MitoPathways3.0: 149 hierarchical pathways

MitoCarta3.0 genes have been annotated into a hierarchy of 149 biological pathways based on literature curation. More complete information is available for download in file Human.MitoCarta3.0.xls and Mouse.MitoCarta3.0.xls. See also MitoCarta3.0 home for a description of the project. Colored by best bidirectional hit (BIDI) present in C.merolae based on BlastP : Cme_top_hit, Cme_BBH, No_Cme_hit, unknown;

MitoPathwayMitoPathways HierarchyHuman MitoCarta3.0 Genes
Mitochondrial central dogma Mitochondrial central dogma AARS2, ALKBH1, ANGEL2, APEX1, ATAD3A, ATAD3B, AURKAIP1, C12orf65, CARS2, CDK5RAP1, CHCHD1, COA3, COX14, DAP3, DARS2, DDX28, DHX30, DNA2, DUS2, EARS2, ELAC2, ENDOG, ERAL1, EXD2, EXOG, FARS2, FASTK, FASTKD1, FASTKD2, FASTKD3, FASTKD5, GADD45GIP1, GARS1, GATB, GATC, GFM1, GFM2, GRSF1, GTPBP10, GTPBP3, GUF1, HARS2, HEMK1, HSD17B10, IARS2, KARS1, LACTB2, LARS2, LIG3, LRPPRC, MALSU1, MARS2, METAP1D, METTL15, METTL17, METTL4, METTL5, METTL8, MGME1, MIEF1, MPV17L2, MRM1, MRM2, MRM3, MRPL1, MRPL10, MRPL11, MRPL12, MRPL13, MRPL14, MRPL15, MRPL16, MRPL17, MRPL18, MRPL19, MRPL2, MRPL20, MRPL21, MRPL22, MRPL23, MRPL24, MRPL27, MRPL28, MRPL3, MRPL30, MRPL32, MRPL33, MRPL34, MRPL35, MRPL36, MRPL37, MRPL38, MRPL39, MRPL4, MRPL40, MRPL41, MRPL42, MRPL43, MRPL44, MRPL45, MRPL46, MRPL47, MRPL48, MRPL49, MRPL50, MRPL51, MRPL52, MRPL53, MRPL54, MRPL55, MRPL57, MRPL58, MRPL9, MRPS10, MRPS11, MRPS12, MRPS14, MRPS15, MRPS16, MRPS17, MRPS18A, MRPS18B, MRPS18C, MRPS2, MRPS21, MRPS22, MRPS23, MRPS24, MRPS25, MRPS26, MRPS27, MRPS28, MRPS30, MRPS31, MRPS33, MRPS34, MRPS35, MRPS36, MRPS5, MRPS6, MRPS7, MRPS9, MRRF, MTERF1, MTERF2, MTERF3, MTERF4, MTFMT, MTG1, MTG2, MTIF2, MTIF3, MTO1, MTPAP, MTRES1, MTRF1, MTRF1L, MUTYH, MYG1, NARS2, NGRN, NOA1, NSUN2, NSUN3, NSUN4, OGG1, OSGEPL1, OXA1L, PARS2, PDE12, PDF, PIF1, PNPT1, POLB, POLDIP2, POLG, POLG2, POLQ, POLRMT, PPA2, PRIMPOL, PRORP, PTCD1, PTCD2, PTCD3, PUS1, PUSL1, QRSL1, QTRT1, RARS2, RBFA, RCC1L, RECQL4, REXO2, RMND1, RNASEH1, RPUSD3, RPUSD4, SARS2, SLIRP, SSBP1, SUPV3L1, TACO1, TARS2, TBRG4, TEFM, TFAM, TFB1M, TFB2M, THG1L, TIMM21, TOP1MT, TOP3A, TRIT1, TRMT1, TRMT10C, TRMT2B, TRMT5, TRMT61B, TRMU, TRNT1, TRUB2, TSFM, TUFM, TWNK, UNG, VARS2, WARS2, YARS2, YBEY, YRDC
mtDNA maintenance Mitochondrial central dogma > mtDNA maintenance APEX1, ATAD3A, ATAD3B, DNA2, ENDOG, EXOG, LIG3, METTL4, MGME1, MTERF1, MTERF2, MUTYH, OGG1, PIF1, POLB, POLDIP2, POLG, POLG2, POLQ, POLRMT, PPA2, PRIMPOL, RECQL4, RNASEH1, SSBP1, TFAM, TFB2M, TOP1MT, TOP3A, TWNK, UNG
mtDNA replication Mitochondrial central dogma > mtDNA maintenance > mtDNA replication DNA2, EXOG, LIG3, MGME1, POLG, POLG2, POLRMT, RNASEH1, SSBP1, TFAM, TFB2M, TOP1MT, TOP3A, TWNK
mtDNA nucleoid Mitochondrial central dogma > mtDNA maintenance > mtDNA nucleoid ATAD3A, ATAD3B, MTERF1, MTERF2, POLDIP2, POLG, POLG2, POLRMT, SSBP1, TFAM, TWNK
mtDNA repair Mitochondrial central dogma > mtDNA maintenance > mtDNA repair APEX1, EXOG, LIG3, OGG1, POLB, POLG, PRIMPOL, RECQL4, UNG
mtDNA modifications Mitochondrial central dogma > mtDNA maintenance > mtDNA modifications METTL4
mtDNA stability and decay Mitochondrial central dogma > mtDNA maintenance > mtDNA stability and decay ENDOG, EXOG, MGME1
mtRNA metabolism Mitochondrial central dogma > mtRNA metabolism ALKBH1, ANGEL2, CDK5RAP1, DDX28, DHX30, DUS2, ELAC2, ENDOG, ERAL1, FASTK, FASTKD1, FASTKD2, FASTKD3, FASTKD5, GRSF1, GTPBP3, HSD17B10, LACTB2, LRPPRC, METTL15, METTL17, METTL5, METTL8, MRM1, MRM2, MRM3, MRPL12, MRPL47, MRPS7, MRPS9, MTERF1, MTO1, MTPAP, MTRES1, MYG1, NGRN, NOA1, NSUN2, NSUN3, NSUN4, OSGEPL1, PDE12, PNPT1, POLRMT, PPA2, PRORP, PTCD1, PTCD2, PUS1, QTRT1, RCC1L, REXO2, RMND1, RNASEH1, RPUSD3, RPUSD4, SLIRP, SUPV3L1, TBRG4, TEFM, TFAM, TFB1M, TFB2M, THG1L, TOP1MT, TRIT1, TRMT1, TRMT10C, TRMT2B, TRMT5, TRMT61B, TRMU, TRNT1, TRUB2, YBEY, YRDC
Transcription Mitochondrial central dogma > mtRNA metabolism > Transcription MRPL12, MTERF1, MTRES1, POLRMT, TEFM, TFAM, TFB2M, TOP1MT
mtRNA granules Mitochondrial central dogma > mtRNA metabolism > mtRNA granules ALKBH1, DDX28, DHX30, ERAL1, FASTK, FASTKD1, FASTKD2, FASTKD5, GRSF1, HSD17B10, MRM1, MRM2, MRPL47, MRPS7, MRPS9, MTPAP, NOA1, PRORP, PTCD1, PTCD2, PUS1, RMND1, RPUSD3, RPUSD4, SUPV3L1, TFB1M, TRMT10C, TRUB2
Polycistronic mtRNA processing Mitochondrial central dogma > mtRNA metabolism > Polycistronic mtRNA processing ELAC2, FASTK, FASTKD1, FASTKD2, FASTKD3, FASTKD5, GRSF1, HSD17B10, PRORP, PTCD2, TBRG4, TRMT10C, YBEY
mt-tRNA modifications Mitochondrial central dogma > mtRNA metabolism > mt-tRNA modifications ALKBH1, CDK5RAP1, DUS2, GTPBP3, HSD17B10, MTO1, NSUN2, NSUN3, OSGEPL1, PUS1, QTRT1, RPUSD4, THG1L, TRIT1, TRMT1, TRMT10C, TRMT2B, TRMT5, TRMT61B, TRMU, TRNT1, YRDC
mt-rRNA modifications Mitochondrial central dogma > mtRNA metabolism > mt-rRNA modifications METTL15, METTL17, METTL5, MRM1, MRM2, MRM3, MTPAP, MYG1, NGRN, NSUN4, RCC1L, RPUSD3, RPUSD4, TFB1M, TRIT1, TRMT2B, TRMT61B, TRUB2
