cerebrum, cerebellum, brainstem, spinalcord, kidney, skeletalmuscle

NUDT6 nudix hydrolase 6 ASFGF2, FGF-AS, FGF2AS, GFG-1, GFG1, P53370, gene:11162 4 APEX_matrix, targetP signal, mito protein domain, coexpression Matrix none none

DMAC2 distal membrane arm assembly complex 2 ATP5SL, Q9NW81, gene:55101 4 APEX_matrix, GFP, targetP signal, induction, MS/MS MIM OXPHOS > Complex I > CI assembly factors;
OXPHOS > OXPHOS assembly factors cerebrum, cerebellum, brainstem, stomach

MRM2 mitochondrial rRNA methyltransferase 2 FJH1, FTSJ2, HEL97, MTDPS17, RRMJ2, Q9UI43, gene:29960 4 literature, Rickettsial homolog, mito protein domain, coexpression Matrix Mitochondrial central dogma > mtRNA metabolism > mtRNA granules;
Mitochondrial central dogma > mtRNA metabolism > mt-rRNA modifications;
Mitochondrial central dogma > Translation > Mitochondrial ribosome assembly cerebrum

BAK1 BCL2 antagonist/killer 1 BAK, BAK-LIKE, BCL2L7, CDN1, Q16611, gene:578 4 literature, mito protein domain, MS/MS++ MOM Mitochondrial dynamics and surveillance > Apoptosis brainstem, spinalcord, liver, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis

DHODH dihydroorotate dehydrogenase (quinone) DHOdehase, POADS, URA1, Q02127, gene:1723 4 literature, mito protein domain, MS/MS++ MIM Metabolism > Nucleotide metabolism > Nucleotide synthesis and processing;
Metabolism > Electron carriers > Q-linked reactions, other cerebrum, spinalcord, kidney, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis

MAOB monoamine oxidase B P27338, gene:4129 4 literature, mito protein domain, MS/MS++ MOM Metabolism > Amino acid metabolism > Catechol metabolism;
Metabolism > Detoxification > Xenobiotic metabolism cerebrum, cerebellum, brainstem, spinalcord, liver, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis

MGST3 microsomal glutathione S-transferase 3 GST-III, O14880, gene:4259 4 APEX_IMS, mito protein domain, MS/MS++ MOM Metabolism > Lipid metabolism > Eicosanoid metabolism all 14

ACP6 acid phosphatase 6, lysophosphatidic ACPL1, LPAP, PACPL1, Q9NPH0, gene:51205 4 literature, APEX_IMS, mito protein domain, MS/MS++ IMS Metabolism > Lipid metabolism > Phospholipid metabolism cerebrum, spinalcord, kidney, liver, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis

MRPL48 mitochondrial ribosomal protein L48 CGI-118, HSPC290, L48MT, MRP-L48, Q96GC5, gene:51642 4 literature, APEX_matrix, mito protein domain, MS/MS++ Matrix Mitochondrial central dogma > Translation > Mitochondrial ribosome all 14

AGXT alanine--glyoxylate and serine--pyruvate aminotransferase AGT, AGT1, AGXT1, PH1, SPAT, SPT, TLH6, P21549, gene:189 4 literature, yeast mito homolog, mito protein domain, MS/MS Matrix Metabolism > Amino acid metabolism > Serine metabolism;
Metabolism > Amino acid metabolism > Glyoxylate metabolism liver

CYB5R3 cytochrome b5 reductase 3 B5R, DIA1, P00387, gene:1727 4 literature, mito protein domain, MS/MS+ MOM Metabolism > Detoxification > Xenobiotic metabolism;
Metabolism > Detoxification > Amidoxime reducing complex;
Metabolism > Lipid metabolism cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis

DDX28 DEAD-box helicase 28 MDDX28, Q9NUL7, gene:55794 4 literature, APEX_matrix, targetP signal, Rickettsial homolog, mito protein domain Matrix Mitochondrial central dogma > mtRNA metabolism > mtRNA granules;
Mitochondrial central dogma > Translation > Mitochondrial ribosome assembly spinalcord, adipose

PNKD PNKD metallo-beta-lactamase domain containing BRP17, DYT8, FKSG19, FPD1, KIPP1184, MR-1, MR-1S, MR1, PDC, PKND1, PNKD1, R1, TAHCCP2, Q8N490, gene:25953 4 literature, targetP signal, mito protein domain, MS/MS MIM OXPHOS > Complex IV > CIV assembly factors;
OXPHOS > OXPHOS assembly factors cerebrum, cerebellum, spinalcord, liver, adipose, smallintestine, largeintestine, stomach, placenta, testis

MICU3 mitochondrial calcium uptake family member 3 EFHA2, Q86XE3, gene:286097 4 manual, targetP signal, mito protein domain, MS/MS MIM Signaling > Calcium homeostasis > Calcium cycle;
Signaling > Calcium homeostasis > EF hand proteins;
Small molecule transport > Calcium uniporter cerebrum, cerebellum, brainstem, spinalcord, skeletalmuscle, stomach

BNIP3 BCL2 interacting protein 3 NIP3, Q12983, gene:664 4 literature, mito protein domain+, MS/MS MOM Mitochondrial dynamics and surveillance > Apoptosis cerebrum, cerebellum, brainstem, heart, skeletalmuscle, adipose

MTX3 metaxin 3 Q5HYI7, gene:345778 4 manual, mito protein domain+, MS/MS MOM Mitochondrial dynamics and surveillance > Intramitochondrial membrane interactions cerebrum, cerebellum, brainstem, spinalcord

MUTYH mutY DNA glycosylase MYH, Q9UIF7, gene:4595 4 GFP, targetP signal+, Rickettsial homolog, mito protein domain Matrix Mitochondrial central dogma > mtDNA maintenance none

GTPBP6 GTP binding protein 6 (putative) PGPL, O43824, gene:8225 4 APEX_matrix, targetP signal+, Rickettsial homolog, mito protein domain Matrix none none

CYP11A1 cytochrome P450 family 11 subfamily A member 1 CYP11A, CYPXIA1, P450SCC, P05108, gene:1583 4 literature, mito protein domain, MS/MS+ MIM Metabolism > Lipid metabolism > Cholesterol, bile acid, steroid synthesis;
Metabolism > Metals and cofactors > Heme-containing proteins adipose, placenta, testis

OCIAD1 OCIA domain containing 1 ASRIJ, OCIA, TPA018, Q9NX40, gene:54940 4 APEX_IMS, GFP, MS/MS++ MOM none all 14

METTL8 methyltransferase like 8 TIP, Q9H825, gene:79828 4 APEX_matrix, targetP signal, Rickettsial homolog, mito protein domain Matrix Mitochondrial central dogma > mtRNA metabolism none

SMIM20 small integral membrane protein 20 C4orf52, MITRAC7, PNX, Q8N5G0, gene:389203 4 literature, coexpression, MS/MS MIM OXPHOS > Complex IV > CIV assembly factors;
OXPHOS > OXPHOS assembly factors cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, heart, adipose, smallintestine, placenta, testis

RPUSD4 RNA pseudouridine synthase D4 Q96CM3, gene:84881 4 APEX_matrix, GFP, Rickettsial homolog, mito protein domain, coexpression Matrix Mitochondrial central dogma > mtRNA metabolism > mtRNA granules;
Mitochondrial central dogma > mtRNA metabolism > mt-tRNA modifications;
Mitochondrial central dogma > mtRNA metabolism > mt-rRNA modifications placenta

