+D
#
Mitochondrial Biogenesis - Microcebus murinus (gray mouse lemur)
%
!
AMitochondrial DNA transcription, translation, and replication factors
B Mitochondrial DNA-encoded proteins
C Mitochondrial respiratory chain complex I
D 26823996 ND1; NADH dehydrogenase subunit 1 K03878 ND1; NADH-ubiquinone oxidoreductase chain 1 [EC:7.1.1.2]
D 26823994 ND2; NADH dehydrogenase subunit 2 K03879 ND2; NADH-ubiquinone oxidoreductase chain 2 [EC:7.1.1.2]
D 26823995 ND3; NADH dehydrogenase subunit 3 K03880 ND3; NADH-ubiquinone oxidoreductase chain 3 [EC:7.1.1.2]
D 26823983 ND4; NADH dehydrogenase subunit 4 K03881 ND4; NADH-ubiquinone oxidoreductase chain 4 [EC:7.1.1.2]
D 26823982 ND4L; NADH dehydrogenase subunit 4L K03882 ND4L; NADH-ubiquinone oxidoreductase chain 4L [EC:7.1.1.2]
D 26823987 ND5; NADH dehydrogenase subunit 5 K03883 ND5; NADH-ubiquinone oxidoreductase chain 5 [EC:7.1.1.2]
D 26823993 ND6; NADH dehydrogenase subunit 6 K03884 ND6; NADH-ubiquinone oxidoreductase chain 6 [EC:7.1.1.2]
C Mitochondrial respiratory chain complex III
D 26823990 CYTB; cytochrome b K00412 CYTB; ubiquinol-cytochrome c reductase cytochrome b subunit
C Cytochrome c oxidase
D 26823973 COX1; cytochrome c oxidase subunit I K02256 COX1; cytochrome c oxidase subunit 1 [EC:7.1.1.9]
D 26823976 COX2; cytochrome c oxidase subunit II K02261 COX2; cytochrome c oxidase subunit 2
D 26823980 COX3; cytochrome c oxidase subunit III K02262 COX3; cytochrome c oxidase subunit 3
C F-type ATPase
D 26823978 ATP8; ATP synthase F0 subunit 8 K02125 ATPeF08; F-type H+-transporting ATPase subunit 8
D 26823979 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 105860835 POLRMT; DNA-directed RNA polymerase, mitochondrial K10908 POLRMT; DNA-directed RNA polymerase, mitochondrial [EC:2.7.7.6]
C Mitochondrial transcription factors
D 105870281 TFAM; transcription factor A, mitochondrial K11830 TFAM; transcription factor A, mitochondrial
D 105868584 TFB1M; dimethyladenosine transferase 1, mitochondrial isoform X1 K15266 TFB1M; dimethyladenosine transferase 1, mitochondrial [EC:2.1.1.-]
D 105863808 TFB2M; dimethyladenosine transferase 2, mitochondrial isoform X1 K17653 TFB2M; dimethyladenosine transferase 2, mitochondrial [EC:2.1.1.-]
C Mitochondrial translation factors
D 105878425 MTIF2; translation initiation factor IF-2, mitochondrial isoform X1 K02519 infB; translation initiation factor IF-2
D 105859843 MTIF3; translation initiation factor IF-3, mitochondrial K02520 infC; translation initiation factor IF-3
D 105878002 TSFM; elongation factor Ts, mitochondrial K02357 tsf; elongation factor Ts
D 105868287 TUFM; elongation factor Tu, mitochondrial K02358 tuf; elongation factor Tu
D 105857415 GFM1; elongation factor G, mitochondrial K02355 fusA; elongation factor G
D 105881805 GFM2; ribosome-releasing factor 2, mitochondrial isoform X1 K02355 fusA; elongation factor G
D 105885222 ribosome-releasing factor 2, mitochondrial-like K02355 fusA; elongation factor G
D 105866407 MTERF4; transcription termination factor 4, mitochondrial isoform X1 K15031 MTERF; mitochondrial transcription termination factor family protein