mt-mRNA modifications Mitochondrial central dogma > mtRNA metabolism > mt-mRNA modifications ANGEL2, MTPAP, MYG1, PDE12
mtRNA stability and decay Mitochondrial central dogma > mtRNA metabolism > mtRNA stability and decay ENDOG, FASTK, FASTKD1, FASTKD2, FASTKD3, FASTKD5, GRSF1, LACTB2, LRPPRC, MTPAP, PDE12, PNPT1, REXO2, RNASEH1, SLIRP, SUPV3L1, TBRG4
Translation Mitochondrial central dogma > Translation AARS2, AURKAIP1, C12orf65, CARS2, CHCHD1, COA3, COX14, DAP3, DARS2, DDX28, DHX30, EARS2, ERAL1, EXD2, FARS2, FASTKD2, GADD45GIP1, GARS1, GATB, GATC, GFM1, GFM2, GRSF1, GTPBP10, GUF1, HARS2, HEMK1, IARS2, KARS1, LARS2, LRPPRC, MALSU1, MARS2, METAP1D, METTL17, MIEF1, MPV17L2, MRM2, MRM3, MRPL1, MRPL10, MRPL11, MRPL12, MRPL13, MRPL14, MRPL15, MRPL16, MRPL17, MRPL18, MRPL19, MRPL2, MRPL20, MRPL21, MRPL22, MRPL23, MRPL24, MRPL27, MRPL28, MRPL3, MRPL30, MRPL32, MRPL33, MRPL34, MRPL35, MRPL36, MRPL37, MRPL38, MRPL39, MRPL4, MRPL40, MRPL41, MRPL42, MRPL43, MRPL44, MRPL45, MRPL46, MRPL47, MRPL48, MRPL49, MRPL50, MRPL51, MRPL52, MRPL53, MRPL54, MRPL55, MRPL57, MRPL58, MRPL9, MRPS10, MRPS11, MRPS12, MRPS14, MRPS15, MRPS16, MRPS17, MRPS18A, MRPS18B, MRPS18C, MRPS2, MRPS21, MRPS22, MRPS23, MRPS24, MRPS25, MRPS26, MRPS27, MRPS28, MRPS30, MRPS31, MRPS33, MRPS34, MRPS35, MRPS36, MRPS5, MRPS6, MRPS7, MRPS9, MRRF, MTERF3, MTERF4, MTFMT, MTG1, MTG2, MTIF2, MTIF3, MTRES1, MTRF1, MTRF1L, NARS2, NGRN, NOA1, NSUN4, OXA1L, PARS2, PDF, PPA2, PTCD3, PUSL1, QRSL1, RARS2, RBFA, RMND1, SARS2, SLIRP, TACO1, TARS2, TFB1M, TIMM21, TRMT61B, TSFM, TUFM, VARS2, WARS2, YARS2, YBEY
Mitochondrial ribosome Mitochondrial central dogma > Translation > Mitochondrial ribosome AURKAIP1, CHCHD1, DAP3, GADD45GIP1, MRPL1, MRPL10, MRPL11, MRPL12, MRPL13, MRPL14, MRPL15, MRPL16, MRPL17, MRPL18, MRPL19, MRPL2, MRPL20, MRPL21, MRPL22, MRPL23, MRPL24, MRPL27, MRPL28, MRPL3, MRPL30, MRPL32, MRPL33, MRPL34, MRPL35, MRPL36, MRPL37, MRPL38, MRPL39, MRPL4, MRPL40, MRPL41, MRPL42, MRPL43, MRPL44, MRPL45, MRPL46, MRPL47, MRPL48, MRPL49, MRPL50, MRPL51, MRPL52, MRPL53, MRPL54, MRPL55, MRPL57, MRPL58, MRPL9, MRPS10, MRPS11, MRPS12, MRPS14, MRPS15, MRPS16, MRPS17, MRPS18A, MRPS18B, MRPS18C, MRPS2, MRPS21, MRPS22, MRPS23, MRPS24, MRPS25, MRPS26, MRPS27, MRPS28, MRPS30, MRPS31, MRPS33, MRPS34, MRPS35, MRPS36, MRPS5, MRPS6, MRPS7, MRPS9, PTCD3
Mitochondrial ribosome assembly Mitochondrial central dogma > Translation > Mitochondrial ribosome assembly DDX28, DHX30, ERAL1, FASTKD2, GRSF1, GTPBP10, MALSU1, METTL17, MIEF1, MPV17L2, MRM2, MRM3, MTERF3, MTERF4, MTG1, MTG2, NGRN, NOA1, NSUN4, PTCD3, RBFA, TFB1M, TRMT61B, YBEY
Translation factors Mitochondrial central dogma > Translation > Translation factors C12orf65, COA3, COX14, GFM1, GFM2, HEMK1, MRPL58, MRRF, MTIF2, MTIF3, MTRF1, MTRF1L, TACO1, TIMM21, TSFM, TUFM
mt-tRNA synthetases Mitochondrial central dogma > Translation > mt-tRNA synthetases AARS2, CARS2, DARS2, EARS2, FARS2, GARS1, GATB, GATC, HARS2, IARS2, KARS1, LARS2, MARS2, NARS2, PARS2, QRSL1, RARS2, SARS2, TARS2, VARS2, WARS2, YARS2
fMet processing Mitochondrial central dogma > Translation > fMet processing METAP1D, MTFMT, PDF
Protein import, sorting and homeostasis Protein import, sorting and homeostasis AFG1L, AFG3L2, AGK, AIFM1, ATAD1, ATP23, CASP3, CASP8, CASP9, CHCHD4, CLPB, CLPP, CLPX, DNAJA3, DNAJC11, DNAJC15, DNAJC19, DNAJC4, DNLZ, FKBP10, GFER, GRPEL1, GRPEL2, HSPD1, HSPE1, HTRA2, IDE, IMMP1L, IMMP2L, LACTB, LAP3, LONP1, MAIP1, METAP1D, MIPEP, MTX1, MTX2, MUL1, NLN, NRDC, OMA1, OXA1L, PAM16, PARK7, PARL, PDF, PHB, PHB2, PITRM1, PMPCA, PMPCB, PRSS35, ROMO1, SAMM50, SPG7, STOML2, TIMM10, TIMM10B, TIMM13, TIMM17A, TIMM17B, TIMM21, TIMM22, TIMM23, TIMM29, TIMM44, TIMM50, TIMM8A, TIMM8B, TIMM9, TOMM20, TOMM20L, TOMM22, TOMM34, TOMM40, TOMM40L, TOMM5, TOMM6, TOMM7, TOMM70, TRAP1, UQCRC1, UQCRC2, USP30, XPNPEP3, YME1L1
Protein import and sorting Protein import, sorting and homeostasis > Protein import and sorting AGK, AIFM1, CHCHD4, DNAJC11, DNAJC15, DNAJC19, GFER, GRPEL1, GRPEL2, IMMP1L, IMMP2L, MIPEP, MTX1, MTX2, NRDC, OXA1L, PAM16, PMPCA, PMPCB, ROMO1, SAMM50, TIMM10, TIMM10B, TIMM13, TIMM17A, TIMM17B, TIMM21, TIMM22, TIMM23, TIMM29, TIMM44, TIMM50, TIMM8A, TIMM8B, TIMM9, TOMM20, TOMM20L, TOMM22, TOMM34, TOMM40, TOMM40L, TOMM5, TOMM6, TOMM7, TOMM70, UQCRC1, UQCRC2, XPNPEP3
TOM Protein import, sorting and homeostasis > Protein import and sorting > TOM TOMM20, TOMM22, TOMM34, TOMM40, TOMM40L, TOMM5, TOMM6, TOMM7, TOMM70
SAM Protein import, sorting and homeostasis > Protein import and sorting > SAM MTX1, MTX2, SAMM50
MIA40 Protein import, sorting and homeostasis > Protein import and sorting > MIA40 AIFM1, CHCHD4, GFER
TIM22 carrier pathway Protein import, sorting and homeostasis > Protein import and sorting > TIM22 carrier pathway AGK, TIMM22, TIMM29
TIM23 presequence pathway Protein import, sorting and homeostasis > Protein import and sorting > TIM23 presequence pathway ROMO1, TIMM17A, TIMM17B, TIMM21, TIMM23, TIMM50
Import motor Protein import, sorting and homeostasis > Protein import and sorting > Import motor DNAJC15, DNAJC19, GRPEL1, GRPEL2, PAM16, TIMM44
