+D
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Mitochondrial Biogenesis
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AMitochondrial DNA transcription, translation, and replication factors
B Mitochondrial DNA-encoded proteins
C Mitochondrial respiratory chain complex I
D K03878 ND1; NADH-ubiquinone oxidoreductase chain 1 [EC:7.1.1.2]
D K03879 ND2; NADH-ubiquinone oxidoreductase chain 2 [EC:7.1.1.2]
D K03880 ND3; NADH-ubiquinone oxidoreductase chain 3 [EC:7.1.1.2]
D K03881 ND4; NADH-ubiquinone oxidoreductase chain 4 [EC:7.1.1.2]
D K03882 ND4L; NADH-ubiquinone oxidoreductase chain 4L [EC:7.1.1.2]
D K03883 ND5; NADH-ubiquinone oxidoreductase chain 5 [EC:7.1.1.2]
D K03884 ND6; NADH-ubiquinone oxidoreductase chain 6 [EC:7.1.1.2]
C Mitochondrial respiratory chain complex III
D K00412 CYTB, petB; ubiquinol-cytochrome c reductase cytochrome b subunit
C Cytochrome c oxidase
D K02256 COX1; cytochrome c oxidase subunit 1 [EC:7.1.1.9]
D K02261 COX2; cytochrome c oxidase subunit 2
D K02262 COX3; cytochrome c oxidase subunit 3
C F-type ATPase
D K02125 ATPeF08, MTATP8, ATP8; F-type H+-transporting ATPase subunit 8
D K02126 ATPeF0A, MTATP6, ATP6; F-type H+-transporting ATPase subunit a
B Mitochondrial transcription and translation factors
C Mitochondrial RNA polymerase
D K10908 POLRMT, RPO41; DNA-directed RNA polymerase, mitochondrial [EC:2.7.7.6]
C Mitochondrial transcription factors
D K11830 TFAM, MTTFA; transcription factor A, mitochondrial
D K15266 TFB1M; dimethyladenosine transferase 1, mitochondrial [EC:2.1.1.-]
D K17653 TFB2M; dimethyladenosine transferase 2, mitochondrial [EC:2.1.1.-]
C Mitochondrial translation factors
D K02519 infB, MTIF2; translation initiation factor IF-2
D K02520 infC, MTIF3; translation initiation factor IF-3
D K02357 tsf, TSFM; elongation factor Ts
D K02358 tuf, TUFM; elongation factor Tu
D K02355 fusA, GFM, EFG; elongation factor G
D K15031 MTERF; mitochondrial transcription termination factor family protein
D K21594 GUF1; translation factor GUF1, mitochondrial [EC:3.6.5.-]
C RNase MRP
D K01164 POP1; ribonuclease P/MRP protein subunit POP1 [EC:3.1.26.5]
D K14522 POP3; ribonuclease P/MRP protein subunit POP3 [EC:3.1.26.5]
D K03538 POP4, RPP29; ribonuclease P protein subunit POP4 [EC:3.1.26.5]
D K03537 POP5; ribonuclease P/MRP protein subunit POP5 [EC:3.1.26.5]
D K14524 POP6; ribonuclease P/MRP protein subunit POP6 [EC:3.1.26.5]
D K14526 POP7, RPP2; ribonuclease P/MRP protein subunit POP7 [EC:3.1.26.5]
D K14528 POP8; ribonuclease P/MRP protein subunit POP8 [EC:3.1.26.5]
D K03539 RPP1, RPP30; ribonuclease P/MRP protein subunit RPP1 [EC:3.1.26.5]
D K14527 RPP20, POP7; ribonuclease P/MRP protein subunit RPP20 [EC:3.1.26.5]
D K14525 RPP25; ribonucleases P/MRP protein subunit RPP25 [EC:3.1.26.5]
D K14523 RPP38; ribonucleases P/MRP protein subunit RPP38 [EC:3.1.26.5]
D K14530 RPP40; ribonucleases P/MRP protein subunit RPP40 [EC:3.1.26.5]
D K14531 SNM1; ribonuclease MRP protein subunit SNM1
C Mitochondrial ribonuclease P
D K17654 TRMT10C, MRPP1; mitochondrial ribonuclease P protein 1 [EC:2.1.1.-]
D K08683 HSD17B10; 3-hydroxyacyl-CoA dehydrogenase / 3-hydroxy-2-methylbutyryl-CoA dehydrogenase [EC:1.1.1.35 1.1.1.178]
D K17655 MRPP3; mitochondrial ribonuclease P protein 3
D K14533 RPM2; ribonuclease P protein component, mitochondrial [EC:3.1.26.5]
C Other mitochondrial DNA transcription and translation factors
D K17656 MSS51; mitochondrial splicing suppressor protein 51
D K17657 MRS1; mitochondrial RNA-splicing protein MRS1
D K17658 TEFM; transcription elongation factor, mitochondrial
