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Mitochondrial Biogenesis - Chlorocebus sabaeus (green monkey)
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AMitochondrial DNA transcription, translation, and replication factors
B Mitochondrial DNA-encoded proteins
C Mitochondrial respiratory chain complex I
D 4097488 ND1; NADH dehydrogenase subunit 1 K03878 ND1; NADH-ubiquinone oxidoreductase chain 1 [EC:7.1.1.2]
D 4097489 ND2; NADH dehydrogenase subunit 2 K03879 ND2; NADH-ubiquinone oxidoreductase chain 2 [EC:7.1.1.2]
D 4097495 ND3; NADH dehydrogenase subunit 3 K03880 ND3; NADH-ubiquinone oxidoreductase chain 3 [EC:7.1.1.2]
D 4097497 ND4; NADH dehydrogenase subunit 4 K03881 ND4; NADH-ubiquinone oxidoreductase chain 4 [EC:7.1.1.2]
D 4097496 ND4L; NADH dehydrogenase subunit 4L K03882 ND4L; NADH-ubiquinone oxidoreductase chain 4L [EC:7.1.1.2]
D 4097498 ND5; NADH dehydrogenase subunit 5 K03883 ND5; NADH-ubiquinone oxidoreductase chain 5 [EC:7.1.1.2]
D 4097499 ND6; NADH dehydrogenase subunit 6 K03884 ND6; NADH-ubiquinone oxidoreductase chain 6 [EC:7.1.1.2]
C Mitochondrial respiratory chain complex III
D 4097500 CYTB; cytochrome b K00412 CYTB; ubiquinol-cytochrome c reductase cytochrome b subunit
C Cytochrome c oxidase
D 4097490 COX1; cytochrome c oxidase subunit I K02256 COX1; cytochrome c oxidase subunit 1 [EC:7.1.1.9]
D 4097491 COX2; cytochrome c oxidase subunit II K02261 COX2; cytochrome c oxidase subunit 2
D 4097494 COX3; cytochrome c oxidase subunit III K02262 COX3; cytochrome c oxidase subunit 3
C F-type ATPase
D 4097492 ATP8; ATP synthase F0 subunit 8 K02125 ATPeF08; F-type H+-transporting ATPase subunit 8
D 4097493 ATP6; ATP synthase F0 subunit 6 K02126 ATPeF0A; F-type H+-transporting ATPase subunit a
B Mitochondrial transcription and translation factors
C Mitochondrial RNA polymerase
D 103234317 POLRMT; RNA polymerase mitochondrial K10908 POLRMT; DNA-directed RNA polymerase, mitochondrial [EC:2.7.7.6]
C Mitochondrial transcription factors
D 103216165 TFAM; transcription factor A, mitochondrial K11830 TFAM; transcription factor A, mitochondrial
D 103240762 TFB1M; transcription factor B1, mitochondrial K15266 TFB1M; dimethyladenosine transferase 1, mitochondrial [EC:2.1.1.-]
D 103230912 TFB2M; transcription factor B2, mitochondrial K17653 TFB2M; dimethyladenosine transferase 2, mitochondrial [EC:2.1.1.-]
C Mitochondrial translation factors
D 103220240 MTIF2; mitochondrial translational initiation factor 2 K02519 infB; translation initiation factor IF-2
D 103249057 MTIF3; mitochondrial translational initiation factor 3 K02520 infC; translation initiation factor IF-3
D 103238595 TSFM; Ts translation elongation factor, mitochondrial K02357 tsf; elongation factor Ts
D 103230326 TUFM; Tu translation elongation factor, mitochondrial K02358 tuf; elongation factor Tu
D 103223136 GFM2; G elongation factor mitochondrial 2 K02355 fusA; elongation factor G
D 103241412 GFM1; G elongation factor mitochondrial 1 K02355 fusA; elongation factor G
D 103218236 MTERF4; mitochondrial transcription termination factor 4 K15031 MTERF; mitochondrial transcription termination factor family protein
D 103226571 MTERF1; mitochondrial transcription termination factor 1 K15031 MTERF; mitochondrial transcription termination factor family protein
D 103237167 MTERF3; mitochondrial transcription termination factor 3 K15031 MTERF; mitochondrial transcription termination factor family protein
D 103239041 MTERF2; mitochondrial transcription termination factor 2 K15031 MTERF; mitochondrial transcription termination factor family protein
D 103246156 GUF1; GUF1 homolog, GTPase K21594 GUF1; translation factor GUF1, mitochondrial [EC:3.6.5.-]
C RNase MRP
D 103237188 POP1; POP1 homolog, ribonuclease P/MRP subunit K01164 POP1; ribonuclease P/MRP protein subunit POP1 [EC:3.1.26.5]
D 103234420 POP4; POP4 homolog, ribonuclease P/MRP subunit K03538 POP4; ribonuclease P protein subunit POP4 [EC:3.1.26.5]
D 103239262 POP5; POP5 homolog, ribonuclease P/MRP subunit K03537 POP5; ribonuclease P/MRP protein subunit POP5 [EC:3.1.26.5]
D 103216241 RPP30; ribonuclease P/MRP subunit p30 K03539 RPP1; ribonuclease P/MRP protein subunit RPP1 [EC:3.1.26.5]
D 103246721 POP7; POP7 homolog, ribonuclease P/MRP subunit K14527 RPP20; ribonuclease P/MRP protein subunit RPP20 [EC:3.1.26.5]
D 103219259 RPP25L; ribonuclease P/MRP subunit p25 like K14525 RPP25; ribonucleases P/MRP protein subunit RPP25 [EC:3.1.26.5]
D 103245319 RPP25; ribonuclease P/MRP subunit p25 K14525 RPP25; ribonucleases P/MRP protein subunit RPP25 [EC:3.1.26.5]
D 103237934 RPP38; ribonuclease P/MRP subunit p38 K14523 RPP38; ribonucleases P/MRP protein subunit RPP38 [EC:3.1.26.5]
D 103222248 RPP40; ribonuclease P/MRP subunit p40 K14530 RPP40; ribonucleases P/MRP protein subunit RPP40 [EC:3.1.26.5]
C Mitochondrial ribonuclease P
D 103228435 TRMT10C; tRNA methyltransferase 10C, mitochondrial RNase P subunit K17654 TRMT10C; mitochondrial ribonuclease P protein 1 [EC:2.1.1.-]
D 103232017 HSD17B10; hydroxysteroid 17-beta dehydrogenase 10 K08683 HSD17B10; 3-hydroxyacyl-CoA dehydrogenase / 3-hydroxy-2-methylbutyryl-CoA dehydrogenase [EC:1.1.1.35 1.1.1.178]
D 103228817 KIAA0391 ortholog K17655 MRPP3; mitochondrial ribonuclease P protein 3
C Other mitochondrial DNA transcription and translation factors
D 103216002 MSS51; MSS51 mitochondrial translational activator K17656 MSS51; mitochondrial splicing suppressor protein 51
D 103242684 TEFM; transcription elongation factor, mitochondrial K17658 TEFM; transcription elongation factor, mitochondrial
D 103246783 PTCD1; pentatricopeptide repeat domain 1 K17710 PTCD1; pentatricopeptide repeat domain-containing protein 1
D 103222927 PTCD2; pentatricopeptide repeat domain 2 K17711 PTCD2; pentatricopeptide repeat domain-containing protein 2
D 103219927 PTCD3; pentatricopeptide repeat domain 3 K17659 PTCD3; pentatricopeptide repeat domain-containing protein 3
D 103227564 DHX30; DExH-box helicase 30 K13185 DHX30; ATP-dependent RNA helicase DHX30 [EC:5.6.2.6]
D 103216057 UQCC1; ubiquinol-cytochrome c reductase complex assembly factor 1 K17662 CBP3; cytochrome b pre-mRNA-processing protein 3
D 103240618 LACE1; lactation elevated 1 K18798 AFG1; peroxisome-assembly ATPase [EC:3.6.4.7]
D 103238598 ATP23; ATP23 metallopeptidase and ATP synthase assembly factor homolog K18156 ATP23; mitochondrial inner membrane protease ATP23 [EC:3.4.24.-]
D 103216077 SUPV3L1; Suv3 like RNA helicase K17675 SUPV3L1; ATP-dependent RNA helicase SUPV3L1/SUV3 [EC:5.6.2.6]
D 103236951 LACTB2; lactamase beta 2 K16639 LACTB2; endoribonuclease LACTB2 [EC:3.1.27.-]
D 103220507 ADP/ATP translocase 2-like K05863 SLC25A4S; solute carrier family 25 (mitochondrial adenine nucleotide translocator), member 4/5/6/31