OXA Protein import, sorting and homeostasis > Protein import and sorting > OXA OXA1L
Preprotein cleavage Protein import, sorting and homeostasis > Protein import and sorting > Preprotein cleavage IMMP1L, IMMP2L, MIPEP, PMPCA, PMPCB, UQCRC1, UQCRC2, XPNPEP3
Protein homeostasis Protein import, sorting and homeostasis > Protein homeostasis AFG1L, AFG3L2, ATAD1, ATP23, CASP3, CASP8, CASP9, CLPB, CLPP, CLPX, DNAJA3, DNAJC4, DNLZ, FKBP10, HSPD1, HSPE1, HTRA2, IDE, IMMP1L, IMMP2L, LACTB, LAP3, LONP1, MAIP1, METAP1D, MIPEP, MUL1, NLN, NRDC, OMA1, PARK7, PARL, PDF, PHB, PHB2, PITRM1, PMPCA, PMPCB, PRSS35, SPG7, STOML2, TIMM10, TIMM10B, TIMM13, TIMM8A, TIMM8B, TIMM9, TRAP1, USP30, XPNPEP3, YME1L1
Proteases Protein import, sorting and homeostasis > Protein homeostasis > Proteases AFG1L, AFG3L2, ATAD1, ATP23, CASP3, CASP9, CLPB, CLPP, CLPX, HTRA2, IDE, IMMP1L, IMMP2L, LACTB, LAP3, LONP1, METAP1D, MIPEP, NLN, OMA1, PARL, PITRM1, PMPCA, PMPCB, SPG7, USP30, XPNPEP3, YME1L1
Chaperones Protein import, sorting and homeostasis > Protein homeostasis > Chaperones DNAJA3, DNLZ, FKBP10, HSPD1, HSPE1, MAIP1, NRDC, PHB, PHB2, TIMM10, TIMM10B, TIMM13, TIMM8A, TIMM8B, TIMM9, TRAP1
OXPHOS OXPHOS ACAD9, AIFM1, ATP5F1A, ATP5F1B, ATP5F1C, ATP5F1D, ATP5F1E, ATP5IF1, ATP5MC1, ATP5MC2, ATP5MC3, ATP5MD, ATP5ME, ATP5MF, ATP5MG, ATP5MPL, ATP5PB, ATP5PD, ATP5PF, ATP5PO, ATPAF1, ATPAF2, ATPSCKMT, BCS1L, CEP89, CMC1, CMC2, COA1, COA3, COA4, COA5, COA6, COA7, COA8, COX10, COX11, COX14, COX15, COX16, COX17, COX18, COX19, COX20, COX4I1, COX4I2, COX5A, COX5B, COX6A1, COX6A2, COX6B1, COX6B2, COX6C, COX7A1, COX7A2, COX7A2L, COX7B, COX7B2, COX7C, COX8A, COX8C, CYC1, CYCS, DMAC1, DMAC2, DMAC2L, ECSIT, FMC1, FOXRED1, HCCS, HIGD1A, HIGD2A, LYRM2, LYRM7, MT-ATP6, MT-ATP8, MT-CO1, MT-CO2, MT-CO3, MT-CYB, MT-ND1, MT-ND2, MT-ND3, MT-ND4, MT-ND4L, MT-ND5, MT-ND6, NDUFA1, NDUFA10, NDUFA11, NDUFA12, NDUFA13, NDUFA2, NDUFA3, NDUFA4, NDUFA5, NDUFA6, NDUFA7, NDUFA8, NDUFA9, NDUFAB1, NDUFAF1, NDUFAF2, NDUFAF3, NDUFAF4, NDUFAF5, NDUFAF6, NDUFAF7, NDUFAF8, NDUFB1, NDUFB10, NDUFB11, NDUFB2, NDUFB3, NDUFB4, NDUFB5, NDUFB6, NDUFB7, NDUFB8, NDUFB9, NDUFC1, NDUFC2, NDUFS1, NDUFS2, NDUFS3, NDUFS4, NDUFS5, NDUFS6, NDUFS7, NDUFS8, NDUFV1, NDUFV2, NDUFV3, NUBPL, PET100, PET117, PNKD, RAB5IF, SCO1, SCO2, SDHA, SDHAF1, SDHAF2, SDHAF3, SDHAF4, SDHB, SDHC, SDHD, SMIM20, SURF1, TACO1, TIMM21, TIMMDC1, TMEM126A, TMEM126B, TMEM177, TMEM186, TMEM70, TTC19, UQCC1, UQCC2, UQCC3, UQCR10, UQCR11, UQCRB, UQCRC1, UQCRC2, UQCRFS1, UQCRH, UQCRQ
OXPHOS
subunits
OXPHOS > OXPHOS subunits ATP5F1A, ATP5F1B, ATP5F1C, ATP5F1D, ATP5F1E, ATP5IF1, ATP5MC1, ATP5MC2, ATP5MC3, ATP5MD, ATP5ME, ATP5MF, ATP5MG, ATP5MPL, ATP5PB, ATP5PD, ATP5PF, ATP5PO, COX4I1, COX4I2, COX5A, COX5B, COX6A1, COX6A2, COX6B1, COX6B2, COX6C, COX7A1, COX7A2, COX7A2L, COX7B, COX7B2, COX7C, COX8A, COX8C, CYC1, CYCS, DMAC2L, HCCS, MT-ATP6, MT-ATP8, MT-CO1, MT-CO2, MT-CO3, MT-CYB, MT-ND1, MT-ND2, MT-ND3, MT-ND4, MT-ND4L, MT-ND5, MT-ND6, NDUFA1, NDUFA10, NDUFA11, NDUFA12, NDUFA13, NDUFA2, NDUFA3, NDUFA4, NDUFA5, NDUFA6, NDUFA7, NDUFA8, NDUFA9, NDUFAB1, NDUFB1, NDUFB10, NDUFB11, NDUFB2, NDUFB3, NDUFB4, NDUFB5, NDUFB6, NDUFB7, NDUFB8, NDUFB9, NDUFC1, NDUFC2, NDUFS1, NDUFS2, NDUFS3, NDUFS4, NDUFS5, NDUFS6, NDUFS7, NDUFS8, NDUFV1, NDUFV2, NDUFV3, SDHA, SDHB, SDHC, SDHD, UQCR10, UQCR11, UQCRB, UQCRC1, UQCRC2, UQCRFS1, UQCRH, UQCRQ
OXPHOS assembly factors OXPHOS > OXPHOS assembly factors ACAD9, AIFM1, ATPAF1, ATPAF2, ATPSCKMT, BCS1L, CEP89, CMC1, CMC2, COA1, COA3, COA4, COA5, COA6, COA7, COA8, COX10, COX11, COX14, COX15, COX16, COX17, COX18, COX19, COX20, COX7A2L, DMAC1, DMAC2, ECSIT, FMC1, FOXRED1, HIGD1A, HIGD2A, LYRM2, LYRM7, NDUFAF1, NDUFAF2, NDUFAF3, NDUFAF4, NDUFAF5, NDUFAF6, NDUFAF7, NDUFAF8, NUBPL, PET100, PET117, PNKD, RAB5IF, SCO1, SCO2, SDHAF1, SDHAF2, SDHAF3, SDHAF4, SMIM20, SURF1, TACO1, TIMM21, TIMMDC1, TMEM126A, TMEM126B, TMEM177, TMEM186, TMEM70, TTC19, UQCC1, UQCC2, UQCC3
Complex I OXPHOS > Complex I ACAD9, AIFM1, COA1, DMAC1, DMAC2, ECSIT, FOXRED1, LYRM2, MT-ND1, MT-ND2, MT-ND3, MT-ND4, MT-ND4L, MT-ND5, MT-ND6, NDUFA1, NDUFA10, NDUFA11, NDUFA12, NDUFA13, NDUFA2, NDUFA3, NDUFA5, NDUFA6, NDUFA7, NDUFA8, NDUFA9, NDUFAB1, NDUFAF1, NDUFAF2, NDUFAF3, NDUFAF4, NDUFAF5, NDUFAF6, NDUFAF7, NDUFAF8, NDUFB1, NDUFB10, NDUFB11, NDUFB2, NDUFB3, NDUFB4, NDUFB5, NDUFB6, NDUFB7, NDUFB8, NDUFB9, NDUFC1, NDUFC2, NDUFS1, NDUFS2, NDUFS3, NDUFS4, NDUFS5, NDUFS6, NDUFS7, NDUFS8, NDUFV1, NDUFV2, NDUFV3, NUBPL, TIMMDC1, TMEM126A, TMEM126B, TMEM186, TMEM70
CI subunits OXPHOS > Complex I > CI subunits MT-ND1, MT-ND2, MT-ND3, MT-ND4, MT-ND4L, MT-ND5, MT-ND6, NDUFA1, NDUFA10, NDUFA11, NDUFA12, NDUFA13, NDUFA2, NDUFA3, NDUFA5, NDUFA6, NDUFA7, NDUFA8, NDUFA9, NDUFAB1, NDUFB1, NDUFB10, NDUFB11, NDUFB2, NDUFB3, NDUFB4, NDUFB5, NDUFB6, NDUFB7, NDUFB8, NDUFB9, NDUFC1, NDUFC2, NDUFS1, NDUFS2, NDUFS3, NDUFS4, NDUFS5, NDUFS6, NDUFS7, NDUFS8, NDUFV1, NDUFV2, NDUFV3