D 105875638 MTERF2; LOW QUALITY PROTEIN: transcription termination factor 2, mitochondrial K15031 MTERF; mitochondrial transcription termination factor family protein
D 105875649 transcription termination factor 3, mitochondrial isoform X1 K15031 MTERF; mitochondrial transcription termination factor family protein
D 105877845 transcription termination factor 3, mitochondrial isoform X1 K15031 MTERF; mitochondrial transcription termination factor family protein
D 105879395 MTERF1; transcription termination factor 1, mitochondrial isoform X1 K15031 MTERF; mitochondrial transcription termination factor family protein
D 105859334 GUF1; translation factor GUF1, mitochondrial isoform X1 K21594 GUF1; translation factor GUF1, mitochondrial [EC:3.6.5.-]
C RNase MRP
D 105869876 POP1; ribonucleases P/MRP protein subunit POP1 isoform X1 K01164 POP1; ribonuclease P/MRP protein subunit POP1 [EC:3.1.26.5]
D 105877009 POP4; ribonuclease P protein subunit p29 K03538 POP4; ribonuclease P protein subunit POP4 [EC:3.1.26.5]
D 105866897 POP5; ribonuclease P/MRP protein subunit POP5 K03537 POP5; ribonuclease P/MRP protein subunit POP5 [EC:3.1.26.5]
D 105871930 RPP30; ribonuclease P protein subunit p30 isoform X1 K03539 RPP1; ribonuclease P/MRP protein subunit RPP1 [EC:3.1.26.5]
D 105868499 POP7; ribonuclease P protein subunit p20 K14527 RPP20; ribonuclease P/MRP protein subunit RPP20 [EC:3.1.26.5]
D 105854854 RPP25; ribonuclease P protein subunit p25 K14525 RPP25; ribonucleases P/MRP protein subunit RPP25 [EC:3.1.26.5]
D 105874346 RPP25L; ribonuclease P protein subunit p25-like protein K14525 RPP25; ribonucleases P/MRP protein subunit RPP25 [EC:3.1.26.5]
D 105856346 RPP38; ribonuclease P protein subunit p38 K14523 RPP38; ribonucleases P/MRP protein subunit RPP38 [EC:3.1.26.5]
D 105873459 RPP40; ribonuclease P protein subunit p40 isoform X1 K14530 RPP40; ribonucleases P/MRP protein subunit RPP40 [EC:3.1.26.5]
C Mitochondrial ribonuclease P
D 105879443 TRMT10C; mitochondrial ribonuclease P protein 1 K17654 TRMT10C; mitochondrial ribonuclease P protein 1 [EC:2.1.1.-]
D 105861240 3-hydroxyacyl-CoA dehydrogenase type-2 K08683 HSD17B10; 3-hydroxyacyl-CoA dehydrogenase / 3-hydroxy-2-methylbutyryl-CoA dehydrogenase [EC:1.1.1.35 1.1.1.178]
D 105855490 KIAA0391; mitochondrial ribonuclease P protein 3 isoform X1 K17655 MRPP3; mitochondrial ribonuclease P protein 3
C Other mitochondrial DNA transcription and translation factors
D 105868800 MSS51; putative protein MSS51 homolog, mitochondrial K17656 MSS51; mitochondrial splicing suppressor protein 51
D 105856465 TEFM; transcription elongation factor, mitochondrial K17658 TEFM; transcription elongation factor, mitochondrial
D 105868319 pentatricopeptide repeat-containing protein 1, mitochondrial isoform X1 K17710 PTCD1; pentatricopeptide repeat domain-containing protein 1
D 105881573 PTCD2; pentatricopeptide repeat-containing protein 2, mitochondrial isoform X1 K17711 PTCD2; pentatricopeptide repeat domain-containing protein 2