D 103236238 SLC25A31; solute carrier family 25 member 31 K05863 SLC25A4S; solute carrier family 25 (mitochondrial adenine nucleotide translocator), member 4/5/6/31
D 103236621 SLC25A4; solute carrier family 25 member 4 K05863 SLC25A4S; solute carrier family 25 (mitochondrial adenine nucleotide translocator), member 4/5/6/31
D 103247334 SLC25A5; solute carrier family 25 member 5 K05863 SLC25A4S; solute carrier family 25 (mitochondrial adenine nucleotide translocator), member 4/5/6/31
D 103247519 SLC25A6; solute carrier family 25 member 6 K05863 SLC25A4S; solute carrier family 25 (mitochondrial adenine nucleotide translocator), member 4/5/6/31
D 103215481 SLC25A37; solute carrier family 25 member 37 K15113 SLC25A28_37; solute carrier family 25 (mitochondrial iron transporter), member 28/37
D 103216373 SLC25A28; solute carrier family 25 member 28 K15113 SLC25A28_37; solute carrier family 25 (mitochondrial iron transporter), member 28/37
D 103226378 MALSU1; mitochondrial assembly of ribosomal large subunit 1 K25803 MALSU1; mitochondrial assembly of ribosomal large subunit protein 1
D 103242637 ERAL1; Era like 12S mitochondrial rRNA chaperone 1 K03595 era; GTPase
D 103218842 YARS2; tyrosyl-tRNA synthetase 2 K01866 YARS; tyrosyl-tRNA synthetase [EC:6.1.1.1]
D 103225174 YARS; tyrosyl-tRNA synthetase K01866 YARS; tyrosyl-tRNA synthetase [EC:6.1.1.1]
D 103228400 tyrosine--tRNA ligase, mitochondrial-like K01866 YARS; tyrosyl-tRNA synthetase [EC:6.1.1.1]
D 103227517 LARS2; leucyl-tRNA synthetase 2, mitochondrial K01869 LARS; leucyl-tRNA synthetase [EC:6.1.1.4]
D 103244738 LARS; leucyl-tRNA synthetase K01869 LARS; leucyl-tRNA synthetase [EC:6.1.1.4]
D 103230563 DARS2; aspartyl-tRNA synthetase 2, mitochondrial K01876 DARS2; aspartyl-tRNA synthetase [EC:6.1.1.12]
D 103226274 GARS; glycyl-tRNA synthetase K01880 GARS; glycyl-tRNA synthetase [EC:6.1.1.14]
D 103240072 RARS2; arginyl-tRNA synthetase 2, mitochondrial K01887 RARS; arginyl-tRNA synthetase [EC:6.1.1.19]
D 103244942 RARS; arginyl-tRNA synthetase K01887 RARS; arginyl-tRNA synthetase [EC:6.1.1.19]
D 103240646 QRSL1; glutaminyl-tRNA synthase (glutamine-hydrolyzing)-like 1 K02433 gatA; aspartyl-tRNA(Asn)/glutamyl-tRNA(Gln) amidotransferase subunit A [EC:6.3.5.6 6.3.5.7]
D 103236387 GATB; glutamyl-tRNA amidotransferase subunit B K02434 gatB; aspartyl-tRNA(Asn)/glutamyl-tRNA(Gln) amidotransferase subunit B [EC:6.3.5.6 6.3.5.7]
D 103239251 GATC; glutamyl-tRNA amidotransferase subunit C K02435 gatC; aspartyl-tRNA(Asn)/glutamyl-tRNA(Gln) amidotransferase subunit C [EC:6.3.5.6 6.3.5.7]
D 103238075 MTPAP; mitochondrial poly(A) polymerase K18060 MTPAP; poly(A) RNA polymerase, mitochondrial [EC:2.7.7.19]
D 103227798 PDE12; phosphodiesterase 12 K19612 PDE12; 2',5'-phosphodiesterase [EC:3.1.13.4 3.1.4.-]
D 103245971 MTG1; mitochondrial ribosome associated GTPase 1 K19828 MTG1; mitochondrial GTPase 1
D 103247084 NOA1; nitric oxide associated 1 K19832 NOA1; nitric oxide-associated protein 1
D 103228513 NSUN3; NOP2/Sun RNA methyltransferase family member 3 K21969 NSUN3; tRNA (cytosine34-C5)-methyltransferase [EC:2.1.1.-]
D 103224889 NSUN4; NOP2/Sun RNA methyltransferase family member 4 K21970 NSUN4; 5-methylcytosine rRNA methyltransferase NSUN4 [EC:2.1.1.-]
D 103227979 RPUSD3; RNA pseudouridylate synthase domain containing 3 K22537 RPUSD3; mitochondrial RNA pseudouridine synthase RPUSD3 [EC:5.4.99.-]
D 103248785 RPUSD4; RNA pseudouridylate synthase domain containing 4 K22538 RPUSD4; mitochondrial RNA pseudouridine synthase RPUSD4 [EC:5.4.99.-]
D 103246612 RCC1L; RCC1 like K23495 RCC1L; RCC1-like G exchanging factor-like protein
D 103231170 NGRN; neugrin, neurite outgrowth associated K23496 NGRN; neugrin
D 103239854 neugrin-like K23496 NGRN; neugrin
D 103239936 TRUB2; TruB pseudouridine synthase family member 2 K23497 TRUB2; mitochondrial mRNA pseudouridine synthase TRUB2 [EC:5.4.99.-]
D 103217271 FASTKD1; FAST kinase domains 1 K27754 FASTKD1; FAST kinase domain-containing protein 1
D 103217711 FASTKD2; FAST kinase domains 2 K18190 FASTKD2; FAST kinase domain-containing protein 2
D 103214984 FASTKD3; FAST kinase domains 3 K27755 FASTKD3; FAST kinase domain-containing protein 3
D 103226119 TBRG4; transforming growth factor beta regulator 4 K27756 TBRG4; FAST kinase domain-containing protein 4
D 103247184 FASTKD5; FAST kinase domains 5 K27757 FASTKD5; FAST kinase domain-containing protein 5
D 103239325 C11H12orf65; chromosome 11 open reading frame, human C12orf65 K23498 COXPD7; peptide chain release factor
D 103240730 RMND1; required for meiotic nuclear division 1 homolog K23499 RMND1; required for meiotic nuclear division protein 1
D 103235760 GRSF1; G-rich RNA sequence binding factor 1 K24984 GRSF1; G-rich sequence factor 1
D 103231710 probable methyltransferase-like protein 15 K25876 METTL15; 12S rRNA N4-methylcytidine (m4C) methyltransferase [EC:2.1.1.-]
D 103238516 METTL15; methyltransferase like 15 K25876 METTL15; 12S rRNA N4-methylcytidine (m4C) methyltransferase [EC:2.1.1.-]
D 103238390 C11H12orf10; chromosome 11 open reading frame, human C12orf10 K25877 MYG1; MYG1 exonuclease [EC:3.1.-.-]
D 103240637 C13H6orf203; chromosome 13 open reading frame, human C6orf203 K26186 MTRES1; mitochondrial transcription rescue factor 1
B Mitochondrial DNA replication factors
C Mitochondrial DNA polymerase
D 103231149 POLG; DNA polymerase gamma, catalytic subunit K02332 POLG; DNA polymerase gamma 1 [EC:2.7.7.7]
D 103243194 POLG2; DNA polymerase gamma 2, accessory subunit K02333 POLG2; DNA polymerase gamma 2
D 103236615 PRIMPOL; primase and DNA directed polymerase K22761 PRIMPOL; DNA-directed primase/polymerase protein [EC:2.7.7.102 2.7.7.7]
C Mitochondrial DNA maintenance factors
D 103239785 ENDOG; endonuclease G K01173 ENDOG; endonuclease G, mitochondrial
D 103214784 MGME1; mitochondrial genome maintenance exonuclease 1 K19465 MGME1; mitochondrial genome maintenance exonuclease 1 [EC:3.1.-.-]
D 103221054 EXOG; exo/endonuclease G K15050 EXOG; nuclease EXOG, mitochondrial
D 103216394 TWNK; twinkle mtDNA helicase K17680 PEO1; twinkle protein [EC:5.6.2.3]
D 103245486 PIF1; PIF1 5'-to-3' DNA helicase K15255 PIF1; ATP-dependent DNA helicase PIF1 [EC:5.6.2.3]
C Other Mitochondrial DNA replication factors
D 103227037 SSBP1; single stranded DNA binding protein 1 K03111 ssb; single-strand DNA-binding protein
D 103225792 ATAD3C; ATPase family, AAA domain containing 3C K17681 ATAD3A_B; ATPase family AAA domain-containing protein 3A/B
D 103225992 ATAD3A; ATPase family, AAA domain containing 3A K17681 ATAD3A_B; ATPase family AAA domain-containing protein 3A/B