CI assembly factors OXPHOS > Complex I > CI assembly factors ACAD9, AIFM1, COA1, DMAC1, DMAC2, ECSIT, FOXRED1, LYRM2, NDUFAF1, NDUFAF2, NDUFAF3, NDUFAF4, NDUFAF5, NDUFAF6, NDUFAF7, NDUFAF8, NUBPL, TIMMDC1, TMEM126A, TMEM126B, TMEM186, TMEM70
Complex II OXPHOS > Complex II SDHA, SDHAF1, SDHAF2, SDHAF3, SDHAF4, SDHB, SDHC, SDHD
CII subunits OXPHOS > Complex II > CII subunits SDHA, SDHB, SDHC, SDHD
CII assembly factors OXPHOS > Complex II > CII assembly factors SDHAF1, SDHAF2, SDHAF3, SDHAF4
Complex III OXPHOS > Complex III BCS1L, CYC1, LYRM7, MT-CYB, TTC19, UQCC1, UQCC2, UQCC3, UQCR10, UQCR11, UQCRB, UQCRC1, UQCRC2, UQCRFS1, UQCRH, UQCRQ
CIII subunits OXPHOS > Complex III > CIII subunits CYC1, MT-CYB, UQCR10, UQCR11, UQCRB, UQCRC1, UQCRC2, UQCRFS1, UQCRH, UQCRQ
CIII assembly factors OXPHOS > Complex III > CIII assembly factors BCS1L, LYRM7, TTC19, UQCC1, UQCC2, UQCC3
Complex IV OXPHOS > Complex IV CEP89, CMC1, CMC2, COA1, COA3, COA4, COA5, COA6, COA7, COA8, COX10, COX11, COX14, COX15, COX16, COX17, COX18, COX19, COX20, COX4I1, COX4I2, COX5A, COX5B, COX6A1, COX6A2, COX6B1, COX6B2, COX6C, COX7A1, COX7A2, COX7A2L, COX7B, COX7B2, COX7C, COX8A, COX8C, HIGD1A, MT-CO1, MT-CO2, MT-CO3, NDUFA4, PET100, PET117, PNKD, SCO1, SCO2, SMIM20, SURF1, TACO1, TIMM21, TMEM177
CIV subunits OXPHOS > Complex IV > CIV subunits COX4I1, COX4I2, COX5A, COX5B, COX6A1, COX6A2, COX6B1, COX6B2, COX6C, COX7A1, COX7A2, COX7A2L, COX7B, COX7B2, COX7C, COX8A, COX8C, MT-CO1, MT-CO2, MT-CO3, NDUFA4
CIV assembly factors OXPHOS > Complex IV > CIV assembly factors CEP89, CMC1, CMC2, COA1, COA3, COA4, COA5, COA6, COA7, COA8, COX10, COX11, COX14, COX15, COX16, COX17, COX18, COX19, COX20, HIGD1A, PET100, PET117, PNKD, SCO1, SCO2, SMIM20, SURF1, TACO1, TIMM21, TMEM177
Complex V OXPHOS > Complex V ATP5F1A, ATP5F1B, ATP5F1C, ATP5F1D, ATP5F1E, ATP5IF1, ATP5MC1, ATP5MC2, ATP5MC3, ATP5MD, ATP5ME, ATP5MF, ATP5MG, ATP5MPL, ATP5PB, ATP5PD, ATP5PF, ATP5PO, ATPAF1, ATPAF2, ATPSCKMT, DMAC2L, FMC1, MT-ATP6, MT-ATP8, TMEM70
CV subunits OXPHOS > Complex V > CV subunits ATP5F1A, ATP5F1B, ATP5F1C, ATP5F1D, ATP5F1E, ATP5IF1, ATP5MC1, ATP5MC2, ATP5MC3, ATP5MD, ATP5ME, ATP5MF, ATP5MG, ATP5MPL, ATP5PB, ATP5PD, ATP5PF, ATP5PO, DMAC2L, MT-ATP6, MT-ATP8
CV assembly factors OXPHOS > Complex V > CV assembly factors ATPAF1, ATPAF2, ATPSCKMT, FMC1, TMEM70
Respirasome assembly OXPHOS > Respirasome assembly COX7A2L, HIGD1A, HIGD2A, RAB5IF
Cytochrome C OXPHOS > Cytochrome C CYCS, HCCS
Metabolism Metabolism AADAT, AASS, ABAT, ABCB10, ABCB6, ABCB7, ABHD10, ABHD11, ACAA1, ACAA2, ACACA, ACACB, ACAD10, ACAD11, ACAD8, ACADL, ACADM, ACADS, ACADSB, ACADVL, ACAT1, ACLY, ACO2, ACOD1, ACOT11, ACOT13, ACOT2, ACOT7, ACOT9, ACP6, ACSF2, ACSF3, ACSL1, ACSL6, ACSM1, ACSM2A, ACSM2B, ACSM3, ACSM4, ACSM5, ACSS1, ACSS3, ADHFE1, AGK, AGMAT, AGPAT4, AGPAT5, AGXT, AGXT2, AIFM2, AIFM3, AK2, AK3, AK4, AKR1B10, AKR7A2, ALAS1, ALAS2, ALDH18A1, ALDH1B1, ALDH1L1, ALDH1L2, ALDH2, ALDH3A2, ALDH4A1, ALDH5A1, ALDH6A1, ALDH7A1, ALDH9A1, AMACR, AMT, ANTKMT, ARG2, AUH, BCAT2, BCKDHA, BCKDHB, BCKDK, BCO2, BDH1, BOLA1, BOLA3, BPHL, CA5A, CA5B, CAT, CBR3, CBR4, CHCHD7, CHDH, CHPT1, CISD1, CISD3, CKMT1A, CKMT1B, CKMT2, CLYBL, CMPK2, COASY, COMT, COQ10A, COQ10B, COQ2, COQ3, COQ4, COQ5, COQ6, COQ7, COQ8A, COQ8B, COQ9, COX10, COX11, COX15, COX17, COX19, CPOX, CPS1, CPT1A, CPT1B, CPT1C, CPT2, CRAT, CRLS1, CROT, CS, CYB5B, CYB5R3, CYC1, CYCS, CYP11A1, CYP11B1, CYP11B2, CYP24A1, CYP27A1, CYP27B1, D2HGDH, DBI, DBT, DCXR, DECR1, DGLUCY, DGUOK, DHODH, DHRS1, DHRS2, DHRS4, DHTKD1, DLAT, DLD, DLST, DMGDH, DNA2, DTYMK, DUT, ECH1, ECHDC1, ECHS1, ECI1, ECI2, EHHADH, EPHX2, ETFA, ETFB, ETFBKMT, ETFDH, ETFRF1, ETHE1, FABP1, FAHD1, FAM210B, FASN, FDPS, FDX1, FDX2, FDXR, FECH, FH, FHIT, FLAD1, FPGS, FTH1, FTMT, FXN, GATM, GCAT, GCDH, GCSH, GLDC, GLRX2, GLRX5, GLS, GLS2, GLUD1, GLUD2, GLYAT, GLYCTK, GOT2, GPAM, GPAT2, GPD2, GPT2, GPX1, GPX4, GRHPR, GRPEL1, GRPEL2, GSR, GSTK1, GSTZ1, GTPBP3, GUK1, HADH, HADHA, HADHB, HAGH, HAO2, HCCS, HIBADH, HIBCH, HINT1, HINT2, HMGCL, HMGCS2, HOGA1, HSCB, HSD17B10, HSD17B4, HSD17B8, HSPA9, HTD2, IBA57, IDH2, IDH3A, IDH3B, IDH3G, IDI1, ISCA1, ISCA2, ISCU, IVD, KMO, KYAT3, L2HGDH, LACTB, LDHAL6B, LDHB, LDHD, LIAS, LIPT1, LIPT2, LYPLA1, LYPLAL1, LYRM4, MAOA, MAOB, MCAT, MCCC1, MCCC2, MCEE, MDH2, ME2, ME3, MECR, MGST1, MGST3, MLYCD, MMAA, MMAB, MMADHC, MMUT, MOCS1, MPC1, MPC1L, MPC2, MPST, MRPS36, MSRA, MSRB2, MSRB3, MT-CO1, MT-CYB, MTARC1, MTARC2, MTFMT, MTHFD1L, MTHFD2, MTHFD2L, MTHFS, MTO1, NADK2, NAGS, NAT8L, NAXD, NAXE, NDUFAB1, NDUFS1, NDUFS2, NDUFS7, NDUFS8, NDUFV1, NDUFV2, NEU4, NFS1, NFU1, NIT1, NIT2, NME3, NME4, NME6, NMNAT3, NNT, NOCT, NT5DC2, NT5M, NTHL1, NUBPL, NUDT13, NUDT19, NUDT2, NUDT5, NUDT8, NUDT9, OAT, OGDH, OGDHL, OSBPL1A, OTC, OXCT1, OXCT2, OXR1, OXSM, PAICS, PANK2, PC, PCBD2, PCCA, PCCB, PCK2, PDHA1, PDHA2, PDHB, PDHX, PDK1, PDK2, PDK3, PDK4, PDP1, PDP2, PDPR, PDSS1, PDSS2, PGS1, PHYH, PISD, PLD6, PLPBP, PLSCR3, PNPLA8, PNPO, PPA2, PPM1K, PPOX, PRDX2, PRDX3, PRDX4, PRDX5, PRDX6, PRELID1, PRELID3A, PRELID3B, PRODH, PRODH2, PRXL2A, PTGES2, PTPMT1, PYCR1, PYCR2, QDPR, RDH13, RDH14, RFK, RPIA, RSAD1, SARDH, SCO1, SCO2, SCP2, SDHA, SDHB, SDHC, SDHD, SDSL, SELENOO, SERAC1, SFXN1, SFXN3, SFXN5, SHMT2, SIRT3, SIRT4, SIRT5, SLC25A1, SLC25A10, SLC25A11, SLC25A12, SLC25A13, SLC25A14, SLC25A15, SLC25A19, SLC25A20, SLC25A21, SLC25A23, SLC25A24, SLC25A25, SLC25A28, SLC25A29, SLC25A3, SLC25A30, SLC25A31, SLC25A32, SLC25A33, SLC25A36, SLC25A37, SLC25A38, SLC25A4, SLC25A41, SLC25A42, SLC25A44, SLC25A5, SLC25A51, SLC25A52, SLC25A6, SOD1, SOD2, SPHK2, SPR, SPTLC2, SQOR, STAR, STARD7, SUCLA2, SUCLG1, SUCLG2, SUGCT, SUOX, TAMM41, TAZ, THEM4, THEM5, TK2, TMLHE, TRIAP1, TSPO, TST, TSTD1, TXN2, TXNRD1, TXNRD2, UQCRFS1, ZADH2