D K17710 PTCD1; pentatricopeptide repeat domain-containing protein 1
D K17711 PTCD2; pentatricopeptide repeat domain-containing protein 2
D K17659 PTCD3; pentatricopeptide repeat domain-containing protein 3
D K13185 DHX30; ATP-dependent RNA helicase DHX30 [EC:5.6.2.6]
D K17660 CBP1; cytochrome b pre-mRNA-processing protein 1
D K17661 CBP2; cytochrome b pre-mRNA-processing protein 2
D K17662 CBP3, UQCC; cytochrome b pre-mRNA-processing protein 3
D K17663 CBP6; cytochrome b pre-mRNA-processing protein 6
D K17664 CBS1; cytochrome b translational activator protein CBS1, mitochondrial
D K17665 CBS2; cytochrome b translational activator protein CBS2, mitochondrial
D K17666 PET54; protein PET54, mitochondrial
D K17667 PET111; protein PET111, mitochondrial
D K17668 PET122; protein PET122, mitochondrial
D K17669 PET309; pentatricopeptide repeat-containing protein PET309
D K17670 PET494; COX3 mRNA-specific translational activator PET494
D K17671 AEP1; ATPase expression protein 1, mitochondrial
D K17672 AEP2; ATPase expression protein 2, mitochondrial
D K18155 AEP3; ATPase expression protein 3, mitochondrial
D K18798 AFG1, LACE1; peroxisome-assembly ATPase [EC:3.6.4.7]
D K17673 ATP22; mitochondrial translation factor ATP22
D K18156 ATP23, XRCC6BP1; mitochondrial inner membrane protease ATP23 [EC:3.4.24.-]
D K17674 SOV1; protein SOV1, mitochondrial
D K17675 SUPV3L1, SUV3; ATP-dependent RNA helicase SUPV3L1/SUV3 [EC:5.6.2.6]
D K19782 DSS1, MSU1; exoribonuclease II, mitochondrial [EC:3.1.13.1]
D K16639 LACTB2; endoribonuclease LACTB2 [EC:3.1.27.-]
D K05863 SLC25A4S, ANT; solute carrier family 25 (mitochondrial adenine nucleotide translocator), member 4/5/6/31
D K15113 SLC25A28_37, MFRN; solute carrier family 25 (mitochondrial iron transporter), member 28/37
D K25803 MALSU1; mitochondrial assembly of ribosomal large subunit protein 1
D K03595 era, ERAL1; GTPase
D K01866 YARS, tyrS; tyrosyl-tRNA synthetase [EC:6.1.1.1]
D K01869 LARS, leuS; leucyl-tRNA synthetase [EC:6.1.1.4]
D K01876 DARS2, aspS; aspartyl-tRNA synthetase [EC:6.1.1.12]
D K01880 GARS, glyS1; glycyl-tRNA synthetase [EC:6.1.1.14]
D K01887 RARS, argS; arginyl-tRNA synthetase [EC:6.1.1.19]
D K02433 gatA, QRSL1; aspartyl-tRNA(Asn)/glutamyl-tRNA(Gln) amidotransferase subunit A [EC:6.3.5.6 6.3.5.7]
D K02434 gatB, PET112; aspartyl-tRNA(Asn)/glutamyl-tRNA(Gln) amidotransferase subunit B [EC:6.3.5.6 6.3.5.7]
D K02435 gatC, GATC; aspartyl-tRNA(Asn)/glutamyl-tRNA(Gln) amidotransferase subunit C [EC:6.3.5.6 6.3.5.7]
D K18060 MTPAP; poly(A) RNA polymerase, mitochondrial [EC:2.7.7.19]
D K19612 PDE12; 2',5'-phosphodiesterase [EC:3.1.13.4 3.1.4.-]
D K19828 MTG1; mitochondrial GTPase 1
D K19832 NOA1; nitric oxide-associated protein 1
D K13427 NOA1; nitric-oxide synthase, plant [EC:1.14.13.39]
D K21969 NSUN3; tRNA (cytosine34-C5)-methyltransferase [EC:2.1.1.-]
D K21970 NSUN4; 5-methylcytosine rRNA methyltransferase NSUN4 [EC:2.1.1.-]
D K15452 PUS2; mitochondrial tRNA pseudouridine27/28 synthase [EC:5.4.99.44]
D K22537 RPUSD3; mitochondrial RNA pseudouridine synthase RPUSD3 [EC:5.4.99.-]
D K22538 RPUSD4; mitochondrial RNA pseudouridine synthase RPUSD4 [EC:5.4.99.-]
D K23495 RCC1L; RCC1-like G exchanging factor-like protein
D K23496 NGRN; neugrin
D K23497 TRUB2; mitochondrial mRNA pseudouridine synthase TRUB2 [EC:5.4.99.-]
D K18190 FASTKD2; FAST kinase domain-containing protein 2
D K23498 COXPD7; peptide chain release factor
D K23499 RMND1; required for meiotic nuclear division protein 1
D K24984 GRSF1; G-rich sequence factor 1
D K25876 METTL15; 12S rRNA N4-methylcytidine (m4C) methyltransferase [EC:2.1.1.-]
D K25877 MYG1; MYG1 exonuclease [EC:3.1.-.-]