D 105876611 PTCD3; pentatricopeptide repeat domain-containing protein 3, mitochondrial K17659 PTCD3; pentatricopeptide repeat domain-containing protein 3
D 105874912 DHX30; putative ATP-dependent RNA helicase DHX30 isoform X1 K13185 DHX30; ATP-dependent RNA helicase DHX30 [EC:5.6.2.6]
D 105865869 ubiquinol-cytochrome-c reductase complex assembly factor 1 isoform X1 K17662 CBP3; cytochrome b pre-mRNA-processing protein 3
D 105864927 LACE1; lactation elevated protein 1 isoform X5 K18798 AFG1; peroxisome-assembly ATPase [EC:3.6.4.7]
D 105878008 ATP23; mitochondrial inner membrane protease ATP23 homolog isoform X1 K18156 ATP23; mitochondrial inner membrane protease ATP23 [EC:3.4.24.-]
D 105870214 SUPV3L1; ATP-dependent RNA helicase SUPV3L1, mitochondrial K17675 SUPV3L1; ATP-dependent RNA helicase SUPV3L1/SUV3 [EC:5.6.2.6]
D 105874692 LACTB2; endoribonuclease LACTB2 K16639 LACTB2; endoribonuclease LACTB2 [EC:3.1.27.-]
D 105857293 SLC25A5; LOW QUALITY PROTEIN: ADP/ATP translocase 2 K05863 SLC25A4S; solute carrier family 25 (mitochondrial adenine nucleotide translocator), member 4/5/6/31
D 105860211 ADP/ATP translocase 2 K05863 SLC25A4S; solute carrier family 25 (mitochondrial adenine nucleotide translocator), member 4/5/6/31
D 105870811 SLC25A6; ADP/ATP translocase 3 K05863 SLC25A4S; solute carrier family 25 (mitochondrial adenine nucleotide translocator), member 4/5/6/31
D 105882495 SLC25A4; ADP/ATP translocase 1 K05863 SLC25A4S; solute carrier family 25 (mitochondrial adenine nucleotide translocator), member 4/5/6/31
D 105885820 SLC25A31; ADP/ATP translocase 4 K05863 SLC25A4S; solute carrier family 25 (mitochondrial adenine nucleotide translocator), member 4/5/6/31
D 105857171 SLC25A37; mitoferrin-1 isoform X1 K15113 SLC25A28_37; solute carrier family 25 (mitochondrial iron transporter), member 28/37
D 105873364 SLC25A28; mitoferrin-2 isoform X1 K15113 SLC25A28_37; solute carrier family 25 (mitochondrial iron transporter), member 28/37
D 105875892 MALSU1; mitochondrial assembly of ribosomal large subunit protein 1 K25803 MALSU1; mitochondrial assembly of ribosomal large subunit protein 1
D 105857895 ERAL1; GTPase Era, mitochondrial K03595 era; GTPase
D 105871797 YARS; tyrosine--tRNA ligase, cytoplasmic K01866 YARS; tyrosyl-tRNA synthetase [EC:6.1.1.1]
D 105884880 YARS2; tyrosine--tRNA ligase, mitochondrial K01866 YARS; tyrosyl-tRNA synthetase [EC:6.1.1.1]
D 105874458 LARS2; probable leucine--tRNA ligase, mitochondrial isoform X1 K01869 LARS; leucyl-tRNA synthetase [EC:6.1.1.4]
D 105876487 LARS; leucine--tRNA ligase, cytoplasmic K01869 LARS; leucyl-tRNA synthetase [EC:6.1.1.4]
D 105871698 DARS2; aspartate--tRNA ligase, mitochondrial K01876 DARS2; aspartyl-tRNA synthetase [EC:6.1.1.12]
D 105855108 GARS; glycine--tRNA ligase K01880 GARS; glycyl-tRNA synthetase [EC:6.1.1.14]
D 105863598 RARS2; probable arginine--tRNA ligase, mitochondrial isoform X1 K01887 RARS; arginyl-tRNA synthetase [EC:6.1.1.19]
D 105880155 RARS; arginine--tRNA ligase, cytoplasmic K01887 RARS; arginyl-tRNA synthetase [EC:6.1.1.19]