D 103221650 UQCC2; ubiquinol-cytochrome c reductase complex assembly factor 2 K17682 MNF1; mitochondrial nucleoid factor 1
D 103243111 C16H17orf80; chromosome 16 open reading frame, human C17orf80 K27759 MTNAP1; mitochondrial nucleoid-associated protein 1
D 103227223 GIMAP1; GTPase, IMAP family member 1 K27758 GIMAP1_3_5; GTPase IMAP family member 1/3/5
D 103227224 GTPase IMAP family member 5 K27758 GIMAP1_3_5; GTPase IMAP family member 1/3/5
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AMitochondrial protein import machinery
B Endoplasmic reticulum membrane and cytosol
C ER-mitochondria encounter structure (ERMES) complex
C Other ER-memrane and cytosol factors
D 103232247 DNAJA2; DnaJ heat shock protein family (Hsp40) member A2 K09503 DNAJA2; DnaJ homolog subfamily A member 2
D 103245508 TOMM34; translocase of outer mitochondrial membrane 34 K17766 TOM34; mitochondrial import receptor subunit TOM34
D 103243817 RHOT1; ras homolog family member T1 K07870 RHOT1; mitochondrial Rho GTPase 1 [EC:3.6.5.-]
D 103221743 HSPA1B; heat shock protein family A (Hsp70) member 1B K03283 HSPA1_6_8; heat shock 70kDa protein 1/6/8
D 103221744 HSPA1L; heat shock protein family A (Hsp70) member 1 like K03283 HSPA1_6_8; heat shock 70kDa protein 1/6/8
D 103221745 HSPA1A; heat shock protein family A (Hsp70) member 1A K03283 HSPA1_6_8; heat shock 70kDa protein 1/6/8
D 103223677 HSPA6; heat shock protein family A (Hsp70) member 6 K03283 HSPA1_6_8; heat shock 70kDa protein 1/6/8
D 103230925 heat shock cognate 71 kDa protein-like K03283 HSPA1_6_8; heat shock 70kDa protein 1/6/8
D 103248693 HSPA8; heat shock protein family A (Hsp70) member 8 K03283 HSPA1_6_8; heat shock 70kDa protein 1/6/8
D 103217314 AIP; aryl hydrocarbon receptor interacting protein K17767 AIP; AH receptor-interacting protein
D 103242250 AIPL1; aryl hydrocarbon receptor interacting protein like 1 K17767 AIP; AH receptor-interacting protein
D 103232750 FATE1; fetal and adult testis expressed 1 K28235 FATE1; fetal and adult testis-expressed transcript protein
B Outer membrane
C Transporter outer membrane (TOM) complex
D 103219178 TOMM5; translocase of outer mitochondrial membrane 5 K17773 TOM5; mitochondrial import receptor subunit TOM5
D 103238805 mitochondrial import receptor subunit TOM5 homolog K17773 TOM5; mitochondrial import receptor subunit TOM5
D 103221508 TOMM6; translocase of outer mitochondrial membrane 6 K17772 TOM6; mitochondrial import receptor subunit TOM6
D 103226389 TOMM7; translocase of outer mitochondrial membrane 7 K17771 TOM7; mitochondrial import receptor subunit TOM7
D 103229082 TOMM20L; translocase of outer mitochondrial membrane 20 like K17770 TOM20; mitochondrial import receptor subunit TOM20
D 103230793 TOMM20; translocase of outer mitochondrial membrane 20 K17770 TOM20; mitochondrial import receptor subunit TOM20
D 103223321 TOMM22; translocase of outer mitochondrial membrane 22 K17769 TOM22; mitochondrial import receptor subunit TOM22
D 103223683 TOMM40L; translocase of outer mitochondrial membrane 40 like K11518 TOM40; mitochondrial import receptor subunit TOM40
D 103234840 TOMM40; translocase of outer mitochondrial membrane 40 K11518 TOM40; mitochondrial import receptor subunit TOM40
D 103228453 TOMM70; translocase of outer mitochondrial membrane 70 K17768 TOM70; mitochondrial import receptor subunit TOM70
C TOB/SAM complex
D 103223454 SAMM50; SAMM50 sorting and assembly machinery component K07277 SAM50; outer membrane protein insertion porin family
C Porin
D 103244541 VDAC1; voltage dependent anion channel 1 K05862 VDAC1; voltage-dependent anion channel protein 1
D 103215964 VDAC2; voltage dependent anion channel 2 K15040 VDAC2; voltage-dependent anion channel protein 2
D 103215691 VDAC3; voltage dependent anion channel 3 K15041 VDAC3; voltage-dependent anion channel protein 3
C ERMES complex
C Other outer membrane factor
D 103217384 MTX2; metaxin 2 K17776 MTX; metaxin
D 103223741 MTX3; metaxin 3 K17776 MTX; metaxin
D 103223888 MTX1; metaxin 1 K17776 MTX; metaxin
D 103243825 MYO19; myosin XIX K25838 MYO19; myosin XIX
D 103232366 ARMCX1; armadillo repeat containing, X-linked 1 K26187 ARMCX1; armadillo repeat-containing X-linked protein 1
D 103232370 ARMCX2; armadillo repeat containing, X-linked 2 K26188 ARMCX2; armadillo repeat-containing X-linked protein 2
D 103232369 ARMCX3; armadillo repeat containing, X-linked 3 K26189 ARMCX3; armadillo repeat-containing X-linked protein 3
D 103232368 protein ARMCX6 K26190 ARMCX6; armadillo repeat-containing X-linked protein 6
D 103230051 MARC1; mitochondrial amidoxime reducing component 1 K27318 MTARC; mitochondrial amidoxime-reducing component [EC:1.7.-.-]
D 103230056 MARC2; mitochondrial amidoxime reducing component 2 K27318 MTARC; mitochondrial amidoxime-reducing component [EC:1.7.-.-]
B Inter membrane space
C Transporter inner membrane (TIM) 9/10 complex
D 103235410 TIMM10; translocase of inner mitochondrial membrane 10 K17778 TIM10; mitochondrial import inner membrane translocase subunit TIM10
D 103241440 TIMM10B; translocase of inner mitochondrial membrane 10B K17779 TIM10B; mitochondrial import inner membrane translocase subunit TIM10B
C TIM8/13 complex
D 103232358 TIMM8A; translocase of inner mitochondrial membrane 8 homolog A (yeast) K17780 TIM8; mitochondrial import inner membrane translocase subunit TIM8
D 103248456 TIMM8B; translocase of inner mitochondrial membrane 8 homolog B K17780 TIM8; mitochondrial import inner membrane translocase subunit TIM8
D 103233632 TIMM13; translocase of inner mitochondrial membrane 13 K17781 TIM13; mitochondrial import inner membrane translocase subunit TIM13
C Mitochondrial intermembrane space assembly (MIA) complex
D 103228045 CHCHD4; coiled-coil-helix-coiled-coil-helix domain containing 4 K17782 MIA40; mitochondrial intermembrane space import and assembly protein 40
D 103226489 GFER; growth factor, augmenter of liver regeneration K17783 ERV1; mitochondrial FAD-linked sulfhydryl oxidase [EC:1.8.3.2]
B Inner mambrane
C MICOS complex
D 103225475 MINOS1; mitochondrial inner membrane organizing system 1 K17784 MICOS10; MICOS complex subunit MIC10
D 103220987 IMMT; inner membrane mitochondrial protein K17785 IMMT; MICOS complex subunit MIC60
D 103233742 C6H19orf70; chromosome 6 open reading frame, human C19orf70 K24624 MICOS13; MICOS complex subunit MIC13
D 103221334 coiled-coil-helix-coiled-coil-helix domain-containing protein 3, mitochondrial-like K17563 CHCHD3; MICOS complex subunit MIC19
D 103226919 CHCHD3; coiled-coil-helix-coiled-coil-helix domain containing 3 K17563 CHCHD3; MICOS complex subunit MIC19
D 103228115 CHCHD6; coiled-coil-helix-coiled-coil-helix domain containing 6 K17564 CHCHD6; MICOS complex subunit MIC25