SMIM8 small integral membrane protein 8 C6orf162, dJ102H19.2, Q96KF7, gene:57150 4 manual, induction, coexpression, MS/MS MOM none cerebrum, cerebellum, brainstem, spinalcord, kidney, adipose, placenta, testis

NME6 NME/NM23 nucleoside diphosphate kinase 6 IPIA-ALPHA, NDK 6, NM23-H6, O75414, gene:10201 3 literature, APEX_matrix, GFP, Rickettsial homolog, mito protein domain, coexpression Matrix Metabolism > Nucleotide metabolism > Nucleotide synthesis and processing largeintestine

FKBP8 FKBP prolyl isomerase 8 FKBP38, FKBPr38, Q14318, gene:23770 3 literature, mito protein domain, coexpression, MS/MS MOM Mitochondrial dynamics and surveillance > Organelle contact sites;
Mitochondrial dynamics and surveillance > Mitophagy;
Mitochondrial dynamics and surveillance > Autophagy cerebrum, brainstem, spinalcord, largeintestine, placenta, testis

ACSM4 acyl-CoA synthetase medium chain family member 4 P0C7M7, gene:341392 3 manual, targetP signal, Rickettsial homolog, mito protein domain Matrix Metabolism > Lipid metabolism > Fatty acid oxidation none

NLRX1 NLR family member X1 CLR11.3, DLNB26, NOD26, NOD5, NOD9, Q86UT6, gene:79671 3 APEX_matrix, targetP signal, MS/MS Matrix Signaling > Immune response all 14

FOXRED1 FAD dependent oxidoreductase domain containing 1 FP634, H17, MC1DN19, Q96CU9, gene:55572 3 literature, mito protein domain, coexpression+ MIM OXPHOS > Complex I > CI assembly factors;
OXPHOS > OXPHOS assembly factors adipose, largeintestine, placenta, testis

TFB2M transcription factor B2, mitochondrial Hkp1, mtTFB2, Q9H5Q4, gene:64216 3 literature, APEX_matrix, targetP signal+, mito protein domain Matrix Mitochondrial central dogma > mtDNA maintenance > mtDNA replication;
Mitochondrial central dogma > mtRNA metabolism > Transcription placenta

PPM1K protein phosphatase, Mg2+/Mn2+ dependent 1K BDP, MSUDMV, PP2Ckappa, PP2Cm, PTMP, UG0882E07, Q8N3J5, gene:152926 3 literature, targetP signal+, mito protein domain Matrix Metabolism > Amino acid metabolism > BCAA metabolism;
Metabolism > Amino acid metabolism > BCAA dehydrogenase complex cerebellum, liver, heart, adipose

ATP5MC2 ATP synthase membrane subunit c locus 2 ATP5A, ATP5G2, Q06055, gene:517 3 literature, yeast mito homolog+, Rickettsial homolog, mito protein domain MIM OXPHOS > Complex V > CV subunits;
OXPHOS > OXPHOS subunits none

MT-ND1 NADH dehydrogenase, subunit 1 (complex I) MTND1, P03886, gene:4535 3 literature, Rickettsial homolog, mito protein domain, MS/MS MIM OXPHOS > Complex I > CI subunits;
OXPHOS > OXPHOS subunits all 14

TAZ tafazzin BTHS, CMD3A, EFE, EFE2, G4.5, LVNCX, Taz1, Q16635, gene:6901 3 literature, yeast mito homolog++, mito protein domain IMS Metabolism > Lipid metabolism > Cardiolipin synthesis none

MSRB3 methionine sulfoxide reductase B3 DFNB74, Q8IXL7, gene:253827 3 literature, yeast mito homolog+, mito protein domain unknown Metabolism > Detoxification > ROS and glutathione metabolism none

ACOT11 acyl-CoA thioesterase 11 BFIT, STARD14, THEA, THEM1, Q8WXI4, gene:26027 3 literature, targetP signal, mito protein domain, coexpression Matrix Metabolism > Lipid metabolism > Fatty acid oxidation none

NIPSNAP3B nipsnap homolog 3B FP944, NIPSNAP3, SNAP1, Q9BS92, gene:55335 3 GFP, targetP signal, MS/MS+ Matrix none cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, adipose, smallintestine, largeintestine, stomach, placenta, testis

NTHL1 nth like DNA glycosylase 1 FAP3, NTH1, OCTS3, hNTH1, P78549, gene:4913 3 literature, yeast mito homolog, Rickettsial homolog, mito protein domain unknown Metabolism > Metals and cofactors > Fe-S-containing proteins none

MARCHF5 membrane associated ring-CH-type finger 5 MARCH-V, MARCH5, MITOL, RNF153, Q9NX47, gene:54708 3 literature, mito protein domain, coexpression, MS/MS MOM Mitochondrial dynamics and surveillance > Mitophagy;
Mitochondrial dynamics and surveillance > Autophagy cerebrum, cerebellum, brainstem, spinalcord, liver, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis

SFXN4 sideroflexin 4 BCRM1, COXPD18, SLC56A4, Q6P4A7, gene:119559 3 literature, mito protein domain+ MIM Small molecule transport > Sideroflexins cerebellum, spinalcord

TRMT5 tRNA methyltransferase 5 COXPD26, KIAA1393, TRM5, Q32P41, gene:57570 3 literature, yeast mito homolog, mito protein domain, coexpression Matrix Mitochondrial central dogma > mtRNA metabolism > mt-tRNA modifications none

SLC25A6 solute carrier family 25 member 6 AAC3, ANT, ANT 2, ANT 3, ANT3, ANT3Y, P12236, gene:293 3 literature, yeast mito homolog+, mito protein domain+ MIM Metabolism > Nucleotide metabolism > Nucleotide import;
Signaling > Calcium homeostasis > Mitochondrial permeability transition pore;
Small molecule transport > SLC25A family none

SLC25A52 solute carrier family 25 member 52 MCART2, Q3SY17, gene:147407 3 manual, yeast mito homolog+, mito protein domain+ MIM Metabolism > Metals and cofactors > NAD biosynthesis and metabolism;
Small molecule transport > SLC25A family none

OGG1 8-oxoguanine DNA glycosylase HMMH, HOGG1, MUTM, OGH1, O15527, gene:4968 3 literature, targetP signal, yeast mito homolog, mito protein domain Matrix Mitochondrial central dogma > mtDNA maintenance > mtDNA repair none

ACOD1 aconitate decarboxylase 1 CAD, IRG1, A6NK06, gene:730249 3 literature, yeast mito homolog, mito protein domain+ Matrix Metabolism > Carbohydrate metabolism > Itaconate metabolism;
Signaling > Immune response none

QTRT1 queuine tRNA-ribosyltransferase catalytic subunit 1 FP3235, TGT, TGUT, Q9BXR0, gene:81890 3 APEX_matrix, targetP signal, Rickettsial homolog, mito protein domain, coexpression Matrix Mitochondrial central dogma > mtRNA metabolism > mt-tRNA modifications none

AIFM2 apoptosis inducing factor mitochondria associated 2 AMID, FSP1, PRG3, Q9BRQ8, gene:84883 3 literature, Rickettsial homolog, mito protein domain, coexpression Membrane Metabolism > Detoxification > ROS and glutathione metabolism;
Mitochondrial dynamics and surveillance > Apoptosis none

PRDX2 peroxiredoxin 2 HEL-S-2a, NKEF-B, NKEFB, PRP, PRX2, PRXII, PTX1, TDPX1, TPX1, TSA, P32119, gene:7001 3 GFP, Rickettsial homolog, mito protein domain, coexpression unknown Metabolism > Detoxification > ROS and glutathione metabolism cerebrum, cerebellum, brainstem, spinalcord, adipose, smallintestine, stomach, placenta, testis