D 105862144 LOW QUALITY PROTEIN: glutamyl-tRNA(Gln) amidotransferase subunit A, mitochondrial-like K02433 gatA; aspartyl-tRNA(Asn)/glutamyl-tRNA(Gln) amidotransferase subunit A [EC:6.3.5.6 6.3.5.7]
D 105864890 QRSL1; glutamyl-tRNA(Gln) amidotransferase subunit A, mitochondrial isoform X1 K02433 gatA; aspartyl-tRNA(Asn)/glutamyl-tRNA(Gln) amidotransferase subunit A [EC:6.3.5.6 6.3.5.7]
D 105881813 GATB; glutamyl-tRNA(Gln) amidotransferase subunit B, mitochondrial K02434 gatB; aspartyl-tRNA(Asn)/glutamyl-tRNA(Gln) amidotransferase subunit B [EC:6.3.5.6 6.3.5.7]
D 105866931 GATC; glutamyl-tRNA(Gln) amidotransferase subunit C, mitochondrial isoform X1 K02435 gatC; aspartyl-tRNA(Asn)/glutamyl-tRNA(Gln) amidotransferase subunit C [EC:6.3.5.6 6.3.5.7]
D 105884787 MTPAP; poly(A) RNA polymerase, mitochondrial isoform X1 K18060 MTPAP; poly(A) RNA polymerase, mitochondrial [EC:2.7.7.19]
D 105884909 PDE12; 2',5'-phosphodiesterase 12 K19612 PDE12; 2',5'-phosphodiesterase [EC:3.1.13.4 3.1.4.-]
D 105861486 MTG1; mitochondrial ribosome-associated GTPase 1 isoform X1 K19828 MTG1; mitochondrial GTPase 1
D 105869298 NSUN3; tRNA (cytosine(34)-C(5))-methyltransferase, mitochondrial isoform X1 K21969 NSUN3; tRNA (cytosine34-C5)-methyltransferase [EC:2.1.1.-]
D 105877494 NSUN4; 5-methylcytosine rRNA methyltransferase NSUN4 isoform X2 K21970 NSUN4; 5-methylcytosine rRNA methyltransferase NSUN4 [EC:2.1.1.-]
D 105869038 RPUSD3; RNA pseudouridylate synthase domain-containing protein 3 isoform X1 K22537 RPUSD3; mitochondrial RNA pseudouridine synthase RPUSD3 [EC:5.4.99.-]
D 105875360 RPUSD4; RNA pseudouridylate synthase domain-containing protein 4 isoform X1 K22538 RPUSD4; mitochondrial RNA pseudouridine synthase RPUSD4 [EC:5.4.99.-]
D 105858821 RCC1L; RCC1-like G exchanging factor-like protein K23495 RCC1L; RCC1-like G exchanging factor-like protein
D 105870607 NGRN; neugrin K23496 NGRN; neugrin
D 105875250 TRUB2; probable tRNA pseudouridine synthase 2 K23497 TRUB2; mitochondrial mRNA pseudouridine synthase TRUB2 [EC:5.4.99.-]
D 105880691 FASTKD1; FAST kinase domain-containing protein 1, mitochondrial isoform X1 K27754 FASTKD1; FAST kinase domain-containing protein 1
D 105856906 FAST kinase domain-containing protein 2, mitochondrial K18190 FASTKD2; FAST kinase domain-containing protein 2
D 105859935 FASTKD3; FAST kinase domain-containing protein 3, mitochondrial isoform X2 K27755 FASTKD3; FAST kinase domain-containing protein 3
D 105870662 TBRG4; protein TBRG4 isoform X1 K27756 TBRG4; FAST kinase domain-containing protein 4
D 105866036 FASTKD5; FAST kinase domain-containing protein 5, mitochondrial K27757 FASTKD5; FAST kinase domain-containing protein 5
D 105880871 C21H12orf65; probable peptide chain release factor C12orf65 homolog, mitochondrial isoform X1 K23498 COXPD7; peptide chain release factor
D 105858062 RMND1; required for meiotic nuclear division protein 1 homolog K23499 RMND1; required for meiotic nuclear division protein 1
D 105883061 GRSF1; G-rich sequence factor 1 isoform X1 K24984 GRSF1; G-rich sequence factor 1