D 103231719 APOO; apolipoprotein O K24625 APOO; MICOS complex subunit MIC26
D 103232282 APOOL; apolipoprotein O like K24626 APOOL; MICOS complex subunit MIC27
C MICOS complex (yeast)
D 103225475 MINOS1; mitochondrial inner membrane organizing system 1 K17784 MICOS10; MICOS complex subunit MIC10
D 103220987 IMMT; inner membrane mitochondrial protein K17785 IMMT; MICOS complex subunit MIC60
C TIM22 complex
D 103242072 TIMM22; translocase of inner mitochondrial membrane 22 K17790 TIM22; mitochondrial import inner membrane translocase subunit TIM22
C TIM23 complex
D 103215822 mitochondrial import inner membrane translocase subunit Tim23 K17794 TIM23; mitochondrial import inner membrane translocase subunit TIM23
D 103230321 TIMM17A; translocase of inner mitochondrial membrane 17 homolog A (yeast) K17795 TIM17; mitochondrial import inner membrane translocase subunit TIM17
D 103231946 TIMM17B; translocase of inner mitochondrial membrane 17 homolog B (yeast) K17795 TIM17; mitochondrial import inner membrane translocase subunit TIM17
D 103244093 TIMM21; translocase of inner mitochondrial membrane 21 K17796 TIM21; mitochondrial import inner membrane translocase subunit TIM21
D 103235503 TIMM50; translocase of inner mitochondrial membrane 50 K17496 TIM50; mitochondrial import inner membrane translocase subunit TIM50
C Mitochondrial calcium uniporter complex
D 103216021 MCU; mitochondrial calcium uniporter K20858 MCU; calcium uniporter protein, mitochondrial
D 103216024 MICU1; mitochondrial calcium uptake 1 K22827 MICU1; calcium uptake protein 1, mitochondrial
D 103248988 MICU2; mitochondrial calcium uptake 2 K22828 MICU2; calcium uptake protein 2, mitochondrial
D 103236103 MCUB; mitochondrial calcium uniporter dominant negative beta subunit K22829 MCUB; calcium uniporter regulatory subunit MCUb, mitochondrial
D 103223409 SMDT1; single-pass membrane protein with aspartate rich tail 1 K22830 SMDT1; essential MCU regulator, mitochondrial
D 103222140 MCUR1; mitochondrial calcium uniporter regulator 1 K22137 MCUR1; mitochondrial calcium uniporter regulator 1
C Other inner membrane factors
D 103246477 LETM1; leucine zipper and EF-hand containing transmembrane protein 1 K17800 LETM1; LETM1 and EF-hand domain-containing protein 1, mitochondrial
D 103238250 IMMP1L; inner mitochondrial membrane peptidase subunit 1 K09647 IMP1; mitochondrial inner membrane protease subunit 1 [EC:3.4.21.-]
D 103226745 IMMP2L; inner mitochondrial membrane peptidase subunit 2 K09648 IMP2; mitochondrial inner membrane protease subunit 2 [EC:3.4.21.-]
D 103248135 TMEM126A; transmembrane protein 126A K18157 TMEM126A; transmembrane protein 126A
D 103231815 MPC1L; mitochondrial pyruvate carrier 1-like K22138 MPC1; mitochondrial pyruvate carrier 1
D 103240904 MPC1; mitochondrial pyruvate carrier 1 K22138 MPC1; mitochondrial pyruvate carrier 1
D 103230647 MPC2; mitochondrial pyruvate carrier 2 K22139 MPC2; mitochondrial pyruvate carrier 2
D 103223022 CHCHD10; coiled-coil-helix-coiled-coil-helix domain containing 10 K22759 CHCHD10; coiled-coil-helix-coiled-coil-helix domain-containing protein 10
D 103246123 OCIAD1; OCIA domain containing 1 K28215 OCIAD1; OCIA domain-containing protein 1
B Matrix
C MMC/PAM proteins
D 103233826 TIMM44; translocase of inner mitochondrial membrane 44 K17804 TIM44; mitochondrial import inner membrane translocase subunit TIM44
D 103244606 HSPA9; heat shock protein family A (Hsp70) member 9 K04043 dnaK; molecular chaperone DnaK
D 103244775 GRPEL2; GrpE like 2, mitochondrial K03687 GRPE; molecular chaperone GrpE
D 103246420 GRPEL1; GrpE like 1, mitochondrial K03687 GRPE; molecular chaperone GrpE
D 103221183 DNAJC19; DnaJ heat shock protein family (Hsp40) member C19 K09539 DNAJC19; DnaJ homolog subfamily C member 19
D 103227625 PAM16; presequence translocase associated motor 16 homolog K17805 PAM16; mitochondrial import inner membrane translocase subunit TIM16
C Other matrix factors
D 103239628 PMPCA; peptidase, mitochondrial processing alpha subunit K01412 PMPCA; mitochondrial-processing peptidase subunit alpha [EC:3.4.24.64]
D 103226683 PMPCB; peptidase, mitochondrial processing beta subunit K17732 PMPCB; mitochondrial-processing peptidase subunit beta [EC:3.4.24.64]
D 103249000 MIPEP; mitochondrial intermediate peptidase K01410 MIPEP; mitochondrial intermediate peptidase [EC:3.4.24.59]
D 103217584 HSPD1; heat shock protein family D (Hsp60) member 1 K04077 groEL; chaperonin GroEL [EC:5.6.1.7]
D 103231778 DNAJA3; DnaJ heat shock protein family (Hsp40) member A3 K09504 DNAJA3; DnaJ homolog subfamily A member 3
D 103228019 TAMM41; TAM41 mitochondrial translocator assembly and maintenance homolog K17807 TAM41; mitochondrial translocator assembly and maintenance protein 41
D 103239633 DNLZ; DNL-type zinc finger K17808 ZIM17; mitochondrial protein import protein ZIM17
D 103234782 ETHE1; ETHE1, persulfide dioxygenase K17725 ETHE1; sulfur dioxygenase [EC:1.13.11.18]
D 103242611 POLDIP2; DNA polymerase delta interacting protein 2 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 103217717 CREB1; cAMP responsive element binding protein 1 K05870 CREB1; cyclic AMP-responsive element-binding protein 1
D 103223136 GFM2; G elongation factor mitochondrial 2 K02355 fusA; elongation factor G
D 103241412 GFM1; G elongation factor mitochondrial 1 K02355 fusA; elongation factor G
D 103238395 SP1; Sp1 transcription factor K04684 SP1; transcription factor Sp1
D 103229690 YY1; YY1 transcription factor K09201 YY; transcription factor YY
D 103231699 YY2; YY2 transcription factor K09201 YY; transcription factor YY
D 103224052 GABPB2; GA binding protein transcription factor beta subunit 2 K09454 GABPB; GA-binding protein transcription factor, beta
D 103245639 GABPB1; GA binding protein transcription factor beta subunit 1 K09454 GABPB; GA-binding protein transcription factor, beta
D 103216165 TFAM; transcription factor A, mitochondrial K11830 TFAM; transcription factor A, mitochondrial
C Nuclear respiratory factors
D 103226884 NRF1; nuclear respiratory factor 1 K11831 NRF1; nuclear respiratory factor 1
D 103217393 NFE2L2; nuclear factor, erythroid 2 like 2 K05638 NFE2L2; nuclear factor erythroid 2-related factor 2
D 103226962 metallothionein-2-like K14739 MT1_2; metallothionein 1/2
D 103233029 metallothionein-2 K14739 MT1_2; metallothionein 1/2
D 103233030 metallothionein-1E-like K14739 MT1_2; metallothionein 1/2
D 103233031 metallothionein-1E K14739 MT1_2; metallothionein 1/2
D 103233032 metallothionein-2-like K14739 MT1_2; metallothionein 1/2
D 103233033 metallothionein-2A-like K14739 MT1_2; metallothionein 1/2
D 103233034 metallothionein-2-like K14739 MT1_2; metallothionein 1/2
D 103233035 metallothionein-1E-like K14739 MT1_2; metallothionein 1/2