FBXL4 F-box and leucine rich repeat protein 4 FBL4, FBL5, MTDPS13, Q9UKA2, gene:26235 2 literature, mito protein domain, coexpression IMS none adipose

GPAM glycerol-3-phosphate acyltransferase, mitochondrial GPAT, GPAT1, Q9HCL2, gene:57678 2 literature, mito protein domain, coexpression Membrane Metabolism > Lipid metabolism > Cardiolipin synthesis;
Metabolism > Lipid metabolism > Phospholipid metabolism cerebellum, spinalcord, kidney, liver, skeletalmuscle, adipose, largeintestine, stomach, placenta, testis

DMAC2L distal membrane arm assembly complex 2 like ATP5S, ATPW, FB, HSU79253, Q99766, gene:27109 2 literature, APEX_matrix, MS/MS++ MIM OXPHOS > Complex V > CV subunits;
OXPHOS > OXPHOS subunits cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, heart, skeletalmuscle, adipose, largeintestine, stomach, placenta, testis

RMDN3 regulator of microtubule dynamics 3 FAM82A2, FAM82C, RMD-3, RMD3, ptpip51, Q96TC7, gene:55177 2 literature, APEX_IMS, GFP, MS/MS++ MOM Mitochondrial dynamics and surveillance > Organelle contact sites cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, adipose, largeintestine, stomach, placenta, testis

PRXL2A peroxiredoxin like 2A Adrx, C10orf58, FAM213A, PAMM, Q9BRX8, gene:84293 2 GFP, MS/MS++ Membrane Metabolism > Detoxification > ROS and glutathione metabolism cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, skeletalmuscle, adipose, smallintestine, placenta, testis

ATP5MD ATP synthase membrane subunit DAPIT DAPIT, HCVFTP2, MC5DN6, USMG5, bA792D24.4, Q96IX5, gene:84833 2 GFP, MS/MS++ MIM OXPHOS > Complex V > CV subunits;
OXPHOS > OXPHOS subunits;
Mitochondrial dynamics and surveillance > Cristae formation all 14

PET117 PET117 cytochrome c oxidase chaperone CSRP2BP, Q6UWS5, gene:100303755 2 literature, yeast mito homolog, MS/MS MIM OXPHOS > Complex IV > CIV assembly factors;
OXPHOS > OXPHOS assembly factors cerebrum, cerebellum, liver, adipose, largeintestine, stomach, placenta

MAOA monoamine oxidase A BRNRS, MAO-A, P21397, gene:4128 2 literature, mito protein domain, MS/MS+ MOM Metabolism > Amino acid metabolism > Catechol metabolism;
Metabolism > Detoxification > Xenobiotic metabolism all 14

C15orf61 chromosome 15 open reading frame 61 A6NNL5, gene:145853 2 APEX_matrix, targetP signal, MS/MS Matrix none brainstem, spinalcord, skeletalmuscle, adipose, largeintestine, stomach, testis

POLQ DNA polymerase theta PRO0327, O75417, gene:10721 2 literature, targetP signal, Rickettsial homolog, mito protein domain Matrix Mitochondrial central dogma > mtDNA maintenance brainstem, testis

BCO2 beta-carotene oxygenase 2 B-DIOX-II, BCDO2, Q9BYV7, gene:83875 2 literature, targetP signal, mito protein domain, MS/MS MIM Metabolism > Vitamin metabolism > Vitamin A metabolism smallintestine

IFI27 interferon alpha inducible protein 27 FAM14D, ISG12, ISG12A, P27, P40305, gene:3429 2 literature, induction, coexpression Membrane Signaling > Immune response;
Mitochondrial dynamics and surveillance > Apoptosis none

CRYZ crystallin zeta Q08257, gene:1429 2 APEX_matrix, mito protein domain, MS/MS Matrix none spinalcord, kidney, liver, adipose, placenta, testis

PYCR1 pyrroline-5-carboxylate reductase 1 ARCL2B, ARCL3B, P5C, P5CR, PIG45, PP222, PRO3, PYCR, P32322, gene:5831 2 literature, mito protein domain, MS/MS Matrix Metabolism > Amino acid metabolism > Proline metabolism cerebrum, cerebellum, brainstem, spinalcord, adipose, largeintestine, stomach, placenta, testis

TMLHE trimethyllysine hydroxylase, epsilon AUTSX6, BBOX2, TMLD, TMLH, TMLHED, XAP130, Q9NVH6, gene:55217 2 literature, APEX_matrix, mito protein domain, MS/MS Matrix Metabolism > Metals and cofactors > Carnitine synthesis and transport kidney, adipose, smallintestine, largeintestine, stomach, placenta, testis

TOMM20 translocase of outer mitochondrial membrane 20 MAS20, MOM19, TOM20, Q15388, gene:9804 2 literature, yeast mito homolog+ MOM Protein import, sorting and homeostasis > Protein import and sorting > TOM;
Mitochondrial dynamics and surveillance cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, heart, skeletalmuscle, adipose, largeintestine, stomach, placenta, testis

YRDC yrdC N6-threonylcarbamoyltransferase domain containing DRIP3, IRIP, SUA5, Q86U90, gene:79693 2 manual, targetP signal+, Rickettsial homolog Matrix Mitochondrial central dogma > mtRNA metabolism > mt-tRNA modifications none

COA7 cytochrome c oxidase assembly factor 7 (putative) C1orf163, RESA1, SCAN3, SELRC1, Q96BR5, gene:65260 2 literature, APEX_IMS, mito protein domain, coexpression, MS/MS IMS OXPHOS > Complex IV > CIV assembly factors;
OXPHOS > OXPHOS assembly factors cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis

DHRS2 dehydrogenase/reductase 2 HEP27, SDR25C1, Q13268, gene:10202 2 literature, targetP signal, Rickettsial homolog, mito protein domain Matrix Metabolism > Detoxification > Xenobiotic metabolism none

MMAA metabolism of cobalamin associated A cblA, Q8IVH4, gene:166785 2 manual, coexpression+ Matrix Metabolism > Vitamin metabolism > Vitamin B12 metabolism cerebrum, cerebellum, brainstem, kidney, liver, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis

MIEF1 mitochondrial elongation factor 1 AltMIEF1, HSU79252, MID51, MIEF1-MP, SMCR7L, dJ1104E15.3, Q9NQG6, gene:54471 2 literature, mito protein domain, induction, coexpression MOM, Matrix Mitochondrial central dogma > Translation > Mitochondrial ribosome assembly;
Mitochondrial dynamics and surveillance > Fission none

C15orf48 chromosome 15 open reading frame 48 COXFA4L3, FOAP-11, MIR147BHG, NMES1, Q9C002, gene:84419 1 GFP, mito protein domain, MS/MS+ Membrane none smallintestine, largeintestine, stomach, placenta, testis

LIG3 DNA ligase 3 LIG2, LIG3alpha, P49916, gene:3980 1 literature, targetP signal, mito protein domain, coexpression Matrix Mitochondrial central dogma > mtDNA maintenance > mtDNA replication;
Mitochondrial central dogma > mtDNA maintenance > mtDNA repair none

BNIP3L BCL2 interacting protein 3 like BNIP3a, NIX, O60238, gene:665 1 literature, mito protein domain+, coexpression MOM Mitochondrial dynamics and surveillance > Apoptosis none