Carbohydrate metabolism Metabolism > Carbohydrate metabolism ABHD11, ACAA2, ACAT1, ACLY, ACO2, ACOD1, AUH, BDH1, CLYBL, CS, D2HGDH, DCXR, DLAT, DLD, DLST, FAHD1, FH, GLYCTK, GOT2, GPD2, HADHB, HMGCL, HMGCS2, IDH2, IDH3A, IDH3B, IDH3G, L2HGDH, LDHAL6B, LDHD, MCEE, MDH2, ME2, ME3, MMUT, MPC1, MPC1L, MPC2, MRPS36, NEU4, OGDH, OGDHL, OXCT1, OXCT2, PC, PCCA, PCCB, PCK2, PDHA1, PDHA2, PDHB, PDHX, PDK1, PDK2, PDK3, PDK4, PDP1, PDP2, PDPR, RPIA, SDHA, SDHB, SDHC, SDHD, SFXN5, SLC25A1, SLC25A10, SLC25A11, SLC25A12, SLC25A13, SUCLA2, SUCLG1, SUCLG2
Gluconeogenesis Metabolism > Carbohydrate metabolism > Gluconeogenesis MDH2, MPC1, MPC1L, MPC2, PC, PCK2, SLC25A1, SLC25A10, SLC25A11
Malate-aspartate shuttle Metabolism > Carbohydrate metabolism > Malate-aspartate shuttle GOT2, MDH2, SLC25A10, SLC25A11, SLC25A12, SLC25A13
Glycerol phosphate shuttle Metabolism > Carbohydrate metabolism > Glycerol phosphate shuttle GPD2
Pyruvate metabolism Metabolism > Carbohydrate metabolism > Pyruvate metabolism DLAT, DLD, MPC1, MPC1L, MPC2, PDHA1, PDHA2, PDHB, PDHX, PDK1, PDK2, PDK3, PDK4, PDP1, PDP2, PDPR
TCA cycle Metabolism > Carbohydrate metabolism > TCA cycle ACO2, CS, DLD, DLST, FH, IDH2, IDH3A, IDH3B, IDH3G, MDH2, MRPS36, OGDH, OGDHL, SDHA, SDHB, SDHC, SDHD, SUCLA2, SUCLG1, SUCLG2
TCA-associated Metabolism > Carbohydrate metabolism > TCA-associated ABHD11, ACLY, D2HGDH, FAHD1, L2HGDH, ME2, ME3, MPC1, MPC1L, MPC2, PC, PCK2, SFXN5, SLC25A1, SLC25A11
Itaconate metabolism Metabolism > Carbohydrate metabolism > Itaconate metabolism ACOD1, AUH, CLYBL, SUCLA2, SUCLG1, SUCLG2
Ketone metabolism Metabolism > Carbohydrate metabolism > Ketone metabolism ACAA2, ACAT1, BDH1, HADHB, HMGCL, HMGCS2, OXCT1, OXCT2
Propanoate metabolism Metabolism > Carbohydrate metabolism > Propanoate metabolism MCEE, MMUT, PCCA, PCCB
Lipid metabolism Metabolism > Lipid metabolism ACAA1, ACAA2, ACACA, ACACB, ACAD10, ACAD11, ACADL, ACADM, ACADS, ACADSB, ACADVL, ACAT1, ACOT11, ACOT13, ACOT2, ACOT7, ACOT9, ACP6, ACSF2, ACSF3, ACSL1, ACSL6, ACSM1, ACSM2A, ACSM2B, ACSM3, ACSM4, ACSM5, ACSS1, ACSS3, AGK, AGPAT4, AGPAT5, AMACR, CBR4, CHPT1, CPT1A, CPT1B, CPT1C, CPT2, CRAT, CRLS1, CROT, CYB5R3, CYP11A1, CYP11B1, CYP11B2, CYP27A1, DBI, DECR1, DHRS1, ECH1, ECHDC1, ECHS1, ECI1, ECI2, EHHADH, EPHX2, ETFA, ETFB, ETFDH, FABP1, FASN, FDPS, FDX1, FDXR, GCSH, GPAM, GPAT2, HADH, HADHA, HADHB, HAO2, HINT2, HSD17B10, HSD17B4, HSD17B8, HTD2, IDI1, LACTB, LIAS, LIPT1, LIPT2, LYPLA1, LYPLAL1, MCAT, MCEE, MECR, MGST3, MLYCD, MMUT, NDUFAB1, OSBPL1A, OXSM, PCCA, PCCB, PGS1, PHYH, PISD, PLD6, PLSCR3, PNPLA8, PRDX6, PRELID1, PRELID3A, PRELID3B, PTGES2, PTPMT1, SCP2, SERAC1, SLC25A1, SLC25A20, SPHK2, SPTLC2, STAR, STARD7, TAMM41, TAZ, THEM4, THEM5, TRIAP1, TSPO, ZADH2
Fatty acid oxidation Metabolism > Lipid metabolism > Fatty acid oxidation ACAA2, ACACB, ACAD10, ACAD11, ACADL, ACADM, ACADS, ACADSB, ACADVL, ACAT1, ACOT11, ACSF2, ACSL1, ACSL6, ACSM1, ACSM2A, ACSM2B, ACSM3, ACSM4, ACSM5, ACSS1, AMACR, CPT1A, CPT1B, CPT1C, CPT2, CRAT, CROT, DECR1, ECHS1, ECI1, ECI2, ETFA, ETFB, ETFDH, HADH, HADHA, HADHB, HSD17B10, MCEE, MMUT, PCCA, PCCB, SLC25A20
Type II fatty acid synthesis Metabolism > Lipid metabolism > Type II fatty acid synthesis ACACA, ACSF3, CBR4, HSD17B8, HTD2, MCAT, MECR, NDUFAB1, OXSM
Lipoate insertion Metabolism > Lipid metabolism > Lipoate insertion GCSH, LIAS, LIPT1, LIPT2
Cardiolipin synthesis Metabolism > Lipid metabolism > Cardiolipin synthesis AGPAT4, AGPAT5, CRLS1, GPAM, GPAT2, HADHA, PGS1, PLD6, PLSCR3, PNPLA8, PRELID1, PRELID3A, PTPMT1, TAMM41, TAZ, TRIAP1
Cholesterol, bile acid, steroid synthesis Metabolism > Lipid metabolism > Cholesterol, bile acid, steroid synthesis CYP11A1, CYP11B1, CYP11B2, CYP27A1, DHRS1, FDX1, FDXR, HINT2, HSD17B10, HSD17B8, OSBPL1A, SCP2, STAR, TSPO
Cholesterol-associated Metabolism > Lipid metabolism > Cholesterol-associated FDPS, IDI1
Phospholipid metabolism Metabolism > Lipid metabolism > Phospholipid metabolism ACP6, AGK, AGPAT4, AGPAT5, CHPT1, GPAM, GPAT2, LACTB, OSBPL1A, PGS1, PISD, PNPLA8, PRDX6, PRELID1, PRELID3A, PRELID3B, PTPMT1, SERAC1, SPHK2, SPTLC2, STARD7, TAMM41, TRIAP1
Eicosanoid metabolism Metabolism > Lipid metabolism > Eicosanoid metabolism MGST3, PTGES2, ZADH2