D 103233036 metallothionein-1E-like K14739 MT1_2; metallothionein 1/2
D 103233038 metallothionein-1E-like K14739 MT1_2; metallothionein 1/2
D 103233040 metallothionein-1H-like K14739 MT1_2; metallothionein 1/2
D 103233041 metallothionein-1E-like K14739 MT1_2; metallothionein 1/2
D 103233042 metallothionein-1E-like K14739 MT1_2; metallothionein 1/2
D 103233043 metallothionein-1 K14739 MT1_2; metallothionein 1/2
D 103233044 MT1X; metallothionein 1X K14739 MT1_2; metallothionein 1/2
D 103233026 MT3; metallothionein 3 K14740 MT3; metallothionein 3
C Coactivators
D 103246278 PPARGC1A; PPARG coactivator 1 alpha K07202 PPARGC1A; alpha peroxisome proliferator-activated receptor gamma coactivator 1-alpha
D 103244783 PPARGC1B; PPARG coactivator 1 beta K17962 PPARGC1B; peroxisome proliferator-activated receptor gamma coactivator 1-beta
D 103216424 PPRC1; peroxisome proliferator-activated receptor gamma, coactivator-related 1 K17963 PPRC1; peroxisome proliferator-activated receptor gamma coactivator-related protein 1
D 103234193 CRTC1; CREB regulated transcription coactivator 1 K15309 CRTC1; CREB-regulated transcription coactivator 1
D 103223371 EP300; E1A binding protein p300 K04498 EP300; E1A/CREB-binding protein [EC:2.3.1.48]
D 103231770 CREBBP; CREB binding protein K04498 EP300; E1A/CREB-binding protein [EC:2.3.1.48]
C Proteins involved in Fe-S cluster biogenesis
D 103216262 NFS1; NFS1, cysteine desulfurase K04487 iscS; cysteine desulfurase [EC:2.8.1.7]
D 103239070 ISCU; iron-sulfur cluster assembly enzyme K22068 ISCU; iron-sulfur cluster assembly enzyme ISCU, mitochondrial
D 103222244 LYRM4; LYR motif containing 4 K22069 LYRM4; LYR motif-containing protein 4
D 103219550 FXN; frataxin K19054 FXN; frataxin [EC:1.16.3.1]
D 103248424 FDX1; ferredoxin 1 K22070 FDX1; ferredoxin-1, mitochondrial
D 103233896 FDX1L; ferredoxin 1 like K22071 FDX2; ferredoxin-2, mitochondrial
D 103243070 FDXR; ferredoxin reductase K18914 FDXR; adrenodoxin-NADP+ reductase [EC:1.18.1.6]
D 103244606 HSPA9; heat shock protein family A (Hsp70) member 9 K04043 dnaK; molecular chaperone DnaK
D 103223121 HSCB; HscB mitochondrial iron-sulfur cluster cochaperone K04082 hscB; molecular chaperone HscB
D 103244629 SIL1; SIL1 nucleotide exchange factor K14001 SIL1; nucleotide exchange factor SIL1
D 103229631 GLRX5; glutaredoxin 5 K07390 grxD; monothiol glutaredoxin
D 103219682 ISCA1; iron-sulfur cluster assembly 1 K22063 ISCA1; iron-sulfur cluster assembly 1
D 103234267 iron-sulfur cluster assembly 1 homolog, mitochondrial-like K22063 ISCA1; iron-sulfur cluster assembly 1
D 103229331 ISCA2; iron-sulfur cluster assembly 2 K22072 ISCA2; iron-sulfur cluster assembly 2
D 103229960 IBA57; IBA57 homolog, iron-sulfur cluster assembly K22073 IBA57; transferase CAF17, mitochondrial [EC:2.1.-.-]
D 103220111 NFU1; NFU1 iron-sulfur cluster scaffold K22074 NFU1; NFU1 iron-sulfur cluster scaffold homolog, mitochondrial
D 103247170 BOLA1; bolA family member 1 K22066 BOLA1; BolA-like protein 1
D 103220040 BOLA3; bolA family member 3 K22075 BOLA3; BolA-like protein 3
D 103216400 SFXN3; sideroflexin 3 K23500 SFXN1_3; sideroflexin-1/3
D 103245017 SFXN1; sideroflexin 1 K23500 SFXN1_3; sideroflexin-1/3
D 103216443 SFXN2; sideroflexin 2 K23501 SFXN2; sideroflexin-2
D 103216601 SFXN4; sideroflexin 4 K23502 SFXN4; sideroflexin-4
D 103220062 SFXN5; sideroflexin 5 K23503 SFXN5; sideroflexin-5
C Other regulator of mitochondrial biogenesis
D 103248480 CPT1A; carnitine palmitoyltransferase 1A K08765 CPT1A; carnitine O-palmitoyltransferase 1, liver isoform [EC:2.3.1.21]
D 103223575 CPT1B; carnitine palmitoyltransferase 1B K19523 CPT1B; carnitine O-palmitoyltransferase 1, muscle isoform [EC:2.3.1.21]
D 103235051 CPT1C; carnitine palmitoyltransferase 1C K19524 CPT1C; carnitine O-palmitoyltransferase 1, brain isoform [EC:2.3.1.21]
D 103238141 DHTKD1; dehydrogenase E1 and transketolase domain containing 1 K15791 DHKTD1; 2-oxoadipate dehydrogenase E1 component [EC:1.2.4.-]
D 103240695 FBXL4; F-box and leucine rich repeat protein 4 K10270 FBXL4; F-box and leucine-rich repeat protein 4
D 103232813 HCFC1; host cell factor C1 K14966 HCFC1; host cell factor 1
D 103238981 HCFC2; host cell factor C2 K27390 HCFC2; host cell factor 2
D 103246449 HTT; huntingtin K04533 HD; huntingtin
D 103233248 ESRRA; estrogen related receptor alpha K08552 NR3B1; estrogen-related receptor alpha
D 103229373 ESRRB; estrogen related receptor beta K08553 NR3B2; estrogen-related receptor beta
D 103230089 ESRRG; estrogen related receptor gamma K08554 NR3B3; estrogen-related receptor gamma
D 103218226 NRIP1; nuclear receptor interacting protein 1 K17965 NRIP1; nuclear receptor-interacting protein 1
D 103215177 PRKAA1; protein kinase AMP-activated catalytic subunit alpha 1 K07198 PRKAA; 5'-AMP-activated protein kinase, catalytic alpha subunit [EC:2.7.11.31]
D 103224740 PRKAA2; protein kinase AMP-activated catalytic subunit alpha 2 K07198 PRKAA; 5'-AMP-activated protein kinase, catalytic alpha subunit [EC:2.7.11.31]
D 103233746 LONP1; lon peptidase 1, mitochondrial K08675 PRSS15; ATP-dependent Lon protease [EC:3.4.21.53]
D 103216104 SIRT1; sirtuin 1 K11411 SIRT1; NAD-dependent protein deacetylase sirtuin 1 [EC:2.3.1.286]
D 103240796 SERAC1; serine active site containing 1 K23504 SERAC1; protein SERAC1
D 103231900 UXT; ubiquitously expressed prefoldin like chaperone K26537 UXT; protein UXT
D 103225836 PGC-1 and ERR-induced regulator in muscle protein 1-like K26738 PERM1; PGC-1 and ERR-induced regulator in muscle protein 1
D 103222991 TANGO2; transport and golgi organization 2 homolog K28098 TANGO2; transport and Golgi organization protein 2
D 103217602 C10H2orf69; chromosome 10 open reading frame, human C2orf69 K28271 C2ORF69; mitochondrial protein C2orf69
D 103219231 FAM214B; family with sequence similarity 214 member B K28528 ATOS; protein Atossa
D 103245603 FAM214A; family with sequence similarity 214 member A K28528 ATOS; protein Atossa
D 103218649 DDX47; DEAD-box helicase 47 K14777 DDX47; ATP-dependent RNA helicase DDX47/RRP3 [EC:5.6.2.7]
B Mitochondrial respiratory chain complex assembly factors
C Complex-I assembly factors
D 103245793 NDUFAF1; NADH:ubiquinone oxidoreductase complex assembly factor 1 K18159 NDUFAF1; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex assembly factor 1
D 103221988 NDUFAF2; NADH:ubiquinone oxidoreductase complex assembly factor 2 K18160 NDUFAF2; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex assembly factor 2
D 103227612 NDUFAF3; NADH:ubiquinone oxidoreductase complex assembly factor 3 K09008 NDUFAF3; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex assembly factor 3