FASTKD5 FAST kinase domains 5 dJ1187M17.5, Q7L8L6, gene:60493 1 APEX_matrix, targetP signal, coexpression Matrix Mitochondrial central dogma > mtRNA metabolism > mtRNA granules;
Mitochondrial central dogma > mtRNA metabolism > Polycistronic mtRNA processing;
Mitochondrial central dogma > mtRNA metabolism > mtRNA stability and decay skeletalmuscle, largeintestine, placenta

PARK7 Parkinsonism associated deglycase DJ-1, DJ1, GATD2, HEL-S-67p, Q99497, gene:11315 1 literature, mito protein domain, coexpression MOM Protein import, sorting and homeostasis > Protein homeostasis;
Mitochondrial dynamics and surveillance > Mitophagy;
Mitochondrial dynamics and surveillance > Autophagy all 14

COA1 cytochrome c oxidase assembly factor 1 homolog C7orf44, MITRAC15, Q9GZY4, gene:55744 1 APEX_IMS, targetP signal, mito protein domain+ MIM OXPHOS > Complex I > CI assembly factors;
OXPHOS > Complex IV > CIV assembly factors;
OXPHOS > OXPHOS assembly factors none

COX7B2 cytochrome c oxidase subunit 7B2 Q8TF08, gene:170712 1 literature, targetP signal, mito protein domain+ MIM OXPHOS > Complex IV > CIV subunits;
OXPHOS > OXPHOS subunits none

MT-ND4L NADH dehydrogenase, subunit 4L (complex I) MTND4L, P03901, gene:4539 1 literature, Rickettsial homolog, mito protein domain MIM OXPHOS > Complex I > CI subunits;
OXPHOS > OXPHOS subunits heart, skeletalmuscle

SIRT4 sirtuin 4 SIR2L4, Q9Y6E7, gene:23409 1 literature, targetP signal, mito protein domain Matrix Metabolism > Metals and cofactors > NAD biosynthesis and metabolism largeintestine

UQCC2 ubiquinol-cytochrome c reductase complex assembly factor 2 C6orf125, C6orf126, Cbp6, M19, MC3DN7, MNF1, bA6B20.2, Q9BRT2, gene:84300 1 APEX_matrix, GFP, coexpression, MS/MS MIM OXPHOS > Complex III > CIII assembly factors;
OXPHOS > OXPHOS assembly factors all 14

USP30 ubiquitin specific peptidase 30 Q70CQ3, gene:84749 1 literature, targetP signal, mito protein domain MOM Protein import, sorting and homeostasis > Protein homeostasis > Proteases;
Mitochondrial dynamics and surveillance > Mitophagy;
Mitochondrial dynamics and surveillance > Autophagy adipose, placenta, testis

PDE12 phosphodiesterase 12 2'-PDE, 2-PDE, Q6L8Q7, gene:201626 1 APEX_matrix, targetP signal, mito protein domain Matrix Mitochondrial central dogma > mtRNA metabolism > mt-mRNA modifications;
Mitochondrial central dogma > mtRNA metabolism > mtRNA stability and decay brainstem, smallintestine, largeintestine, testis

NME3 NME/NM23 nucleoside diphosphate kinase 3 DR-nm23, NDPK-C, NDPKC, NM23-H3, NM23H3, c371H6.2, Q13232, gene:4832 1 GFP, Rickettsial homolog, mito protein domain MOM Metabolism > Nucleotide metabolism > Nucleotide synthesis and processing cerebrum, cerebellum, brainstem, kidney, adipose, placenta, testis

DHX30 DExH-box helicase 30 DDX30, NEDMIAL, RETCOR, Q7L2E3, gene:22907 1 literature, mito protein domain Matrix Mitochondrial central dogma > mtRNA metabolism > mtRNA granules;
Mitochondrial central dogma > Translation > Mitochondrial ribosome assembly cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, skeletalmuscle, adipose, largeintestine, stomach, placenta, testis

FASTK Fas activated serine/threonine kinase FAST, Q14296, gene:10922 1 GFP, induction, coexpression MIM Mitochondrial central dogma > mtRNA metabolism > mtRNA granules;
Mitochondrial central dogma > mtRNA metabolism > Polycistronic mtRNA processing;
Mitochondrial central dogma > mtRNA metabolism > mtRNA stability and decay none

METTL15 methyltransferase like 15 METT5D1, A6NJ78, gene:196074 1 APEX_matrix, Rickettsial homolog, coexpression Matrix Mitochondrial central dogma > mtRNA metabolism > mt-rRNA modifications largeintestine, placenta

CYP24A1 cytochrome P450 family 24 subfamily A member 1 CP24, CYP24, HCAI, HCINF1, P450-CC24, Q07973, gene:1591 1 literature, targetP signal+, mito protein domain MIM Metabolism > Metals and cofactors > Heme-containing proteins;
Metabolism > Vitamin metabolism > Vitamin D metabolism none

NOCT nocturnin CCR4L, CCRN4L, Ccr4c, NOC, Q9UK39, gene:25819 1 literature, targetP signal+, mito protein domain Matrix Metabolism > Metals and cofactors > NAD biosynthesis and metabolism none

PANK2 pantothenate kinase 2 C20orf48, HARP, HSS, NBIA1, PKAN, Q9BZ23, gene:80025 1 literature, targetP signal+, mito protein domain IMS Metabolism > Metals and cofactors > Coenzyme A metabolism none

CA5A carbonic anhydrase 5A CA5, CA5AD, CAV, CAVA, GS1-21A4.1, P35218, gene:763 1 literature, mito protein domain, MS/MS Matrix Metabolism > Amino acid metabolism > Urea cycle liver

NT5DC2 5'-nucleotidase domain containing 2 Q9H857, gene:64943 1 APEX_matrix, targetP signal, MS/MS Matrix Metabolism > Nucleotide metabolism > Nucleotide synthesis and processing spinalcord, largeintestine, placenta

ATAD3B ATPase family AAA domain containing 3B AAA-TOB3, TOB3, Q5T9A4, gene:83858 1 APEX_IMS, APEX_matrix, GFP, Rickettsial homolog, mito protein domain+ MIM Mitochondrial central dogma > mtDNA maintenance > mtDNA nucleoid;
Mitochondrial dynamics and surveillance > Organelle contact sites;
Mitochondrial dynamics and surveillance > Intramitochondrial membrane interactions none

TRUB2 TruB pseudouridine synthase family member 2 CLONE24922, O95900, gene:26995 1 APEX_matrix, Rickettsial homolog, mito protein domain Matrix Mitochondrial central dogma > mtRNA metabolism > mtRNA granules;
Mitochondrial central dogma > mtRNA metabolism > mt-rRNA modifications none

PRKACA protein kinase cAMP-activated catalytic subunit alpha PKACA, PPNAD4, P17612, gene:5566 0 literature, mito protein domain, coexpression MIM Signaling > cAMP-PKA signaling cerebrum, brainstem, spinalcord, adipose, testis

COMTD1 catechol-O-methyltransferase domain containing 1 Q86VU5, gene:118881 0 GFP, MS/MS++ Membrane none all 14

CPT1C carnitine palmitoyltransferase 1C CATL1, CPT1-B, CPT1P, CPTI-B, CPTIC, SPG73, Q8TCG5, gene:126129 0 literature, yeast mito homolog+, mito protein domain MOM Metabolism > Lipid metabolism > Fatty acid oxidation;
Metabolism > Metals and cofactors > Carnitine synthesis and transport;
Metabolism > Metals and cofactors > Carnitine shuttle none

C3orf33 chromosome 3 open reading frame 33 AC3-33, Q6P1S2, gene:285315 0 APEX_matrix, targetP signal, MS/MS Matrix none adipose, testis