Amino acid metabolism Metabolism > Amino acid metabolism AADAT, AASS, ABAT, ACAA2, ACAD8, ACADSB, ACAT1, ADHFE1, AGMAT, AGXT, AGXT2, AKR7A2, ALDH18A1, ALDH4A1, ALDH5A1, ALDH6A1, ALDH7A1, ALDH9A1, AMT, ARG2, AUH, BCAT2, BCKDHA, BCKDHB, BCKDK, CA5A, CA5B, CHDH, COMT, CPS1, DBT, DGLUCY, DHTKD1, DLD, DLST, DMGDH, ECHS1, ETFA, ETFB, ETFDH, GCAT, GCDH, GCSH, GLDC, GLS, GLS2, GLUD1, GLUD2, GLYAT, GOT2, GPT2, GRHPR, HADH, HADHA, HADHB, HIBADH, HIBCH, HMGCL, HOGA1, HSD17B10, IVD, KMO, KYAT3, LDHB, MAOA, MAOB, MCCC1, MCCC2, NAGS, NAT8L, OAT, OTC, PPM1K, PRODH, PRODH2, PYCR1, PYCR2, SARDH, SDSL, SFXN1, SFXN3, SHMT2, SLC25A12, SLC25A13, SLC25A15, SLC25A21, SLC25A29, SLC25A38, SLC25A44, SUGCT
Branched-chain amino acid metabolism Metabolism > Amino acid metabolism > Branched-chain amino acid metabolism ACAD8, ACADSB, ACAT1, ALDH6A1, AUH, BCAT2, BCKDHA, BCKDHB, BCKDK, DBT, DLD, ECHS1, ETFA, ETFB, ETFDH, HADHA, HIBADH, HIBCH, HMGCL, HSD17B10, IVD, MCCC1, MCCC2, PPM1K, SLC25A44
Branched-chain amino acid dehydrogenase complex Metabolism > Amino acid metabolism > Branched-chain amino acid dehydrogenase complex BCKDHA, BCKDHB, BCKDK, DBT, DLD, PPM1K
Lysine metabolism Metabolism > Amino acid metabolism > Lysine metabolism AADAT, AASS, ACAA2, ACAT1, ALDH7A1, DHTKD1, DLD, DLST, ECHS1, ETFA, ETFB, ETFDH, GCDH, HADH, HADHB, SLC25A29
Serine metabolism Metabolism > Amino acid metabolism > Serine metabolism AGXT, SDSL, SFXN1, SFXN3, SHMT2
Glycine metabolism Metabolism > Amino acid metabolism > Glycine metabolism ALDH7A1, AMT, CHDH, DLD, DMGDH, ETFA, ETFB, ETFDH, GCAT, GCSH, GLDC, GLYAT, SARDH, SHMT2, SLC25A38
Glycine cleavage system Metabolism > Amino acid metabolism > Glycine cleavage system AMT, DLD, GCSH, GLDC
Glutamate metabolism Metabolism > Amino acid metabolism > Glutamate metabolism GLS, GLS2, GLUD1, GLUD2, GOT2, GPT2, NAT8L, SLC25A12
Proline metabolism Metabolism > Amino acid metabolism > Proline metabolism ALDH18A1, ALDH4A1, OAT, PRODH, PYCR1, PYCR2
Glyoxylate metabolism Metabolism > Amino acid metabolism > Glyoxylate metabolism AGXT, AGXT2, ALDH4A1, GOT2, GRHPR, HOGA1, LDHB, PRODH2
GABA metabolism Metabolism > Amino acid metabolism > GABA metabolism ABAT, ADHFE1, AKR7A2, ALDH5A1, ALDH9A1, GLUD1, GLUD2
Catechol metabolism Metabolism > Amino acid metabolism > Catechol metabolism COMT, MAOA, MAOB
Kynurenine metabolism Metabolism > Amino acid metabolism > Kynurenine metabolism GOT2, KMO, KYAT3
Urea cycle Metabolism > Amino acid metabolism > Urea cycle CA5A, CA5B, CPS1, NAGS, OTC, SLC25A13, SLC25A15
Nucleotide metabolism Metabolism > Nucleotide metabolism AK2, AK3, AK4, ANTKMT, CKMT1A, CKMT1B, CKMT2, CMPK2, DGUOK, DHODH, DTYMK, DUT, FHIT, GATM, GUK1, HINT1, NME3, NME4, NME6, NT5DC2, NT5M, NUDT2, NUDT5, NUDT9, PAICS, PPA2, SLC25A23, SLC25A24, SLC25A25, SLC25A31, SLC25A33, SLC25A36, SLC25A4, SLC25A41, SLC25A5, SLC25A6, SUCLA2, SUCLG1, SUCLG2, TK2
Nucleotide import Metabolism > Nucleotide metabolism > Nucleotide import SLC25A23, SLC25A24, SLC25A25, SLC25A31, SLC25A33, SLC25A36, SLC25A4, SLC25A41, SLC25A5, SLC25A6
Nucleotide synthesis and processing Metabolism > Nucleotide metabolism > Nucleotide synthesis and processing AK2, AK3, AK4, CMPK2, DGUOK, DHODH, DTYMK, DUT, FHIT, GUK1, HINT1, NME3, NME4, NME6, NT5DC2, NT5M, NUDT2, NUDT5, NUDT9, PAICS, SUCLA2, SUCLG1, SUCLG2, TK2
Creatine metabolism Metabolism > Nucleotide metabolism > Creatine metabolism CKMT1A, CKMT1B, CKMT2, GATM
Metals and cofactors Metabolism > Metals and cofactors ABAT, ABCB10, ABCB6, ABCB7, ACO2, AIFM3, ALAS1, ALAS2, BOLA1, BOLA3, CHCHD7, CISD1, CISD3, COASY, COQ10A, COQ10B, COQ2, COQ3, COQ4, COQ5, COQ6, COQ7, COQ8A, COQ8B, COQ9, COX10, COX11, COX15, COX17, COX19, CPOX, CPT1A, CPT1B, CPT1C, CPT2, CYB5B, CYC1, CYCS, CYP11A1, CYP11B1, CYP11B2, CYP24A1, CYP27A1, CYP27B1, DNA2, ETFDH, FABP1, FAM210B, FDX1, FDX2, FDXR, FECH, FTH1, FTMT, FXN, GLRX2, GLRX5, GRPEL1, GRPEL2, HCCS, HSCB, HSPA9, IBA57, ISCA1, ISCA2, ISCU, KMO, KYAT3, LIAS, LYRM4, MOCS1, MT-CO1, MT-CYB, MTARC1, MTARC2, NADK2, NAXD, NAXE, NDUFAB1, NDUFS1, NDUFS2, NDUFS7, NDUFS8, NDUFV1, NDUFV2, NFS1, NFU1, NMNAT3, NNT, NOCT, NTHL1, NUBPL, NUDT13, NUDT19, NUDT8, PANK2, PCBD2, PDSS1, PDSS2, PPOX, QDPR, RSAD1, SCO1, SCO2, SDHB, SDHC, SDHD, SIRT3, SIRT4, SIRT5, SLC25A20, SLC25A28, SLC25A3, SLC25A37, SLC25A38, SLC25A42, SLC25A51, SLC25A52, SPR, SUOX, TMLHE, UQCRFS1
Carnitine synthesis and transport Metabolism > Metals and cofactors > Carnitine synthesis and transport CPT1A, CPT1B, CPT1C, CPT2, SLC25A20, TMLHE
Carnitine shuttle Metabolism > Metals and cofactors > Carnitine shuttle CPT1A, CPT1B, CPT1C, CPT2, SLC25A20
Coenzyme A metabolism Metabolism > Metals and cofactors > Coenzyme A metabolism COASY, NUDT19, NUDT8, PANK2, SLC25A42
Coenzyme Q metabolism Metabolism > Metals and cofactors > Coenzyme Q metabolism COQ10A, COQ10B, COQ2, COQ3, COQ4, COQ5, COQ6, COQ7, COQ8A, COQ8B, COQ9, PDSS1, PDSS2
Copper metabolism Metabolism > Metals and cofactors > Copper metabolism CHCHD7, COX11, COX17, COX19, SCO1, SCO2, SLC25A3
Iron homeostasis Metabolism > Metals and cofactors > Iron homeostasis FAM210B, FTH1, FTMT, SLC25A28, SLC25A37