D 103240706 NDUFAF4; NADH:ubiquinone oxidoreductase complex assembly factor 4 K18161 NDUFAF4; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex assembly factor 4
D 103214452 NDUFAF5; NADH:ubiquinone oxidoreductase complex assembly factor 5 K18162 NDUFAF5; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex assembly factor 5 [EC:2.1.1.-]
D 103237154 NDUFAF6; NADH:ubiquinone oxidoreductase complex assembly factor 6 K18163 NDUFAF6; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex assembly factor 6
D 103220479 NDUFAF7; NADH:ubiquinone oxidoreductase complex assembly factor 7 K18164 NDUFAF7; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex assembly factor 7
D 103243713 NDUFAF8; NADH:ubiquinone oxidoreductase complex assembly factor 8 K24726 NDUFAF8; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex assembly factor 8
D 103248136 TMEM126B; transmembrane protein 126B K18165 TMEM126B; complex I assembly factor TMEM126B
D 103233934 ECSIT; ECSIT signalling integrator K04405 ECSIT; evolutionarily conserved signaling intermediate in Toll pathway
D 103228084 acyl-CoA dehydrogenase family member 9, mitochondrial K15980 ACAD9; acyl-CoA dehydrogenase family member 9 [EC:1.3.99.-]
D 103228792 NUBPL; nucleotide binding protein like K03593 mrp; ATP-binding protein involved in chromosome partitioning
D 103248788 FOXRED1; FAD dependent oxidoreductase domain containing 1 K18166 FOXRED1; FAD-dependent oxidoreductase domain-containing protein 1
D 103240644 RTN4IP1; reticulon 4 interacting protein 1 K23164 RTN4IP1; reticulon-4-interacting protein 1, mitochondrial
D 103228229 TIMMDC1; translocase of inner mitochondrial membrane domain containing 1 K23505 TIMMDC1; complex I assembly factor TIMMDC1
C Complex-II assembly factors
D 103234550 SDHAF1; succinate dehydrogenase complex assembly factor 1 K18167 SDHAF1; succinate dehydrogenase assembly factor 1
D 103234439 SDHAF2; succinate dehydrogenase complex assembly factor 2 K18168 SDHAF2; succinate dehydrogenase assembly factor 2
C Complex-III assembly factors
D 103217874 BCS1L; BCS1 homolog, ubiquinol-cytochrome c reductase complex chaperone K08900 BCS1; mitochondrial chaperone BCS1
D 103242455 TTC19; tetratricopeptide repeat domain 19 K18169 TTC19; tetratricopeptide repeat protein 19, mitochondrial
D 103244489 LYRM7; LYR motif containing 7 K18170 LYRM7; complex III assembly factor LYRM7
D 103216057 UQCC1; ubiquinol-cytochrome c reductase complex assembly factor 1 K17662 CBP3; cytochrome b pre-mRNA-processing protein 3
D 103221650 UQCC2; ubiquinol-cytochrome c reductase complex assembly factor 2 K17682 MNF1; mitochondrial nucleoid factor 1
D 103233987 C1H11orf83; chromosome 1 open reading frame, human C11orf83 K23165 UQCC3; ubiquinol-cytochrome-c reductase complex assembly factor 3
D 103226080 C5H16orf91; chromosome 5 open reading frame, human C16orf91 K27339 UQCC4; ubiquinol-cytochrome c reductase complex assembly factor 4
D 103227738 SMIM4; small integral membrane protein 4 K27340 UQCC5; ubiquinol-cytochrome c reductase complex assembly factor 5
D 103238980 C11H12orf73; chromosome 11 open reading frame, human C12orf73 K27341 UQCC6; ubiquinol-cytochrome c reductase complex assembly factor 6
D 103226956 C21H7orf73; chromosome 21 open reading frame, human C7orf73 K27319 STMP1; short transmembrane mitochondrial protein 1
D 103242248 uncharacterized protein C7orf73-like K27319 STMP1; short transmembrane mitochondrial protein 1
C Complex-IV assembly factors
D 103241793 CMC1; C-X9-C motif containing 1 K18171 CMC1; COX assembly mitochondrial protein 1
D 103233362 CMC2; C-X9-C motif containing 2 K18172 CMC2; COX assembly mitochondrial protein 2
D 103226155 COA1; cytochrome c oxidase assembly factor 1 homolog K18173 COA1; cytochrome c oxidase assembly factor 1
D 103231354 cytochrome c oxidase assembly factor 3 homolog, mitochondrial K18175 CCDC56; cytochrome c oxidase assembly factor 3, animal type
D 103243804 COA3; cytochrome c oxidase assembly factor 3 K18175 CCDC56; cytochrome c oxidase assembly factor 3, animal type
D 103248021 COA4; cytochrome c oxidase assembly factor 4 homolog, mitochondrial K18177 COA4; cytochrome c oxidase assembly factor 4
D 103241099 COA5; cytochrome c oxidase assembly factor 5 K18178 COA5; cytochrome c oxidase assembly factor 5
D 103230782 COA6; cytochrome c oxidase assembly factor 6 K18179 COA6; cytochrome c oxidase assembly factor 6
D 103224805 COA7; cytochrome c oxidase assembly factor 7 K18180 COA7; cytochrome c oxidase assembly factor 7
D 103242416 COX10; COX10, heme A:farnesyltransferase cytochrome c oxidase assembly factor K02257 COX10; heme o synthase [EC:2.5.1.141]
D 103242898 COX11; COX11, cytochrome c oxidase copper chaperone K02258 COX11; cytochrome c oxidase assembly protein subunit 11
D 103238297 cytochrome c oxidase assembly protein COX14 K18181 COX14; cytochrome c oxidase assembly factor 14
D 103216376 COX15; COX15, cytochrome c oxidase assembly homolog K02259 COX15; heme a synthase [EC:1.17.99.9]
D 103229262 COX16; COX16, cytochrome c oxidase assembly homolog K18182 COX16; cytochrome c oxidase assembly protein subunit 16
D 103228222 COX17; COX17, cytochrome c oxidase copper chaperone K02260 COX17; cytochrome c oxidase assembly protein subunit 17
D 103235773 mitochondrial inner membrane protein COX18 K17797 COX18; mitochondrial inner membrane protein COX18
D 103246898 COX19; COX19, cytochrome c oxidase assembly factor K18183 COX19; cytochrome c oxidase assembly protein subunit 19
D 103218044 cytochrome c oxidase protein 20 homolog K18184 COX20; cytochrome c oxidase assembly protein subunit 20
D 103230894 COX20; COX20, cytochrome c oxidase assembly factor K18184 COX20; cytochrome c oxidase assembly protein subunit 20
D 103216002 MSS51; MSS51 mitochondrial translational activator K17656 MSS51; mitochondrial splicing suppressor protein 51
D 103233807 PET100; PET100 homolog K18186 PET100; protein PET100, animal type
D 103214828 PET117; PET117 homolog K18188 PET117; protein PET117
D 103226597 protein PET117 homolog, mitochondrial-like K18188 PET117; protein PET117
D 103231443 OXA1L; OXA1L, mitochondrial inner membrane protein K03217 yidC; YidC/Oxa1 family membrane protein insertase
D 103239687 SURF1; surfeit 1 K14998 SURF1; surfeit locus 1 family protein
D 103242389 SCO1; SCO1, cytochrome c oxidase assembly protein K07152 SCO1; protein SCO1
D 103223567 SCO2; SCO2, cytochrome c oxidase assembly protein K23755 SCO2; protein SCO2
D 103243227 TACO1; translational activator of cytochrome c oxidase 1 K18189 TACO1; translational activator of cytochrome c oxidase 1
D 103220377 LRPPRC; leucine rich pentatricopeptide repeat containing K17964 LRPPRC; leucine-rich PPR motif-containing protein, mitochondrial
D 103229762 APOPT1; apoptogenic 1, mitochondrial K23506 APOPT1; apoptogenic protein 1
