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;
| MitoPathway | MitoPathways Hierarchy | Human 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 |