MIGA2 mitoguardin 2 C9orf54, FAM73B, Q7L4E1, gene:84895 0 literature, coexpression MOM Mitochondrial dynamics and surveillance > Fusion;
Mitochondrial dynamics and surveillance > Organelle contact sites cerebrum, cerebellum, brainstem, spinalcord, liver, testis

SND1 staphylococcal nuclease and tudor domain containing 1 TDRD11, Tudor-SN, p100, Q7KZF4, gene:27044 0 GFP, yeast mito homolog, mito protein domain unknown none cerebrum, cerebellum, brainstem, liver, smallintestine, placenta, testis

GLUD2 glutamate dehydrogenase 2 GDH2, GLUDP1, P49448, gene:2747 0 literature, targetP signal+, mito protein domain Matrix Metabolism > Amino acid metabolism > Glutamate metabolism;
Metabolism > Amino acid metabolism > GABA metabolism none

HPDL 4-hydroxyphenylpyruvate dioxygenase like 4-HPPD-L, GLOXD1, Q96IR7, gene:84842 0 manual, targetP signal, induction unknown none none

ANGEL2 angel homolog 2 Ccr4d, KIAA0759L, Q5VTE6, gene:90806 0 APEX_matrix, mito protein domain, MS/MS Matrix Mitochondrial central dogma > mtRNA metabolism > mt-mRNA modifications adipose, smallintestine, placenta, testis

ANTKMT adenine nucleotide translocase lysine methyltransferase ANT-KMT, C16orf24, FAM173A, Q9BQD7, gene:65990 0 literature, coexpression, MS/MS MIM Metabolism > Nucleotide metabolism cerebrum, cerebellum, brainstem, spinalcord, adipose, largeintestine, placenta

UQCC3 ubiquinol-cytochrome c reductase complex assembly factor 3 C11orf83, CCDS41658.1, MC3DN9, UNQ655, Q6UW78, gene:790955 0 literature, coexpression, MS/MS MIM OXPHOS > Complex III > CIII assembly factors;
OXPHOS > OXPHOS assembly factors liver, skeletalmuscle, adipose, largeintestine, stomach, placenta, testis

PRDX4 peroxiredoxin 4 AOE37-2, AOE372, HEL-S-97n, PRX-4, Q13162, gene:10549 0 APEX_IMS, Rickettsial homolog, mito protein domain IMS Metabolism > Detoxification > ROS and glutathione metabolism cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis

CDK5RAP1 CDK5 regulatory subunit associated protein 1 C20orf34, C42, CGI-05, HSPC167, Q96SZ6, gene:51654 0 literature, targetP signal, Rickettsial homolog, mito protein domain Matrix Mitochondrial central dogma > mtRNA metabolism > mt-tRNA modifications none

PAICS phosphoribosylaminoimidazole carboxylase and phosphoribosylaminoimidazolesuccinocarboxamide synthase ADE2, ADE2H1, AIRC, PAIS, P22234, gene:10606 0 APEX_matrix, Rickettsial homolog, coexpression Matrix Metabolism > Nucleotide metabolism > Nucleotide synthesis and processing none

MGARP mitochondria localized glutamic acid rich protein C4orf49, CESP-1, HUMMR, OSAP, Q8TDB4, gene:84709 0 literature, APEX_IMS, targetP signal, coexpression Membrane Mitochondrial dynamics and surveillance > Trafficking none

HDHD3 haloacid dehalogenase like hydrolase domain containing 3 2810435D12Rik, C9orf158, Q9BSH5, gene:81932 0 APEX_matrix, MS/MS++ Matrix none cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, adipose, smallintestine, largeintestine, stomach, placenta, testis

CYP11B2 cytochrome P450 family 11 subfamily B member 2 ALDOS, CPN2, CYP11B, CYP11BL, CYPXIB2, P-450C18, P450C18, P450aldo, P19099, gene:1585 0 literature, targetP signal, mito protein domain, coexpression MIM Metabolism > Lipid metabolism > Cholesterol, bile acid, steroid synthesis;
Metabolism > Metals and cofactors > Heme-containing proteins none

AGPAT4 1-acylglycerol-3-phosphate O-acyltransferase 4 1-AGPAT4, LPAAT-delta, dJ473J16.2, Q9NRZ5, gene:56895 0 literature, mito protein domain, induction MOM Metabolism > Lipid metabolism > Cardiolipin synthesis;
Metabolism > Lipid metabolism > Phospholipid metabolism testis

RCC1L RCC1 like WBSCR16, Q96I51, gene:81554 -0 literature, APEX_matrix, targetP signal, coexpression Matrix Mitochondrial central dogma > mtRNA metabolism > mt-rRNA modifications placenta

FAM185A family with sequence similarity 185 member A Q8N0U4, gene:222234 -0 APEX_matrix, targetP signal, coexpression Matrix none none

PMAIP1 phorbol-12-myristate-13-acetate-induced protein 1 APR, NOXA, Q13794, gene:5366 -0 literature, mito protein domain+ MOM Mitochondrial dynamics and surveillance > Apoptosis none

TDRKH tudor and KH domain containing TDRD2, Q9Y2W6, gene:11022 -0 GFP, mito protein domain, MS/MS Membrane none cerebrum, cerebellum, brainstem, spinalcord, adipose, testis

SLC8B1 solute carrier family 8 member B1 NCKX6, NCLX, SLC24A6, Q6J4K2, gene:80024 -0 literature, mito protein domain, induction MIM Signaling > Calcium homeostasis > Calcium cycle;
Small molecule transport placenta

CYP27B1 cytochrome P450 family 27 subfamily B member 1 CP2B, CYP1, CYP1alpha, CYP27B, P450c1, PDDR, VDD1, VDDR, VDDRI, VDR, O15528, gene:1594 -1 literature, targetP signal, mito protein domain MIM Metabolism > Metals and cofactors > Heme-containing proteins;
Metabolism > Vitamin metabolism > Vitamin D metabolism none

POLG2 DNA polymerase gamma 2, accessory subunit HP55, MTDPS16, MTPOLB, PEOA4, POLB, POLG-BETA, POLGB, Q9UHN1, gene:11232 -1 literature, APEX_matrix, targetP signal, mito protein domain Matrix Mitochondrial central dogma > mtDNA maintenance > mtDNA replication;
Mitochondrial central dogma > mtDNA maintenance > mtDNA nucleoid none

THEM5 thioesterase superfamily member 5 ACOT15, Q8N1Q8, gene:284486 -1 literature, targetP signal, mito protein domain Matrix Metabolism > Lipid metabolism none

BIK BCL2 interacting killer BIP1, BP4, NBK, Q13323, gene:638 -1 manual, mito protein domain+ MOM Mitochondrial dynamics and surveillance > Apoptosis none

MTERF1 mitochondrial transcription termination factor 1 MTERF, Q99551, gene:7978 -1 literature, APEX_matrix, mito protein domain+ Matrix Mitochondrial central dogma > mtDNA maintenance > mtDNA nucleoid;
Mitochondrial central dogma > mtRNA metabolism > Transcription none

MTFR2 mitochondrial fission regulator 2 DUFD1, FAM54A, Q6P444, gene:113115 -1 manual, mito protein domain+ Membrane Mitochondrial dynamics and surveillance > Fission none

GPX4 glutathione peroxidase 4 GPx-4, GSHPx-4, MCSP, PHGPx, SMDS, snGPx, snPHGPx, P36969, gene:2879 -1 literature, mito protein domain MIM Metabolism > Detoxification > ROS and glutathione metabolism;
Metabolism > Detoxification > Selenoproteins all 14