D K26186 MTRES1; mitochondrial transcription rescue factor 1
B Mitochondrial DNA replication factors
C Mitochondrial DNA polymerase
D K02332 POLG; DNA polymerase gamma 1 [EC:2.7.7.7]
D K02333 POLG2; DNA polymerase gamma 2
D K22761 PRIMPOL; DNA-directed primase/polymerase protein [EC:2.7.7.102 2.7.7.7]
C Mitochondrial DNA maintenance factors
D K17676 CCE1; cruciform cutting endonuclease 1 [EC:3.1.21.10]
D K01173 ENDOG; endonuclease G, mitochondrial
D K19465 MGME1, DDK1; mitochondrial genome maintenance exonuclease 1 [EC:3.1.-.-]
D K15050 EXOG; nuclease EXOG, mitochondrial
D K17677 IRC3; ATP-dependent helicase IRC3 [EC:5.6.2.-]
D K17678 MRH4; ATP-dependent RNA helicase MRH4, mitochondrial [EC:5.6.2.7]
D K17679 MSS116; ATP-dependent RNA helicase MSS116, mitochondrial [EC:5.6.2.7]
D K17680 PEO1; twinkle protein [EC:5.6.2.3]
D K15255 PIF1; ATP-dependent DNA helicase PIF1 [EC:5.6.2.3]
D K19781 HMI1; ATP-dependent DNA helicase HMI1, mitochondrial [EC:5.6.2.4]
C Other Mitochondrial DNA replication factors
D K03111 ssb; single-strand DNA-binding protein
D K17681 ATAD3A_B; ATPase family AAA domain-containing protein 3A/B
D K17682 MNF1; mitochondrial nucleoid factor 1
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AMitochondrial protein import machinery
B Endoplasmic reticulum membrane and cytosol
C ER-mitochondria encounter structure (ERMES) complex
D K17764 MMM1; maintenance of mitochondrial morphology protein 1
D K17765 MDM12; mitochondrial distribution and morphology protein 12
C Other ER-memrane and cytosol factors
D K09503 DNAJA2; DnaJ homolog subfamily A member 2
D K17766 TOM34; mitochondrial import receptor subunit TOM34
D K07870 RHOT1, ARHT1; mitochondrial Rho GTPase 1 [EC:3.6.5.-]
D K03283 HSPA1_6_8; heat shock 70kDa protein 1/6/8
D K17767 AIP, XAP2; AH receptor-interacting protein
B Outer membrane
C Transporter outer membrane (TOM) complex
D K11518 TOM40; mitochondrial import receptor subunit TOM40
D K17768 TOM70; mitochondrial import receptor subunit TOM70
D K17769 TOM22; mitochondrial import receptor subunit TOM22
D K17770 TOM20; mitochondrial import receptor subunit TOM20
D K17771 TOM7; mitochondrial import receptor subunit TOM7
D K17772 TOM6; mitochondrial import receptor subunit TOM6
D K17773 TOM5; mitochondrial import receptor subunit TOM5
C TOB/SAM complex
D K07277 SAM50, TOB55, bamA; outer membrane protein insertion porin family
D K17714 SAM37, TOM37; sorting and assembly machinery component 37
D K17715 SAM35, TOM38; sorting and assembly machinery component 35
C Porin
D K05862 VDAC1; voltage-dependent anion channel protein 1
D K15040 VDAC2; voltage-dependent anion channel protein 2
D K15041 VDAC3; voltage-dependent anion channel protein 3
C ERMES complex
D K17774 MDM10; mitochondrial distribution and morphology protein 10
D K17775 MDM34, MMM2; mitochondrial distribution and morphology protein 34
C Other outer membrane factor
D K17776 MTX; metaxin
D K25838 MYO19; myosin XIX
D K26187 ARMCX1; armadillo repeat-containing X-linked protein 1
D K26188 ARMCX2; armadillo repeat-containing X-linked protein 2
D K26189 ARMCX3; armadillo repeat-containing X-linked protein 3
D K26190 ARMCX6; armadillo repeat-containing X-linked protein 6
D K27318 MTARC; mitochondrial amidoxime-reducing component [EC:1.7.-.-]
B Inter membrane space
C Transporter inner membrane (TIM) 9/10 complex
D K17777 TIM9; mitochondrial import inner membrane translocase subunit TIM9
D K17778 TIM10; mitochondrial import inner membrane translocase subunit TIM10
D K17779 TIM10B; mitochondrial import inner membrane translocase subunit TIM10B
C TIM8/13 complex