Heme synthesis and processing Metabolism > Metals and cofactors > Heme synthesis and processing ABCB10, ABCB6, ABCB7, ALAS1, ALAS2, COX10, COX15, CPOX, FABP1, FDX1, FDX2, FDXR, FECH, HCCS, PPOX, SLC25A38
Fe-S cluster biosynthesis Metabolism > Metals and cofactors > Fe-S cluster biosynthesis ABCB7, BOLA1, BOLA3, CISD1, CISD3, FDX1, FDX2, FDXR, FXN, GLRX5, GRPEL1, GRPEL2, HSCB, HSPA9, IBA57, ISCA1, ISCA2, ISCU, LYRM4, NDUFAB1, NFS1, NFU1, NUBPL
Molybdenum cofactor synthesis and proteins Metabolism > Metals and cofactors > Molybdenum cofactor synthesis and proteins ABCB7, MOCS1, MTARC1, MTARC2, SUOX
NAD biosynthesis and metabolism Metabolism > Metals and cofactors > NAD biosynthesis and metabolism KMO, KYAT3, NADK2, NAXD, NAXE, NMNAT3, NNT, NOCT, NUDT13, SIRT3, SIRT4, SIRT5, SLC25A51, SLC25A52
Tetrahydobiopterin synthesis Metabolism > Metals and cofactors > Tetrahydobiopterin synthesis PCBD2, QDPR, SPR
Fe-S-containing proteins Metabolism > Metals and cofactors > Fe-S-containing proteins ABAT, ACO2, AIFM3, BOLA1, BOLA3, CISD1, CISD3, DNA2, ETFDH, FDX1, FDX2, FECH, GLRX2, GLRX5, ISCA1, ISCA2, ISCU, LIAS, NDUFS1, NDUFS2, NDUFS7, NDUFS8, NDUFV1, NDUFV2, NFS1, NFU1, NTHL1, NUBPL, RSAD1, SDHB, UQCRFS1
Heme-containing proteins Metabolism > Metals and cofactors > Heme-containing proteins CYB5B, CYC1, CYCS, CYP11A1, CYP11B1, CYP11B2, CYP24A1, CYP27A1, CYP27B1, MT-CO1, MT-CYB, SDHC, SDHD
Vitamin metabolism Metabolism > Vitamin metabolism ACACA, ACACB, AKR1B10, ALDH1L1, ALDH1L2, ALDH7A1, AMT, BCO2, CHDH, CYP24A1, CYP27B1, DHRS4, DMGDH, ETFA, ETFB, ETFDH, FDX1, FDXR, FLAD1, FPGS, GLRX2, GTPBP3, MCCC1, MMAA, MMAB, MMADHC, MMUT, MTFMT, MTHFD1L, MTHFD2, MTHFD2L, MTHFS, MTO1, PC, PCCA, PLPBP, PNPO, QDPR, RDH13, RDH14, RFK, SARDH, SFXN1, SFXN3, SHMT2, SLC25A19, SLC25A32, SLC25A38, TXN2
Choline and betaine metabolism Metabolism > Vitamin metabolism > Choline and betaine metabolism ALDH7A1, CHDH, DMGDH, ETFA, ETFB, ETFDH, SARDH
Biotin utilizing proteins Metabolism > Vitamin metabolism > Biotin utilizing proteins ACACA, ACACB, MCCC1, PC, PCCA
Vitamin A metabolism Metabolism > Vitamin metabolism > Vitamin A metabolism AKR1B10, BCO2, DHRS4, RDH13, RDH14
Vitamin B1 metabolism Metabolism > Vitamin metabolism > Vitamin B1 metabolism SLC25A19
Vitamin B2 metabolism Metabolism > Vitamin metabolism > Vitamin B2 metabolism FLAD1, RFK, SLC25A32
Vitamin B6 metabolism Metabolism > Vitamin metabolism > Vitamin B6 metabolism PLPBP, PNPO
Vitamin B12 metabolism Metabolism > Vitamin metabolism > Vitamin B12 metabolism MMAA, MMAB, MMADHC, MMUT
Vitamin C metabolism Metabolism > Vitamin metabolism > Vitamin C metabolism GLRX2, TXN2
Vitamin D metabolism Metabolism > Vitamin metabolism > Vitamin D metabolism CYP24A1, CYP27B1, FDX1, FDXR
Folate and 1-C metabolism Metabolism > Vitamin metabolism > Folate and 1-C metabolism ALDH1L1, ALDH1L2, ALDH7A1, AMT, CHDH, DMGDH, FPGS, GTPBP3, MTFMT, MTHFD1L, MTHFD2, MTHFD2L, MTHFS, MTO1, QDPR, SARDH, SFXN1, SFXN3, SHMT2, SLC25A32, SLC25A38
Detoxification Metabolism > Detoxification ABHD10, ACSM2A, ACSM5, AIFM2, AKR1B10, AKR7A2, ALDH1B1, ALDH2, ALDH3A2, ALDH7A1, ALDH9A1, BPHL, CAT, CBR3, CYB5B, CYB5R3, DHRS2, EPHX2, GLRX2, GLYAT, GPX1, GPX4, GSR, GSTK1, GSTZ1, HAGH, MAOA, MAOB, MGST1, MPST, MSRA, MSRB2, MSRB3, MTARC1, MTARC2, NIT1, NIT2, OXR1, PRDX2, PRDX3, PRDX4, PRDX5, PRDX6, PRXL2A, SELENOO, SOD1, SOD2, TST, TXN2, TXNRD1, TXNRD2
Xenobiotic metabolism Metabolism > Detoxification > Xenobiotic metabolism ABHD10, ACSM2A, ACSM5, AKR1B10, AKR7A2, ALDH1B1, ALDH2, BPHL, CBR3, CYB5B, CYB5R3, DHRS2, EPHX2, GLYAT, GSTK1, GSTZ1, MAOA, MAOB, MGST1, MPST, MTARC1, MTARC2, TST
ROS and glutathione metabolism Metabolism > Detoxification > ROS and glutathione metabolism AIFM2, ALDH3A2, ALDH7A1, ALDH9A1, CAT, GLRX2, GPX1, GPX4, GSR, HAGH, MGST1, MSRA, MSRB2, MSRB3, NIT1, OXR1, PRDX2, PRDX3, PRDX4, PRDX5, PRDX6, PRXL2A, SOD1, SOD2, TXN2, TXNRD1, TXNRD2
Amidoxime reducing complex Metabolism > Detoxification > Amidoxime reducing complex CYB5B, CYB5R3, MTARC1, MTARC2
Selenoproteins Metabolism > Detoxification > Selenoproteins GPX1, GPX4, SELENOO, TXNRD1, TXNRD2
Electron carriers Metabolism > Electron carriers CHDH, CYB5B, CYC1, CYCS, DHODH, ETFA, ETFB, ETFBKMT, ETFDH, ETFRF1, GPD2, MT-CYB, PRODH, PRODH2, SQOR
Cytochromes Metabolism > Electron carriers > Cytochromes CYB5B, CYC1, CYCS, MT-CYB
Q-linked reactions, other Metabolism > Electron carriers > Q-linked reactions, other CHDH, DHODH, ETFA, ETFB, ETFBKMT, ETFDH, ETFRF1, GPD2, PRODH, PRODH2, SQOR
Sulfur metabolism Metabolism > Sulfur metabolism ETHE1, GOT2, MPST, MSRA, MSRB2, SLC25A14, SLC25A30, SQOR, SUOX, TST, TSTD1
Small molecule transport Small molecule transport ABCA9, ABCB10, ABCB6, ABCB7, ABCB8, ABCD1, ABCD2, ABCD3, CCDC51, LETMD1, MCU, MCUB, MICU1, MICU2, MICU3, MPC1, MPC1L, MPC2, MPV17, MPV17L, MRS2, MTCH1, MTCH2, ROMO1, SFXN1, SFXN2, SFXN3, SFXN4, SFXN5, SLC25A1, SLC25A10, SLC25A11, SLC25A12, SLC25A13, SLC25A14, SLC25A15, SLC25A16, SLC25A18, SLC25A19, SLC25A20, SLC25A21, SLC25A22, SLC25A23, SLC25A24, SLC25A25, SLC25A26, SLC25A27, SLC25A28, SLC25A29, SLC25A3, SLC25A30, SLC25A31, SLC25A32, SLC25A33, SLC25A34, SLC25A35, SLC25A36, SLC25A37, SLC25A38, SLC25A39, SLC25A4, SLC25A40, SLC25A41, SLC25A42, SLC25A43, SLC25A44, SLC25A45, SLC25A46, SLC25A47, SLC25A48, SLC25A5, SLC25A51, SLC25A52, SLC25A53, SLC25A6, SLC30A9, SLC8B1, SMDT1, STARD7, UCP1, UCP2, UCP3, VDAC1, VDAC2, VDAC3