TOMM20L translocase of outer mitochondrial membrane 20 like UNQ9438, Q6UXN7, gene:387990 -1 manual, yeast mito homolog+ MOM Protein import, sorting and homeostasis > Protein import and sorting none

COMT catechol-O-methyltransferase HEL-S-98n, P21964, gene:1312 -1 GFP, induction, coexpression Membrane Metabolism > Amino acid metabolism > Catechol metabolism liver

MYG1 MYG1 exonuclease C12orf10, Gamm1, MST024, MSTP024, MYG, Q86UA3, gene:60314 -1 literature, mito protein domain, coexpression Matrix Mitochondrial central dogma > mtRNA metabolism > mt-rRNA modifications;
Mitochondrial central dogma > mtRNA metabolism > mt-mRNA modifications none

SPATA20 spermatogenesis associated 20 HEL-S-98, SSP411, Tisp78, Q8TB22, gene:64847 -1 APEX_matrix, targetP signal Matrix none testis

OXCT2 3-oxoacid CoA-transferase 2 FKSG25, SCOTT, Q9BYC2, gene:64064 -1 manual, targetP signal+, mito protein domain Matrix Metabolism > Carbohydrate metabolism > Ketone metabolism none

CCDC127 coiled-coil domain containing 127 Q96BQ5, gene:133957 -1 APEX_IMS, MS/MS Membrane none cerebrum, cerebellum, brainstem, spinalcord, liver, heart, adipose, smallintestine, largeintestine, stomach, placenta, testis

CRY1 cryptochrome circadian regulator 1 DSPD, PHLL1, Q16526, gene:1407 -1 literature, mito protein domain, coexpression unknown none none

ACSM2B acyl-CoA synthetase medium chain family member 2B ACSM2, HXMA, HYST1046, Q68CK6, gene:348158 -1 manual, targetP signal, mito protein domain Matrix Metabolism > Lipid metabolism > Fatty acid oxidation none

MYO19 myosin XIX MYOHD1, Q96H55, gene:80179 -1 literature, mito protein domain MOM Mitochondrial dynamics and surveillance > Trafficking adipose

MGME1 mitochondrial genome maintenance exonuclease 1 C20orf72, DDK1, MTDPS11, bA504H3.4, Q9BQP7, gene:92667 -2 literature, APEX_matrix, GFP, targetP signal Matrix Mitochondrial central dogma > mtDNA maintenance > mtDNA replication;
Mitochondrial central dogma > mtDNA maintenance > mtDNA stability and decay largeintestine, placenta

SEPTIN4 septin 4 ARTS, BRADEION, C17orf47, CE5B3, H5, MART, PNUTL2, SEP4, SEPT4, hCDCREL-2, hucep-7, O43236, gene:5414 -2 literature, mito protein domain MOM Mitochondrial dynamics and surveillance > Apoptosis brainstem, spinalcord

EFHD1 EF-hand domain family member D1 MST133, MSTP133, PP3051, SWS2, Q9BUP0, gene:80303 -2 literature, mito protein domain, induction MIM Signaling > Calcium homeostasis > EF hand proteins none

RAB5IF RAB5 interacting factor C20orf24, PNAS-11, RCAF1, RIP5, Q9BUV8, gene:55969 -2 GFP, induction, coexpression MIM OXPHOS > OXPHOS assembly factors;
OXPHOS > Respirasome assembly none

APEX1 apurinic/apyrimidinic endodeoxyribonuclease 1 APE, APE1, APEN, APEX, APX, HAP1, REF1, P27695, gene:328 -2 literature, Rickettsial homolog, mito protein domain Matrix Mitochondrial central dogma > mtDNA maintenance > mtDNA repair none

SPTLC2 serine palmitoyltransferase long chain base subunit 2 HSN1C, LCB2, LCB2A, NSAN1C, SPT2, hLCB2a, O15270, gene:9517 -2 GFP, Rickettsial homolog, mito protein domain Membrane Metabolism > Lipid metabolism > Phospholipid metabolism none

DMAC1 distal membrane arm assembly complex 1 C9orf123, TMEM261, Q96GE9, gene:90871 -2 literature, MS/MS MIM OXPHOS > Complex I > CI assembly factors;
OXPHOS > OXPHOS assembly factors cerebrum, spinalcord, kidney, heart, adipose, smallintestine, largeintestine, stomach, placenta, testis

ATPSCKMT ATP synthase c subunit lysine N-methyltransferase FAM173B, JS-2, hFAM173B, Q6P4H8, gene:134145 -2 literature, coexpression MIM OXPHOS > Complex V > CV assembly factors;
OXPHOS > OXPHOS assembly factors placenta

NEU4 neuraminidase 4 Q8WWR8, gene:129807 -2 GFP, targetP signal MOM Metabolism > Carbohydrate metabolism none

CASP9 caspase 9 APAF-3, APAF3, ICE-LAP6, MCH6, PPP1R56, P55211, gene:842 -2 literature, targetP signal, mito protein domain IMS Protein import, sorting and homeostasis > Protein homeostasis > Proteases;
Mitochondrial dynamics and surveillance > Apoptosis none

AGK acylglycerol kinase CATC5, CTRCT38, MTDPS10, MULK, Q53H12, gene:55750 -2 literature, APEX_IMS, mito protein domain MIM Protein import, sorting and homeostasis > Protein import and sorting > TIM22 carrier pathway;
Metabolism > Lipid metabolism > Phospholipid metabolism cerebrum, cerebellum, brainstem, spinalcord, liver, heart, adipose, smallintestine, largeintestine, stomach, placenta, testis

MCL1 MCL1 apoptosis regulator, BCL2 family member BCL2L3, EAT, MCL1-ES, MCL1L, MCL1S, Mcl-1, TM, bcl2-L-3, mcl1/EAT, Q07820, gene:4170 -3 manual, mito protein domain MOM Mitochondrial dynamics and surveillance > Apoptosis adipose, largeintestine, placenta, testis

DUS2 dihydrouridine synthase 2 DUS2L, SMM1, URLC8, Q9NX74, gene:54920 -3 GFP, Rickettsial homolog, mito protein domain Matrix Mitochondrial central dogma > mtRNA metabolism > mt-tRNA modifications none

PLD6 phospholipase D family member 6 ZUC, Q8N2A8, gene:201164 -3 literature, Rickettsial homolog, mito protein domain MOM Metabolism > Lipid metabolism > Cardiolipin synthesis;
Mitochondrial dynamics and surveillance > Fusion none

TOMM34 translocase of outer mitochondrial membrane 34 HTOM34P, TOM34, URCC3, Q15785, gene:10953 -3 literature, mito protein domain MOM Protein import, sorting and homeostasis > Protein import and sorting > TOM testis

PRORP protein only RNase P catalytic subunit KIAA0391, MRPP3, O15091, gene:9692 -3 APEX_matrix Matrix Mitochondrial central dogma > mtRNA metabolism > mtRNA granules;
Mitochondrial central dogma > mtRNA metabolism > Polycistronic mtRNA processing spinalcord, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis

PLSCR3 phospholipid scramblase 3 Q9NRY6, gene:57048 -3 literature, mito protein domain MIM Metabolism > Lipid metabolism > Cardiolipin synthesis none

CKMT1B creatine kinase, mitochondrial 1B CKMT, CKMT1, UMTCK, 0, gene:1159 -3 literature, APEX_IMS, targetP signal, mito protein domain MIM Metabolism > Nucleotide metabolism > Creatine metabolism none