D K17780 TIM8; mitochondrial import inner membrane translocase subunit TIM8
D K17781 TIM13; mitochondrial import inner membrane translocase subunit TIM13
C Mitochondrial intermembrane space assembly (MIA) complex
D K17782 MIA40, CHCHD4; mitochondrial intermembrane space import and assembly protein 40
D K17783 ERV1, GFER, ALR; mitochondrial FAD-linked sulfhydryl oxidase [EC:1.8.3.2]
B Inner mambrane
C MICOS complex
D K17784 MICOS10, MINOS1, MIC10; MICOS complex subunit MIC10
D K17785 IMMT, MIC60; MICOS complex subunit MIC60
D K24624 MICOS13; MICOS complex subunit MIC13
D K17563 CHCHD3; MICOS complex subunit MIC19
D K17564 CHCHD6; MICOS complex subunit MIC25
D K24625 APOO; MICOS complex subunit MIC26
D K24626 APOOL; MICOS complex subunit MIC27
C MICOS complex (yeast)
D K17784 MICOS10, MINOS1, MIC10; MICOS complex subunit MIC10
D K17785 IMMT, MIC60; MICOS complex subunit MIC60
D K17787 MIC12; MICOS complex subunit MIC12
D K17788 MIC19; MICOS complex subunit MIC19
D K17786 MIC26; MICOS complex subunit MIC26
D K17789 MIC27; MICOS complex subunit MIC27
C TIM22 complex
D K17790 TIM22; mitochondrial import inner membrane translocase subunit TIM22
D K17791 TIM18; mitochondrial import inner membrane translocase subunit TIM18
D K17792 TIM54; mitochondrial import inner membrane translocase subunit TIM54
D K17793 TIM12; mitochondrial import inner membrane translocase subunit TIM12
C TIM23 complex
D K17794 TIM23; mitochondrial import inner membrane translocase subunit TIM23
D K17795 TIM17; mitochondrial import inner membrane translocase subunit TIM17
D K17796 TIM21; mitochondrial import inner membrane translocase subunit TIM21
D K17496 TIM50; mitochondrial import inner membrane translocase subunit TIM50
C Mitochondrial calcium uniporter complex
D K20858 MCU; calcium uniporter protein, mitochondrial
D K22827 MICU1; calcium uptake protein 1, mitochondrial
D K22828 MICU2; calcium uptake protein 2, mitochondrial
D K22829 MCUB; calcium uniporter regulatory subunit MCUb, mitochondrial
D K22830 SMDT1, EMRE; essential MCU regulator, mitochondrial
D K22137 MCUR1; mitochondrial calcium uniporter regulator 1
C Other inner membrane factors
D K17798 MSS2; mitochondrial protein MSS2
D K17799 PNT1; pentamidine resistance factor, mitochondrial
D K17800 LETM1, MDM38; LETM1 and EF-hand domain-containing protein 1, mitochondrial
D K17801 MBA1; mitochondrial protein MBA1
D K09647 IMP1; mitochondrial inner membrane protease subunit 1 [EC:3.4.21.-]
D K09648 IMP2; mitochondrial inner membrane protease subunit 2 [EC:3.4.21.-]
D K17802 SOM1; mitochondrial inner membrane protease subunit SOM1
D K17803 OMS1; methyltransferase OMS1, mitochondrial [EC:2.1.1.-]
D K18157 TMEM126A; transmembrane protein 126A
D K22138 MPC1; mitochondrial pyruvate carrier 1
D K22139 MPC2; mitochondrial pyruvate carrier 2
D K22759 CHCHD10; coiled-coil-helix-coiled-coil-helix domain-containing protein 10
B Matrix
C MMC/PAM proteins
D K17804 TIM44; mitochondrial import inner membrane translocase subunit TIM44
D K04043 dnaK, HSPA9; molecular chaperone DnaK
D K03687 GRPE; molecular chaperone GrpE
D K09539 DNAJC19; DnaJ homolog subfamily C member 19
D K17805 PAM16, TIM16; mitochondrial import inner membrane translocase subunit TIM16
D K17806 PAM17, TIM23; mitochondrial import inner membrane translocase subunit TIM23
C Other matrix factors
D K01412 PMPCA, MAS2; mitochondrial-processing peptidase subunit alpha [EC:3.4.24.64]
D K17732 PMPCB, MAS1; mitochondrial-processing peptidase subunit beta [EC:3.4.24.64]
D K01410 MIPEP; mitochondrial intermediate peptidase [EC:3.4.24.59]
D K04077 groEL, HSPD1; chaperonin GroEL [EC:5.6.1.7]