D 103226956 C21H7orf73; chromosome 21 open reading frame, human C7orf73 K27319 STMP1; short transmembrane mitochondrial protein 1
D 103242248 uncharacterized protein C7orf73-like K27319 STMP1; short transmembrane mitochondrial protein 1
D 103227738 SMIM4; small integral membrane protein 4 K27340 UQCC5; ubiquinol-cytochrome c reductase complex assembly factor 5
D 103233859 TMEM223; transmembrane protein 223 K27342 TMEM223; transmembrane protein 223
D 103227469 HIGD1A; HIG1 hypoxia inducible domain family member 1A K28216 HIGD1A_C; HIG1 domain family member 1A/C, mitochondrial
D 103238311 HIGD1C; HIG1 hypoxia inducible domain family member 1C K28216 HIGD1A_C; HIG1 domain family member 1A/C, mitochondrial
D 103245026 HIGD2A; HIG1 hypoxia inducible domain family member 2A K28217 HIGD2A; HIG1 domain family member 2A, mitochondrial
C Complex-V assembly factors
D 103224880 ATPAF1; ATP synthase mitochondrial F1 complex assembly factor 1 K07555 ATPeAF1; ATP synthase mitochondrial F1 complex assembly factor 1
D 103242483 ATPAF2; ATP synthase mitochondrial F1 complex assembly factor 2 K07556 ATPeAF2; ATP synthase mitochondrial F1 complex assembly factor 2
D 103236979 TMEM70; transmembrane protein 70 K17966 TMEM70; transmembrane protein 70, mitochondrial
D 103248233 6.8 kDa mitochondrial proteolipid-like K18193 MP68; 6.8 kDa mitochondrial proteolipid
D 103216454 USMG5; up-regulated during skeletal muscle growth 5 homolog (mouse) K18194 USMG5; up-regulated during skeletal muscle growth protein 5
B Mitochondrial dynamics
C Fission and Fusion factors
D 103221210 MFN1; mitofusin 1 K21356 MFN1; mitofusin 1 [EC:3.6.5.-]
D 103225593 MFN2; mitofusin 2 K06030 MFN2; mitofusin 2 [EC:3.6.5.-]
D 103241927 OPA1; OPA1, mitochondrial dynamin like GTPase K17079 OPA1; optic atrophy protein 1 [EC:3.6.5.5]
D 103234867 OPA3; OPA3, outer mitochondrial membrane lipid metabolism regulator K23166 OPA3; optic atrophy 3 protein
D 103239252 TRIAP1; TP53 regulated inhibitor of apoptosis 1 K17968 TRIAP1; TRIAP1/MDM35 family protein
D 103221155 PARL; presenilin associated rhomboid like K09650 PARL; rhomboid-like protein [EC:3.4.21.105]
D 103221651 BAK1; BCL2 antagonist/killer 1 K14021 BAK; Bcl-2 homologous antagonist/killer
D 103235004 BAX; BCL2 associated X, apoptosis regulator K02159 BAX; apoptosis regulator BAX
D 103218837 DNM1L; dynamin 1 like K17065 DNM1L; dynamin 1-like protein [EC:3.6.5.5]
D 103246687 FIS1; fission, mitochondrial 1 K17969 FIS1; mitochondrial fission 1 protein
D 103217664 SUMO1; small ubiquitin-like modifier 1 K12160 SUMO; small ubiquitin-related modifier
D 103219991 SUMO3; small ubiquitin-like modifier 3 K12160 SUMO; small ubiquitin-related modifier
D 103243054 SUMO2; small ubiquitin-like modifier 2 K12160 SUMO; small ubiquitin-related modifier
D 103224496 SH3GLB1; SH3 domain containing GRB2 like endophilin B1 K11248 SH3GLB1; endophilin-B1
D 103221576 MTCH1; mitochondrial carrier 1 K17885 MTCH; mitochondrial carrier
D 103235909 MTCH2; mitochondrial carrier 2 K17885 MTCH; mitochondrial carrier
D 103217987 MFF; mitochondrial fission factor K22076 MFF; mitochondrial fission factor
D 103236985 GDAP1; ganglioside induced differentiation associated protein 1 K22077 GDAP1; ganglioside-induced differentiation-associated protein 1
D 103245677 GDAP1L1; ganglioside induced differentiation associated protein 1 like 1 K22077 GDAP1; ganglioside-induced differentiation-associated protein 1
D 103243371 VAT1; vesicle amine transport 1 K23167 VAT1; synaptic vesicle membrane protein VAT-1
D 103223344 MIEF1; mitochondrial elongation factor 1 K23507 MIEF1_2; mitochondrial dynamics protein MID51/49
D 103242489 MIEF2; mitochondrial elongation factor 2 K23507 MIEF1_2; mitochondrial dynamics protein MID51/49
D 103242466 PLD6; phospholipase D family member 6 K16862 PLD6; mitochondrial cardiolipin hydrolase [EC:3.1.4.-]
D 103224578 MIGA1; mitoguardin 1 K27289 MIGA; mitoguardin
D 103239932 MIGA2; mitoguardin 2 K27289 MIGA; mitoguardin
C Actin cytoskeleton organization
D 103215020 actin, cytoplasmic 2-like K05692 ACTB_G1; actin beta/gamma 1
D 103217237 actin, cytoplasmic 2-like K05692 ACTB_G1; actin beta/gamma 1
D 103219323 actin, cytoplasmic 1 K05692 ACTB_G1; actin beta/gamma 1
D 103230823 actin-like K05692 ACTB_G1; actin beta/gamma 1
D 103240948 actin, cytoplasmic 2-like K05692 ACTB_G1; actin beta/gamma 1
D 103243719 ACTG1; actin gamma 1 K05692 ACTB_G1; actin beta/gamma 1
D 103246953 ACTB; actin beta K05692 ACTB_G1; actin beta/gamma 1
D 103215121 NAA20; N(alpha)-acetyltransferase 20, NatB catalytic subunit K17972 NAA20; N-terminal acetyltransferase B complex catalytic subunit [EC:2.3.1.254]
D 103239144 NAA25; N(alpha)-acetyltransferase 25, NatB auxiliary subunit K17973 NAA25; N-terminal acetyltransferase B complex non-catalytic subunit
C Mitochondrial movement
D 103228219 prohibitin-like K17080 PHB1; prohibitin 1
D 103232438 prohibitin-like K17080 PHB1; prohibitin 1
D 103243640 PHB; prohibitin K17080 PHB1; prohibitin 1
D 103218493 PHB2; prohibitin 2 K17081 PHB2; prohibitin 2
D 103229038 KTN1; kinectin 1 K17975 KTN1; kinectin
C Retention of mitochondria
D 103218837 DNM1L; dynamin 1 like K17065 DNM1L; dynamin 1-like protein [EC:3.6.5.5]
D 103222428 MYO5B; myosin VB K10357 MYO5; myosin V
D 103245607 MYO5A; myosin VA K10357 MYO5; myosin V
D 103245609 MYO5C; myosin VC K10357 MYO5; myosin V
D 103247375 XIAP; X-linked inhibitor of apoptosis K04725 XIAP; E3 ubiquitin-protein ligase XIAP [EC:2.3.2.27]
D 103239302 DIABLO; diablo IAP-binding mitochondrial protein K10522 DIABLO; diablo
C Morphogenesis factors
D 103236626 SNX25; sorting nexin 25 K17887 SNX25; sorting nexin-25
D 103243817 RHOT1; ras homolog family member T1 K07870 RHOT1; mitochondrial Rho GTPase 1 [EC:3.6.5.-]
D 103220987 IMMT; inner membrane mitochondrial protein K17785 IMMT; MICOS complex subunit MIC60
D 103223166 MTFP1; mitochondrial fission process 1 K17981 MTFP1; mitochondrial fission process protein 1
D 103223877 DAP3; death associated protein 3 K17408 DAP3; small subunit ribosomal protein S29
D 103245581 NEDD4; neural precursor cell expressed, developmentally down-regulated 4, E3 ubiquitin protein ligase K10591 NEDD4; E3 ubiquitin-protein ligase NEDD4 [EC:2.3.2.26]
D 103237275 OXR1; oxidation resistance 1 K25437 OXR1; oxidation resistance protein 1
D 103225840 protein misato homolog 1-like K27320 MSTO1; protein misato
D 103218368 PRELI domain-containing protein 1, mitochondrial K27964 PRELID1; PRELI domain-containing protein 1, mitochondrial
D 103245047 PRELID1; PRELI domain containing 1 K27964 PRELID1; PRELI domain-containing protein 1, mitochondrial
D 103244730 PRELID2; PRELI domain containing 2 K27965 PRELID2; PRELI domain-containing protein 2 mitochondrial