SLC25A family Small molecule transport > SLC25A family MTCH1, MTCH2, SLC25A1, SLC25A10, SLC25A11, SLC25A12, SLC25A13, SLC25A14, SLC25A15, SLC25A16, SLC25A18, SLC25A19, SLC25A20, SLC25A21, SLC25A22, SLC25A23, SLC25A24, SLC25A25, SLC25A26, SLC25A27, SLC25A28, SLC25A29, SLC25A3, SLC25A30, SLC25A31, SLC25A32, SLC25A33, SLC25A34, SLC25A35, SLC25A36, SLC25A37, SLC25A38, SLC25A39, SLC25A4, SLC25A40, SLC25A41, SLC25A42, SLC25A43, SLC25A44, SLC25A45, SLC25A46, SLC25A47, SLC25A48, SLC25A5, SLC25A51, SLC25A52, SLC25A53, SLC25A6, UCP1, UCP2, UCP3
ABC transporters Small molecule transport > ABC transporters ABCA9, ABCB10, ABCB6, ABCB7, ABCB8, ABCD1, ABCD2, ABCD3
Sideroflexins Small molecule transport > Sideroflexins SFXN1, SFXN2, SFXN3, SFXN4, SFXN5
Calcium uniporter Small molecule transport > Calcium uniporter MCU, MCUB, MICU1, MICU2, MICU3, SMDT1
Signaling Signaling ACOD1, ADCY10, AKAP1, AKAP10, BLOC1S1, C1QBP, CSKMT, DELE1, EFHD1, FUNDC2, IFI27, LETM1, LYPLA1, MACROD1, MAVS, MCRIP2, MCU, MCUB, MICU1, MICU2, MICU3, MRS2, MUL1, NLRX1, OCIAD2, PDE2A, PINK1, PPIF, PPTC7, PRKACA, RHOT1, RHOT2, SIRT3, SLC25A12, SLC25A13, SLC25A23, SLC25A24, SLC25A25, SLC25A3, SLC25A31, SLC25A4, SLC25A5, SLC25A6, SLC8B1, SMDT1, SPHKAP, VDAC1, VDAC3
Calcium homeostasis Signaling > Calcium homeostasis EFHD1, LETM1, MCU, MCUB, MICU1, MICU2, MICU3, PPIF, RHOT1, RHOT2, SLC25A12, SLC25A13, SLC25A23, SLC25A24, SLC25A25, SLC25A3, SLC25A31, SLC25A4, SLC25A5, SLC25A6, SLC8B1, SMDT1, VDAC1, VDAC3
Calcium cycle Signaling > Calcium homeostasis > Calcium cycle LETM1, MCU, MCUB, MICU1, MICU2, MICU3, SLC25A3, SLC8B1, SMDT1
Mitochondrial permeability transition pore Signaling > Calcium homeostasis > Mitochondrial permeability transition pore PPIF, SLC25A31, SLC25A4, SLC25A5, SLC25A6, VDAC1, VDAC3
EF hand proteins Signaling > Calcium homeostasis > EF hand proteins EFHD1, MICU1, MICU2, MICU3, RHOT1, RHOT2, SLC25A12, SLC25A13, SLC25A23, SLC25A24, SLC25A25
Immune response Signaling > Immune response ACOD1, C1QBP, IFI27, MAVS, NLRX1
cAMP-PKA signaling Signaling > cAMP-PKA signaling ADCY10, AKAP1, AKAP10, PDE2A, PRKACA, SPHKAP
Mitochondrial dynamics and surveillance Mitochondrial dynamics and surveillance AHCYL1, AIFM1, AIFM2, AIFM3, APOO, APOOL, ARL2, ARMC10, ARMCX1, ARMCX3, ATAD3A, ATAD3B, ATP5MD, ATP5ME, ATP5MG, BAD, BAK1, BAX, BBC3, BCL2, BCL2A1, BCL2L1, BCL2L10, BCL2L11, BCL2L13, BCL2L2, BID, BIK, BNIP3, BNIP3L, BOK, CASP3, CASP8, CASP9, CHCHD2, CHCHD3, CHCHD6, CYCS, DIABLO, DNAJC11, DNM1L, ENDOG, FIS1, FKBP8, FUNDC1, FUNDC2, GHITM, HTRA2, IFI27, IMMT, MARCHF5, MCL1, MFF, MFN1, MFN2, MGARP, MICOS10, MICOS13, MIEF1, MIEF2, MIGA1, MIGA2, MTCH2, MTFP1, MTFR1, MTFR2, MTX1, MTX2, MTX3, MUL1, MYO19, NBR1, NIPSNAP1, NIPSNAP2, OMA1, OPA1, PARK7, PARL, PGAM5, PIGBOS1, PINK1, PLD6, PMAIP1, PRKN, RAB24, RHOT1, RHOT2, RMDN3, RTL10, SAMM50, SEPTIN4, SLC25A46, SNAP29, SPHK2, SPIRE1, STX17, STYXL1, SYNJ2BP, TMEM11, TOMM20, TOMM7, USP30, VDAC1, VPS13D
Fusion Mitochondrial dynamics and surveillance > Fusion ARL2, MFN1, MFN2, MIGA1, MIGA2, MTCH2, OMA1, OPA1, PLD6
Fission Mitochondrial dynamics and surveillance > Fission ARMC10, DNM1L, FIS1, MFF, MIEF1, MIEF2, MTFP1, MTFR1, MTFR2, MUL1, OMA1, RAB24, SLC25A46, SPIRE1, STX17
Organelle contact sites Mitochondrial dynamics and surveillance > Organelle contact sites AHCYL1, ATAD3A, ATAD3B, FKBP8, FUNDC2, MFN1, MFN2, MIGA2, PIGBOS1, RMDN3, SPIRE1, STX17, SYNJ2BP, VDAC1
Intramitochondrial membrane interactions Mitochondrial dynamics and surveillance > Intramitochondrial membrane interactions ATAD3A, ATAD3B, CHCHD3, DNAJC11, MTX1, MTX2, MTX3, SAMM50, SLC25A46
Trafficking Mitochondrial dynamics and surveillance > Trafficking ARMC10, ARMCX1, ARMCX3, MGARP, MYO19, RHOT1, RHOT2
Mitophagy Mitochondrial dynamics and surveillance > Mitophagy BCL2L13, FKBP8, FUNDC1, MARCHF5, NIPSNAP1, NIPSNAP2, PARK7, PARL, PGAM5, PINK1, PRKN, TOMM7, USP30, VPS13D
Autophagy Mitochondrial dynamics and surveillance > Autophagy BCL2L13, FKBP8, FUNDC1, MARCHF5, NBR1, NIPSNAP1, NIPSNAP2, PARK7, PARL, PGAM5, PINK1, PRKN, RAB24, SNAP29, STX17, TOMM7, USP30, VPS13D
Apoptosis Mitochondrial dynamics and surveillance > Apoptosis AIFM1, AIFM2, AIFM3, BAD, BAK1, BAX, BBC3, BCL2, BCL2A1, BCL2L1, BCL2L10, BCL2L11, BCL2L13, BCL2L2, BID, BIK, BNIP3, BNIP3L, BOK, CASP3, CASP8, CASP9, CHCHD2, CYCS, DIABLO, ENDOG, GHITM, HTRA2, IFI27, MCL1, PMAIP1, RTL10, SEPTIN4, SPHK2, STYXL1
Cristae formation Mitochondrial dynamics and surveillance > Cristae formation APOO, APOOL, ATP5MD, ATP5ME, ATP5MG, CHCHD3, CHCHD6, IMMT, MICOS10, MICOS13, OPA1
MICOS complex Mitochondrial dynamics and surveillance > Cristae formation > MICOS complex APOO, APOOL, CHCHD3, CHCHD6, IMMT, MICOS10, MICOS13