D K04078 groES, HSPE1; chaperonin GroES
D K03686 dnaJ; molecular chaperone DnaJ
D K09504 DNAJA3; DnaJ homolog subfamily A member 3
D K17807 TAM41, MMP37; mitochondrial translocator assembly and maintenance protein 41
D K17808 ZIM17, DNLZ, Tim15; mitochondrial protein import protein ZIM17
D K17725 ETHE1; sulfur dioxygenase [EC:1.13.11.18]
D K17809 POLDIP2; polymerase delta-interacting protein 2
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AMitochondrial quality control factors
B Regulator of mitochondrial biogenesis
C Ubiquitas transcription factors
D K05870 CREB1; cyclic AMP-responsive element-binding protein 1
D K02355 fusA, GFM, EFG; elongation factor G
D K04684 SP1; transcription factor Sp1
D K09201 YY; transcription factor YY
D K09454 GABPB; GA-binding protein transcription factor, beta
D K11830 TFAM, MTTFA; transcription factor A, mitochondrial
C Nuclear respiratory factors
D K11831 NRF1; nuclear respiratory factor 1
D K05638 NFE2L2, NRF2; nuclear factor erythroid 2-related factor 2
D K14739 MT1_2; metallothionein 1/2
D K14740 MT3; metallothionein 3
D K14741 MT4; metallothionein 4
C Coactivators
D K07202 PPARGC1A, PGC1A; alpha peroxisome proliferator-activated receptor gamma coactivator 1-alpha
D K17962 PPARGC1B, PGC1B; peroxisome proliferator-activated receptor gamma coactivator 1-beta
D K17963 PPRC1, PRC; peroxisome proliferator-activated receptor gamma coactivator-related protein 1
D K15309 CRTC1, TORC1; CREB-regulated transcription coactivator 1
D K04498 EP300, CREBBP, KAT3; E1A/CREB-binding protein [EC:2.3.1.48]
C Proteins involved in Fe-S cluster biogenesis
D K04487 iscS, NFS1; cysteine desulfurase [EC:2.8.1.7]
D K22068 ISCU; iron-sulfur cluster assembly enzyme ISCU, mitochondrial
D K22069 LYRM4; LYR motif-containing protein 4
D K19054 FXN; frataxin [EC:1.16.3.1]
D K22070 FDX1, ADX; ferredoxin-1, mitochondrial
D K22071 FDX2; ferredoxin-2, mitochondrial
D K18914 FDXR; adrenodoxin-NADP+ reductase [EC:1.18.1.6]
D K04043 dnaK, HSPA9; molecular chaperone DnaK
D K04082 hscB, HSCB, HSC20; molecular chaperone HscB
D K14001 SIL1, SLS1; nucleotide exchange factor SIL1
D K07390 grxD, GLRX5; monothiol glutaredoxin
D K22063 ISCA1; iron-sulfur cluster assembly 1
D K22072 ISCA2; iron-sulfur cluster assembly 2
D K22073 IBA57; transferase CAF17, mitochondrial [EC:2.1.-.-]
D K22074 NFU1, HIRIP5; NFU1 iron-sulfur cluster scaffold homolog, mitochondrial
D K22066 BOLA1; BolA-like protein 1
D K22075 BOLA3; BolA-like protein 3
D K23500 SFXN1_3; sideroflexin-1/3
D K23501 SFXN2; sideroflexin-2
D K23502 SFXN4; sideroflexin-4
D K23503 SFXN5; sideroflexin-5
C Other regulator of mitochondrial biogenesis
D K08765 CPT1A; carnitine O-palmitoyltransferase 1, liver isoform [EC:2.3.1.21]
D K19523 CPT1B; carnitine O-palmitoyltransferase 1, muscle isoform [EC:2.3.1.21]
D K19524 CPT1C; carnitine O-palmitoyltransferase 1, brain isoform [EC:2.3.1.21]
D K15791 DHKTD1; 2-oxoadipate dehydrogenase E1 component [EC:1.2.4.-]
D K10270 FBXL4; F-box and leucine-rich repeat protein 4
D K14966 HCFC1; host cell factor 1
D K27390 HCFC2; host cell factor 2
D K04533 HD; huntingtin
D K08552 NR3B1, ESRRA; estrogen-related receptor alpha
D K08553 NR3B2, ESRRB; estrogen-related receptor beta
D K08554 NR3B3, ESRRG; estrogen-related receptor gamma
D K17965 NRIP1; nuclear receptor-interacting protein 1
D K07198 PRKAA, AMPK; 5'-AMP-activated protein kinase, catalytic alpha subunit [EC:2.7.11.31]
D K08675 PRSS15, PIM1; ATP-dependent Lon protease [EC:3.4.21.53]
D K11411 SIRT1, SIR2L1; NAD-dependent protein deacetylase sirtuin 1 [EC:2.3.1.286]
D K23504 SERAC1; protein SERAC1