NDUFB1 NADH:ubiquinone oxidoreductase subunit B1 CI-MNLL, CI-SGDH, MNLL, O75438, gene:4707 -3 literature, mito protein domain+ MIM OXPHOS > Complex I > CI subunits;
OXPHOS > OXPHOS subunits none

ATP5MF-PTCD1 ATP5MF-PTCD1 readthrough ATP5J2-PTCD1, B4DJ38, gene:100526740 -3 APEX_matrix, mito protein domain+ unknown none none

CASP8 caspase 8 ALPS2B, CAP4, Casp-8, FLICE, MACH, MCH5, Q14790, gene:841 -3 literature, mito protein domain MOM Protein import, sorting and homeostasis > Protein homeostasis;
Mitochondrial dynamics and surveillance > Apoptosis none

BCL2L1 BCL2 like 1 BCL-XL/S, BCL2L, BCLX, Bcl-X, PPP1R52, Q07817, gene:598 -3 literature, mito protein domain MOM Mitochondrial dynamics and surveillance > Apoptosis cerebrum, cerebellum, placenta

EXD2 exonuclease 3'-5' domain containing 2 C14orf114, EXDL2, Q9NVH0, gene:55218 -3 literature, mito protein domain Matrix Mitochondrial central dogma > Translation cerebrum, cerebellum, testis

ARMC10 armadillo repeat containing 10 PNAS-112, PNAS112, PSEC0198, SVH, Q8N2F6, gene:83787 -4 GFP, MS/MS MOM Mitochondrial dynamics and surveillance > Fission;
Mitochondrial dynamics and surveillance > Trafficking cerebrum, cerebellum, brainstem, spinalcord, liver, adipose, largeintestine, stomach, placenta, testis

MIEF2 mitochondrial elongation factor 2 MID49, SMCR7, Q96C03, gene:125170 -4 literature, mito protein domain MOM Mitochondrial dynamics and surveillance > Fission none

THG1L tRNA-histidine guanylyltransferase 1 like ICF45, IHG-1, IHG1, SCAR28, THG1, hTHG1, Q9NWX6, gene:54974 -4 literature, targetP signal, coexpression Matrix Mitochondrial central dogma > mtRNA metabolism > mt-tRNA modifications none

GOLPH3 golgi phosphoprotein 3 GOPP1, GPP34, MIDAS, Vps74, Q9H4A6, gene:64083 -4 literature, induction IMS none none

PREPL prolyl endopeptidase like CMS22, Q4J6C6, gene:9581 -4 APEX_matrix, Rickettsial homolog Matrix none spinalcord, largeintestine

FASTKD3 FAST kinase domains 3 Q14CZ7, gene:79072 -4 APEX_matrix, targetP signal Matrix Mitochondrial central dogma > mtRNA metabolism > Polycistronic mtRNA processing;
Mitochondrial central dogma > mtRNA metabolism > mtRNA stability and decay none

CEP89 centrosomal protein 89 CCDC123, CEP123, Q96ST8, gene:84902 -4 literature, targetP signal IMS OXPHOS > Complex IV > CIV assembly factors;
OXPHOS > OXPHOS assembly factors none

GPAT2 glycerol-3-phosphate acyltransferase 2, mitochondrial CT123, Q6NUI2, gene:150763 -5 manual MOM Metabolism > Lipid metabolism > Cardiolipin synthesis;
Metabolism > Lipid metabolism > Phospholipid metabolism testis

NBR1 NBR1 autophagy cargo receptor 1A1-3B, IAI3B, M17S2, MIG19, Q14596, gene:4077 -5 APEX_IMS, coexpression IMS Mitochondrial dynamics and surveillance > Autophagy cerebellum, liver

BOK BCL2 family apoptosis regulator BOK BCL2L9, BOKL, Q9UMX3, gene:666 -5 manual, mito protein domain MOM Mitochondrial dynamics and surveillance > Apoptosis none

FKBP10 FKBP prolyl isomerase 10 BRKS1, FKBP65, OI11, OI6, PPIASE, hFKBP65, Q96AY3, gene:60681 -5 APEX_IMS, mito protein domain IMS Protein import, sorting and homeostasis > Protein homeostasis > Chaperones none

SETD9 SET domain containing 9 C5orf35, Q8NE22, gene:133383 -5 APEX_matrix, targetP signal Matrix none none

CYP11B1 cytochrome P450 family 11 subfamily B member 1 CPN1, CYP11B, FHI, P450C11, P15538, gene:1584 -5 manual, targetP signal, mito protein domain MIM Metabolism > Lipid metabolism > Cholesterol, bile acid, steroid synthesis;
Metabolism > Metals and cofactors > Heme-containing proteins none

CSKMT citrate synthase lysine methyltransferase CS-KMT, METTL12, U99HG, A8MUP2, gene:751071 -5 literature, targetP signal, mito protein domain Matrix Signaling none

MIGA1 mitoguardin 1 FAM73A, Q8NAN2, gene:374986 -5 literature MOM Mitochondrial dynamics and surveillance > Fusion cerebrum, cerebellum, brainstem, spinalcord, smallintestine, testis

RPIA ribose 5-phosphate isomerase A RPI, RPIAD, P49247, gene:22934 -5 APEX_matrix Matrix Metabolism > Carbohydrate metabolism placenta

BBC3 BCL2 binding component 3 JFY-1, JFY1, PUMA, Q96PG8, gene:27113 -6 manual MOM Mitochondrial dynamics and surveillance > Apoptosis none

BAD BCL2 associated agonist of cell death BBC2, BCL2L8, Q92934, gene:572 -6 literature, mito protein domain MOM Mitochondrial dynamics and surveillance > Apoptosis none

BCL2 BCL2 apoptosis regulator Bcl-2, PPP1R50, P10415, gene:596 -6 literature, mito protein domain MOM Mitochondrial dynamics and surveillance > Apoptosis none

BCL2A1 BCL2 related protein A1 ACC-1, ACC-2, ACC1, ACC2, BCL2L5, BFL1, GRS, HBPA1, Q16548, gene:597 -6 manual, mito protein domain MOM Mitochondrial dynamics and surveillance > Apoptosis none

BCL2L2 BCL2 like 2 BCL-W, BCL2-L-2, BCLW, PPP1R51, Q92843, gene:599 -6 literature, mito protein domain MOM Mitochondrial dynamics and surveillance > Apoptosis none

CASP3 caspase 3 CPP32, CPP32B, SCA-1, P42574, gene:836 -6 literature, mito protein domain IMS Protein import, sorting and homeostasis > Protein homeostasis > Proteases;
Mitochondrial dynamics and surveillance > Apoptosis none

DMPK DM1 protein kinase DM, DM1, DM1PK, DMK, MDPK, MT-PK, Q09013, gene:1760 -6 literature, mito protein domain MOM none none

DNA2 DNA replication helicase/nuclease 2 DNA2L, hDNA2, P51530, gene:1763 -6 literature, mito protein domain Matrix Mitochondrial central dogma > mtDNA maintenance > mtDNA replication;
Metabolism > Metals and cofactors > Fe-S-containing proteins none

MT-ND6 NADH dehydrogenase, subunit 6 (complex I) MTND6, P03923, gene:4541 -6 literature, mito protein domain MIM OXPHOS > Complex I > CI subunits;
OXPHOS > OXPHOS subunits none

PRKN parkin RBR E3 ubiquitin protein ligase AR-JP, LPRS2, PARK2, PDJ, O60260, gene:5071 -6 manual, mito protein domain MOM Mitochondrial dynamics and surveillance > Mitophagy;
Mitochondrial dynamics and surveillance > Autophagy none