D 103243417 PRELID3B; PRELI domain containing 3B K27966 PRELID3; PRELI domain-containing protein 3, mitochondrial
B Mitophagy factors
C Autophagy-related proteins
D 103229646 ATG2B; autophagy related 2B K17906 ATG2; autophagy-related protein 2
D 103232842 ATG2A; autophagy related 2A K17906 ATG2; autophagy-related protein 2
D 103228318 ATG3; autophagy related 3 K08343 ATG3; ubiquitin-like-conjugating enzyme ATG3
D 103218245 ATG4B; autophagy related 4B cysteine peptidase K08342 ATG4; cysteine protease ATG4 [EC:3.4.22.-]
D 103224699 ATG4C; autophagy related 4C cysteine peptidase K08342 ATG4; cysteine protease ATG4 [EC:3.4.22.-]
D 103232466 ATG4A; autophagy related 4A cysteine peptidase K08342 ATG4; cysteine protease ATG4 [EC:3.4.22.-]
D 103233906 ATG4D; autophagy related 4D cysteine peptidase K08342 ATG4; cysteine protease ATG4 [EC:3.4.22.-]
D 103240649 ATG5; autophagy related 5 K08339 ATG5; autophagy-related protein 5
D 103230857 beclin-2 K08334 BECN; beclin
D 103243387 BECN1; beclin 1 K08334 BECN; beclin
D 103228012 ATG7; autophagy related 7 K08337 ATG7; ubiquitin-like modifier-activating enzyme ATG7
D 103218578 GABARAPL1; GABA type A receptor associated protein like 1 K08341 GABARAP; GABA(A) receptor-associated protein
D 103231198 gamma-aminobutyric acid receptor-associated protein-like 3 K08341 GABARAP; GABA(A) receptor-associated protein
D 103233317 GABARAPL2; GABA type A receptor associated protein like 2 K08341 GABARAP; GABA(A) receptor-associated protein
D 103242281 GABARAP; GABA type A receptor-associated protein K08341 GABARAP; GABA(A) receptor-associated protein
D 103217902 ATG9A; autophagy related 9A K17907 ATG9; autophagy-related protein 9
D 103227235 ATG9B; autophagy related 9B K17907 ATG9; autophagy-related protein 9
D 103224003 ATG10; autophagy related 10 K17888 ATG10L; ubiquitin-like-conjugating enzyme ATG10
D 103244369 ATG12; autophagy related 12 K08336 ATG12; ubiquitin-like protein ATG12
D 103229035 ATG14; autophagy related 14 K17889 ATG14L; beclin 1-associated autophagy-related key regulator
D 103218104 ATG16L1; autophagy related 16 like 1 K17890 ATG16L1; autophagy-related protein 16-1
D 103248007 ATG16L2; autophagy related 16 like 2 K20868 ATG16L2; autophagy-related protein 16-2
D 103243149 WIPI1; WD repeat domain, phosphoinositide interacting 1 K17908 WIPI1_2; autophagy-related protein 18
D 103246841 WIPI2; WD repeat domain, phosphoinositide interacting 2 K17908 WIPI1_2; autophagy-related protein 18
C Parkin-dependent mechanism factors
D 103240862 PARK2; parkin RBR E3 ubiquitin protein ligase K04556 PARK2; parkin [EC:2.3.2.31]
D 103238512 E3 ubiquitin-protein ligase NRDP1-like K11981 RNF41; E3 ubiquitin-protein ligase NRDP1 [EC:2.3.2.27]
D 103238513 E3 ubiquitin-protein ligase NRDP1 K11981 RNF41; E3 ubiquitin-protein ligase NRDP1 [EC:2.3.2.27]
D 103228238 CLEC16A; C-type lectin domain family 16 member A K19513 CLEC16A; protein CLEC16A
D 103225450 PINK1; PTEN induced putative kinase 1 K05688 PINK1; PTEN induced putative kinase 1 [EC:2.7.11.1]
D 103221155 PARL; presenilin associated rhomboid like K09650 PARL; rhomboid-like protein [EC:3.4.21.105]
D 103245112 SQSTM1; sequestosome 1 K14381 SQSTM1; sequestosome 1
D 103226301 TAX1BP1; Tax1 binding protein 1 K21347 TAX1BP1; Tax1-binding protein 1
D 103243627 CALCOCO2; calcium binding and coiled-coil domain 2 K21348 CALCOCO2; calcium-binding and coiled-coil domain-containing protein 2
D 103236448 AMBRA1; autophagy and beclin 1 regulator 1 K17985 AMBRA1; activating molecule in BECN1-regulated autophagy protein 1
C Parkin-independent mechanism factors
D 103236386 ATG13; autophagy related 13 K08331 ATG13; autophagy-related protein 13
D 103239428 ULK1; unc-51 like autophagy activating kinase 1 K21357 ULK1; serine/threonine-protein kinase ULK1 [EC:2.7.11.1]
D 103242520 ULK2; unc-51 like autophagy activating kinase 2 K08269 ULK2; serine/threonine-protein kinase ULK2 [EC:2.7.11.1]
D 103236790 RB1CC1; RB1 inducible coiled-coil 1 K17589 RB1CC1; RB1-inducible coiled-coil protein 1
D 103221436 HSP90AB1; heat shock protein 90 alpha family class B member 1 K04079 HSP90A; molecular chaperone HtpG
D 103229726 HSP90AA1; heat shock protein 90 alpha family class A member 1 K04079 HSP90A; molecular chaperone HtpG
D 103236665 heat shock protein HSP 90-alpha-like K04079 HSP90A; molecular chaperone HtpG
D 103233899 CDC37; cell division cycle 37 K09554 CDC37; cell division cycle protein 37
D 103243842 SMURF2; SMAD specific E3 ubiquitin protein ligase 2 K04678 SMURF; E3 ubiquitin ligase SMURF1/2 [EC:2.3.2.26]
D 103246792 SMURF1; SMAD specific E3 ubiquitin protein ligase 1 K04678 SMURF; E3 ubiquitin ligase SMURF1/2 [EC:2.3.2.26]
D 103215511 BNIP3L; BCL2 interacting protein 3 like K15465 BNIP3L; BCL2/adenovirus E1B 19 kDa protein-interacting protein 3-like
D 103231849 FUNDC1; FUN14 domain containing 1 K17986 FUNDC1; FUN14 domain-containing protein 1
C Other mitophagy factors
D 103215177 PRKAA1; protein kinase AMP-activated catalytic subunit alpha 1 K07198 PRKAA; 5'-AMP-activated protein kinase, catalytic alpha subunit [EC:2.7.11.31]
D 103224740 PRKAA2; protein kinase AMP-activated catalytic subunit alpha 2 K07198 PRKAA; 5'-AMP-activated protein kinase, catalytic alpha subunit [EC:2.7.11.31]
D 103219881 microtubule-associated proteins 1A/1B light chain 3B K10435 MAP1LC; microtubule-associated protein 1 light chain
D 103230861 MAP1LC3C; microtubule associated protein 1 light chain 3 gamma K10435 MAP1LC; microtubule-associated protein 1 light chain
D 103233426 MAP1LC3B; microtubule associated protein 1 light chain 3 beta K10435 MAP1LC; microtubule-associated protein 1 light chain
D 103248775 MAP1LC3A; microtubule associated protein 1 light chain 3 alpha K10435 MAP1LC; microtubule-associated protein 1 light chain
D 103243366 NBR1; NBR1, autophagy cargo receptor K17987 NBR1; next to BRCA1 gene 1 protein
D 103246800 TECPR1; tectonin beta-propeller repeat containing 1 K17988 TECPR1; tectonin beta-propeller repeat-containing protein 1
D 103229301 ZFYVE1; zinc finger FYVE-type containing 1 K17603 ZFYVE1; zinc finger FYVE domain-containing protein 1
D 103243403 COASY; Coenzyme A synthase K02318 COASY; phosphopantetheine adenylyltransferase / dephospho-CoA kinase [EC:2.7.7.3 2.7.1.24]
D 103223308 PLA2G6; phospholipase A2 group VI K16343 PLA2G6; calcium-independent phospholipase A2 [EC:3.1.1.4]
D 103234424 C6H19orf12; chromosome 6 open reading frame, human C19orf12 K23168 MPAN; protein MPAN
D 103232847 FUNDC2; FUN14 domain containing 2 K24308 FUNDC2; FUN14 domain-containing protein 2
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#[ BRITE | KEGG2 | KEGG ]
#Last updated: September 5, 2025