D K26537 UXT; protein UXT
D K26738 PERM1; PGC-1 and ERR-induced regulator in muscle protein 1
D K18158 NCA2; nuclear control of ATPase protein 2
B Mitochondrial respiratory chain complex assembly factors
C Complex-I assembly factors
D K18159 NDUFAF1, CIA30; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex assembly factor 1
D K18160 NDUFAF2; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex assembly factor 2
D K09008 NDUFAF3; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex assembly factor 3
D K18161 NDUFAF4; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex assembly factor 4
D K18162 NDUFAF5; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex assembly factor 5 [EC:2.1.1.-]
D K18163 NDUFAF6; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex assembly factor 6
D K18164 NDUFAF7; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex assembly factor 7
D K24726 NDUFAF8; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex assembly factor 8
D K18165 TMEM126B; complex I assembly factor TMEM126B
D K04405 ECSIT; evolutionarily conserved signaling intermediate in Toll pathway
D K15980 ACAD9; acyl-CoA dehydrogenase family member 9 [EC:1.3.99.-]
D K03593 mrp, NUBPL; ATP-binding protein involved in chromosome partitioning
D K18166 FOXRED1; FAD-dependent oxidoreductase domain-containing protein 1
D K23164 RTN4IP1; reticulon-4-interacting protein 1, mitochondrial
D K23505 TIMMDC1; complex I assembly factor TIMMDC1
C Complex-II assembly factors
D K18167 SDHAF1; succinate dehydrogenase assembly factor 1
D K18168 SDHAF2, SDH5; succinate dehydrogenase assembly factor 2
C Complex-III assembly factors
D K08900 BCS1; mitochondrial chaperone BCS1
D K18169 TTC19; tetratricopeptide repeat protein 19, mitochondrial
D K18170 LYRM7, MZM1; complex III assembly factor LYRM7
D K17662 CBP3, UQCC; cytochrome b pre-mRNA-processing protein 3
D K17682 MNF1; mitochondrial nucleoid factor 1
D K23165 UQCC3; ubiquinol-cytochrome-c reductase complex assembly factor 3
D K27339 UQCC4; ubiquinol-cytochrome c reductase complex assembly factor 4
D K27340 UQCC5, SMIM4; ubiquinol-cytochrome c reductase complex assembly factor 5
D K27341 UQCC6; ubiquinol-cytochrome c reductase complex assembly factor 6
D K27319 STMP1; short transmembrane mitochondrial protein 1
C Complex-IV assembly factors
D K18171 CMC1; COX assembly mitochondrial protein 1
D K18172 CMC2; COX assembly mitochondrial protein 2
D K18173 COA1; cytochrome c oxidase assembly factor 1
D K18174 COA2; cytochrome c oxidase assembly factor 2
D K18175 CCDC56, COA3; cytochrome c oxidase assembly factor 3, animal type
D K18176 COA3; cytochrome c oxidase assembly factor 3, fungi type
D K18177 COA4; cytochrome c oxidase assembly factor 4
D K18178 COA5, PET191; cytochrome c oxidase assembly factor 5
D K18179 COA6; cytochrome c oxidase assembly factor 6
D K18180 COA7, SELRC1, RESA1; cytochrome c oxidase assembly factor 7
D K02257 COX10, ctaB, cyoE; heme o synthase [EC:2.5.1.141]
D K02258 COX11, ctaG; cytochrome c oxidase assembly protein subunit 11
D K18181 COX14; cytochrome c oxidase assembly factor 14
D K02259 COX15, ctaA; heme a synthase [EC:1.17.99.9]
D K18182 COX16; cytochrome c oxidase assembly protein subunit 16
D K02260 COX17; cytochrome c oxidase assembly protein subunit 17
D K17797 COX18; mitochondrial inner membrane protein COX18
D K18183 COX19; cytochrome c oxidase assembly protein subunit 19
D K18184 COX20; cytochrome c oxidase assembly protein subunit 20
D K18185 COX23; cytochrome c oxidase assembly protein subunit 23
D K17801 MBA1; mitochondrial protein MBA1