ALKBH1 alkB homolog 1, histone H2A dioxygenase ABH, ABH1, ALKBH, alkB, hABH, Q13686, gene:8846 -6 literature, APEX_matrix, mito protein domain Matrix Mitochondrial central dogma > mtRNA metabolism > mtRNA granules;
Mitochondrial central dogma > mtRNA metabolism > mt-tRNA modifications none

PICK1 protein interacting with PRKCA 1 PICK, PRKCABP, Q9NRD5, gene:9463 -6 literature, mito protein domain unknown none none

BCL2L10 BCL2 like 10 BCL-B, Boo, Diva, bcl2-L-10, Q9HD36, gene:10017 -6 manual, mito protein domain MOM Mitochondrial dynamics and surveillance > Apoptosis none

AKAP10 A-kinase anchoring protein 10 AKAP-10, D-AKAP-2, D-AKAP2, PRKA10, O43572, gene:11216 -6 literature, mito protein domain MOM Signaling > cAMP-PKA signaling none

STYXL1 serine/threonine/tyrosine interacting like 1 DUSP24, MK-STYX, MKSTYX, Q9Y6J8, gene:51657 -6 literature, mito protein domain Matrix Mitochondrial dynamics and surveillance > Apoptosis none

NSUN2 NOP2/Sun RNA methyltransferase 2 MISU, MRT5, SAKI, TRM4, Q08J23, gene:54888 -6 literature, mito protein domain Matrix Mitochondrial central dogma > mtRNA metabolism > mt-tRNA modifications none

ADCY10 adenylate cyclase 10 HCA2, HEL-S-7a, SAC, SACI, Sacy, hsAC, Q96PN6, gene:55811 -6 literature, mito protein domain Membrane Signaling > cAMP-PKA signaling none

SPHK2 sphingosine kinase 2 SK 2, SK-2, SPK 2, SPK-2, Q9NRA0, gene:56848 -6 literature, mito protein domain MIM Metabolism > Lipid metabolism > Phospholipid metabolism;
Mitochondrial dynamics and surveillance > Apoptosis none

NSUN3 NOP2/Sun RNA methyltransferase 3 MST077, MSTP077, Q9H649, gene:63899 -6 APEX_matrix, mito protein domain Matrix Mitochondrial central dogma > mtRNA metabolism > mt-tRNA modifications none

PABPC5 poly(A) binding protein cytoplasmic 5 PABP5, Q96DU9, gene:140886 -6 literature, mito protein domain Matrix none none

PRSS35 serine protease 35 C6orf158, dJ223E3.1, Q8N3Z0, gene:167681 -6 GFP, mito protein domain unknown Protein import, sorting and homeostasis > Protein homeostasis none

PRIMPOL primase and DNA directed polymerase CCDC111, MYP22, Primpol1, Q96LW4, gene:201973 -6 literature, mito protein domain Matrix Mitochondrial central dogma > mtDNA maintenance > mtDNA repair none

SERAC1 serine active site containing 1 Q96JX3, gene:84947 -6 literature, APEX_IMS MOM Metabolism > Lipid metabolism > Phospholipid metabolism cerebrum, cerebellum, skeletalmuscle, stomach, testis

STX17 syntaxin 17 P56962, gene:55014 -6 APEX_IMS, coexpression MOM Mitochondrial dynamics and surveillance > Fission;
Mitochondrial dynamics and surveillance > Organelle contact sites;
Mitochondrial dynamics and surveillance > Autophagy testis

TRMT61B tRNA methyltransferase 61B Q9BVS5, gene:55006 -7 APEX_matrix, mito protein domain Matrix Mitochondrial central dogma > mtRNA metabolism > mt-tRNA modifications;
Mitochondrial central dogma > mtRNA metabolism > mt-rRNA modifications;
Mitochondrial central dogma > Translation > Mitochondrial ribosome assembly none

PDE2A phosphodiesterase 2A CGS-PDE, PDE2A1, PED2A4, cGSPDE, O00408, gene:5138 -7 literature MIM Signaling > cAMP-PKA signaling cerebrum, cerebellum, brainstem, largeintestine

PLGRKT plasminogen receptor with a C-terminal lysine AD025, C9orf46, MDS030, PLG-RKT, Plg-R(KT), Q9HBL7, gene:55848 -7 GFP Membrane none cerebrum, cerebellum, brainstem, spinalcord, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis

ETFBKMT electron transfer flavoprotein subunit beta lysine methyltransferase C12orf72, ETFB-KMT, METTL20, Q8IXQ9, gene:254013 -7 literature, targetP signal Matrix Metabolism > Electron carriers > Q-linked reactions, other none

RTL10 retrotransposon Gag like 10 BOP, C22orf29, Q7L3V2, gene:79680 -7 manual, targetP signal MOM Mitochondrial dynamics and surveillance > Apoptosis none

BCL2L11 BCL2 like 11 BAM, BIM, BOD, O43521, gene:10018 -8 manual MOM Mitochondrial dynamics and surveillance > Apoptosis none

SPIRE1 spire type actin nucleation factor 1 Spir-1, Q08AE8, gene:56907 -8 literature MOM Mitochondrial dynamics and surveillance > Fission;
Mitochondrial dynamics and surveillance > Organelle contact sites none

C2orf69 chromosome 2 open reading frame 69 Q8N8R5, gene:205327 -8 APEX_matrix Matrix none none

ARMCX2 armadillo repeat containing X-linked 2 ALEX2, GASP9, Q7L311, gene:9823 -10 literature Membrane none none

ARMCX1 armadillo repeat containing X-linked 1 ALEX1, GASP7, Q9P291, gene:51309 -10 literature MOM Mitochondrial dynamics and surveillance > Trafficking none

POLB DNA polymerase beta P06746, gene:5423 -10 literature Matrix Mitochondrial central dogma > mtDNA maintenance > mtDNA repair none

SNAP29 synaptosome associated protein 29 CEDNIK, SNAP-29, O95721, gene:9342 -10 APEX_IMS IMS Mitochondrial dynamics and surveillance > Autophagy none

ARMCX3 armadillo repeat containing X-linked 3 ALEX3, GASP6, dJ545K15.2, Q9UH62, gene:51566 -10 literature MOM Mitochondrial dynamics and surveillance > Trafficking none

ARMCX6 armadillo repeat containing X-linked 6 GASP10, Q7L4S7, gene:54470 -10 literature Membrane none none

METTL4 methyltransferase like 4 HsT661, Q8N3J2, gene:64863 -10 literature Matrix Mitochondrial central dogma > mtDNA maintenance > mtDNA modifications none

SPHKAP SPHK1 interactor, AKAP domain containing SKIP, Q2M3C7, gene:80309 -10 manual MIM Signaling > cAMP-PKA signaling none

NAT8L N-acetyltransferase 8 like CML3, NACED, NAT8-LIKE, Q8N9F0, gene:339983 -10 literature Membrane Metabolism > Amino acid metabolism > Glutamate metabolism none

MCCD1 mitochondrial coiled-coil domain 1 P59942, gene:401250 -10 manual unknown none none

PIGBOS1 PIGB opposite strand 1 HP06981, A0A0B4J2F0, gene:101928527 NA literature MOM Mitochondrial dynamics and surveillance > Organelle contact sites NA

HTD2 hydroxyacyl-thioester dehydratase type 2 P86397, gene:109703458 NA literature Matrix Metabolism > Lipid metabolism > Type II fatty acid synthesis NA

RP11_469A15.2 RP11_469A15.2 0, gene:ENSG00000230623 NA literature MIM NA