D K17656 MSS51; mitochondrial splicing suppressor protein 51
D K18186 PET100; protein PET100, animal type
D K18187 PET100F; protein PET100, fungi type
D K18188 PET117; protein PET117
D K03217 yidC, spoIIIJ, OXA1, ccfA; YidC/Oxa1 family membrane protein insertase
D K14998 SURF1, SHY1; surfeit locus 1 family protein
D K07152 SCO1; protein SCO1
D K23755 SCO2; protein SCO2
D K18189 TACO1; translational activator of cytochrome c oxidase 1
D K17964 LRPPRC; leucine-rich PPR motif-containing protein, mitochondrial
D K23506 APOPT1; apoptogenic protein 1
D K27319 STMP1; short transmembrane mitochondrial protein 1
D K27340 UQCC5, SMIM4; ubiquinol-cytochrome c reductase complex assembly factor 5
D K27342 TMEM223; transmembrane protein 223
C Complex-V assembly factors
D K07555 ATPeAF1, ATPAF1, ATP11; ATP synthase mitochondrial F1 complex assembly factor 1
D K07556 ATPeAF2, ATPAF2, ATP12; ATP synthase mitochondrial F1 complex assembly factor 2
D K18191 FMC1; ATP synthase assembly factor FMC1, mitochondrial
D K17966 TMEM70; transmembrane protein 70, mitochondrial
D K18192 ATP10; mitochondrial ATPase complex subunit ATP10
D K18193 MP68, MLQ; 6.8 kDa mitochondrial proteolipid
D K18194 USMG5, DAPIT; up-regulated during skeletal muscle growth protein 5
B Mitochondrial dynamics
C Fission and Fusion factors
D K21356 MFN1; mitofusin 1 [EC:3.6.5.-]
D K06030 MFN2, FZO1; mitofusin 2 [EC:3.6.5.-]
D K22140 MGM1; dynamin-like GTPase MGM1, mitochondrial
D K17967 UGO1; mitochondrial fusion and transport protein UGO1
D K17079 OPA1; optic atrophy protein 1 [EC:3.6.5.5]
D K23166 OPA3; optic atrophy 3 protein
D K15070 MDM30; mitochondrial distribution and morphology protein 30
D K17968 TRIAP1, MDM35; TRIAP1/MDM35 family protein
D K09650 PARL, PSARL, PCP1; rhomboid-like protein [EC:3.4.21.105]
D K14021 BAK, BAK1; Bcl-2 homologous antagonist/killer
D K02159 BAX; apoptosis regulator BAX
D K17065 DNM1L; dynamin 1-like protein [EC:3.6.5.5]
D K17969 FIS1, TTC11, MDV2; mitochondrial fission 1 protein
D K17970 MDV1, FIS2; mitochondrial division protein 1
D K12160 SUMO, SMT3; small ubiquitin-related modifier
D K11248 SH3GLB1; endophilin-B1
D K17885 MTCH; mitochondrial carrier
D K22076 MFF; mitochondrial fission factor
D K22077 GDAP1; ganglioside-induced differentiation-associated protein 1
D K23167 VAT1; synaptic vesicle membrane protein VAT-1
D K23507 MIEF1_2; mitochondrial dynamics protein MID51/49
D K16862 PLD6; mitochondrial cardiolipin hydrolase [EC:3.1.4.-]
D K27289 MIGA, FAM73; mitoguardin
C Actin cytoskeleton organization
D K05692 ACTB_G1; actin beta/gamma 1
D K11238 BNI1; cytokinesis protein
D K17971 BNR1; BNI1-related protein 1
D K17945 TPM1_2; tropomyosin, fungi type
D K17972 NAA20, NAT3; N-terminal acetyltransferase B complex catalytic subunit [EC:2.3.1.254]
D K17973 NAA25, MDM20; N-terminal acetyltransferase B complex non-catalytic subunit
C Mitochondrial movement
D K17764 MMM1; maintenance of mitochondrial morphology protein 1
D K17774 MDM10; mitochondrial distribution and morphology protein 10
D K17775 MDM34, MMM2; mitochondrial distribution and morphology protein 34
D K17974 JSN1, PUF1; protein JSN1
D K17944 PUF3; mRNA-binding protein PUF3
D K17080 PHB1; prohibitin 1
D K17081 PHB2; prohibitin 2
D K17975 KTN1; kinectin
C Retention of mitochondria
D K17976 MMR1; mitochondrial MYO2 receptor-related protein 1
D K17065 DNM1L; dynamin 1-like protein [EC:3.6.5.5]
D K17977 YPT11; GTP-binding protein YPT11
D K10357 MYO5; myosin V
D K17978 NUM1; nuclear migration protein NUM1
D K04725 XIAP, BIRC4; E3 ubiquitin-protein ligase XIAP [EC:2.3.2.27]
D K10522 DIABLO, SMAC; diablo
C Morphogenesis factors
D K17887 SNX25, MDM1; sorting nexin-25
D K17979 MDM31; mitochondrial distribution and morphology protein 31
D K17980 MDM32; mitochondrial distribution and morphology protein 32
D K07870 RHOT1, ARHT1; mitochondrial Rho GTPase 1 [EC:3.6.5.-]
D K17785 IMMT, MIC60; MICOS complex subunit MIC60
D K17981 MTFP1, MTP18; mitochondrial fission process protein 1
D K17408 DAP3, MRPS29; small subunit ribosomal protein S29
D K10591 NEDD4, RSP5; E3 ubiquitin-protein ligase NEDD4 [EC:2.3.2.26]
D K25437 OXR1; oxidation resistance protein 1
D K27320 MSTO1, MST; protein misato
D K17983 SHE9; sensitive to high expression protein 9, mitochondrial
B Mitophagy factors
C Autophagy-related proteins
D K17906 ATG2; autophagy-related protein 2
D K08343 ATG3; ubiquitin-like-conjugating enzyme ATG3
D K08342 ATG4; cysteine protease ATG4 [EC:3.4.22.-]
D K08339 ATG5; autophagy-related protein 5
D K08334 BECN, VPS30, ATG6; beclin
D K08337 ATG7; ubiquitin-like modifier-activating enzyme ATG7
D K08341 GABARAP, ATG8, LC3; GABA(A) receptor-associated protein
D K17907 ATG9; autophagy-related protein 9
D K08338 ATG10; ubiquitin-like-conjugating enzyme ATG10
D K17888 ATG10L, ATG10; ubiquitin-like-conjugating enzyme ATG10
D K08330 ATG11; autophagy-related protein 11
D K08336 ATG12; ubiquitin-like protein ATG12
D K08335 ATG14; autophagy-related protein 14
D K17889 ATG14L, ATG14; beclin 1-associated autophagy-related key regulator
D K17900 ATG15, AUT5; lipase ATG15 [EC:3.1.1.3]
D K08340 ATG16; autophagy-related protein 16
D K17890 ATG16L1; autophagy-related protein 16-1
D K20868 ATG16L2; autophagy-related protein 16-2
D K08329 ATG17; autophagy-related protein 17
D K17908 WIPI1_2, ATG18; autophagy-related protein 18
D K17984 ATG32; autophagy-related protein 32
D K08332 VAC8; vacuolar protein 8
C Parkin-dependent mechanism factors
D K04556 PARK2; parkin [EC:2.3.2.31]
D K11981 RNF41, NRDP1; E3 ubiquitin-protein ligase NRDP1 [EC:2.3.2.27]
D K19513 CLEC16A; protein CLEC16A
D K05688 PINK1; PTEN induced putative kinase 1 [EC:2.7.11.1]
D K09650 PARL, PSARL, PCP1; rhomboid-like protein [EC:3.4.21.105]
D K14381 SQSTM1; sequestosome 1
D K21347 TAX1BP1; Tax1-binding protein 1
D K21348 CALCOCO2, NDP52; calcium-binding and coiled-coil domain-containing protein 2
D K17985 AMBRA1; activating molecule in BECN1-regulated autophagy protein 1
C Parkin-independent mechanism factors
D K08331 ATG13; autophagy-related protein 13
D K21357 ULK1; serine/threonine-protein kinase ULK1 [EC:2.7.11.1]
D K08269 ULK2, ATG1; serine/threonine-protein kinase ULK2 [EC:2.7.11.1]
D K17589 RB1CC1; RB1-inducible coiled-coil protein 1
D K04079 HSP90A, htpG; molecular chaperone HtpG
D K09554 CDC37; cell division cycle protein 37
D K04678 SMURF; E3 ubiquitin ligase SMURF1/2 [EC:2.3.2.26]
D K15465 BNIP3L; BCL2/adenovirus E1B 19 kDa protein-interacting protein 3-like
D K17986 FUNDC1; FUN14 domain-containing protein 1
C Other mitophagy factors
D K07198 PRKAA, AMPK; 5'-AMP-activated protein kinase, catalytic alpha subunit [EC:2.7.11.31]
D K10435 MAP1LC; microtubule-associated protein 1 light chain
D K17987 NBR1; next to BRCA1 gene 1 protein
D K17988 TECPR1; tectonin beta-propeller repeat-containing protein 1
D K17603 ZFYVE1; zinc finger FYVE domain-containing protein 1
D K02318 COASY; phosphopantetheine adenylyltransferase / dephospho-CoA kinase [EC:2.7.7.3 2.7.1.24]
D K16343 PLA2G6, IPLA2; calcium-independent phospholipase A2 [EC:3.1.1.4]
D K23168 MPAN; protein MPAN
D K24308 FUNDC2; FUN14 domain-containing protein 2
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#[ BRITE | KEGG2 | KEGG ]
#Last updated: March 14, 2024