D 105861803 METTL15; probable methyltransferase-like protein 15 K25876 METTL15; 12S rRNA N4-methylcytidine (m4C) methyltransferase [EC:2.1.1.-]
D 105870763 C7H12orf10; UPF0160 protein MYG1, mitochondrial K25877 MYG1; MYG1 exonuclease [EC:3.1.-.-]
D 105864977 C6H6orf203; uncharacterized protein C6orf203 homolog K26186 MTRES1; mitochondrial transcription rescue factor 1
B Mitochondrial DNA replication factors
C Mitochondrial DNA polymerase
D 105884713 POLG; DNA polymerase subunit gamma-1 K02332 POLG; DNA polymerase gamma 1 [EC:2.7.7.7]
D 105861653 POLG2; DNA polymerase subunit gamma-2, mitochondrial isoform X1 K02333 POLG2; DNA polymerase gamma 2
D 105882499 PRIMPOL; DNA-directed primase/polymerase protein isoform X1 K22761 PRIMPOL; DNA-directed primase/polymerase protein [EC:2.7.7.102 2.7.7.7]
C Mitochondrial DNA maintenance factors
D 105875267 ENDOG; endonuclease G, mitochondrial K01173 ENDOG; endonuclease G, mitochondrial
D 105879724 mitochondrial genome maintenance exonuclease 1-like K19465 MGME1; mitochondrial genome maintenance exonuclease 1 [EC:3.1.-.-]
D 105880386 mitochondrial genome maintenance exonuclease 1-like isoform X1 K19465 MGME1; mitochondrial genome maintenance exonuclease 1 [EC:3.1.-.-]
D 105866813 EXOG; nuclease EXOG, mitochondrial isoform X1 K15050 EXOG; nuclease EXOG, mitochondrial
D 105873807 TWNK; twinkle protein, mitochondrial K17680 PEO1; twinkle protein [EC:5.6.2.3]
D 105868134 PIF1; ATP-dependent DNA helicase PIF1 isoform X1 K15255 PIF1; ATP-dependent DNA helicase PIF1 [EC:5.6.2.3]
C Other Mitochondrial DNA replication factors
D 105873268 SSBP1; single-stranded DNA-binding protein, mitochondrial K03111 ssb; single-strand DNA-binding protein
D 105877654 ATPase family AAA domain-containing protein 3 K17681 ATAD3A_B; ATPase family AAA domain-containing protein 3A/B
D 105861403 ubiquinol-cytochrome-c reductase complex assembly factor 2 K17682 MNF1; mitochondrial nucleoid factor 1
D 105866675 C16H17orf80; uncharacterized protein C17orf80 homolog isoform X1 K27759 MTNAP1; mitochondrial nucleoid-associated protein 1
D 105883120 LOW QUALITY PROTEIN: GTPase IMAP family member 5 K27758 GIMAP1_3_5; GTPase IMAP family member 1/3/5
D 105883166 GIMAP5; GTPase IMAP family member 5 K27758 GIMAP1_3_5; GTPase IMAP family member 1/3/5
#
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 105876213 DNAJA2; dnaJ homolog subfamily A member 2 K09503 DNAJA2; DnaJ homolog subfamily A member 2
D 105879934 TOMM34; mitochondrial import receptor subunit TOM34 isoform X1 K17766 TOM34; mitochondrial import receptor subunit TOM34
D 105855903 RHOT1; mitochondrial Rho GTPase 1 isoform X1 K07870 RHOT1; mitochondrial Rho GTPase 1 [EC:3.6.5.-]
D 105854849 LOW QUALITY PROTEIN: heat shock cognate 71 kDa protein-like K03283 HSPA1_6_8; heat shock 70kDa protein 1/6/8
D 105858167 heat shock 70 kDa protein 1 K03283 HSPA1_6_8; heat shock 70kDa protein 1/6/8
D 105858168 heat shock 70 kDa protein 1-like K03283 HSPA1_6_8; heat shock 70kDa protein 1/6/8
D 105873559 HSPA6; heat shock 70 kDa protein 6 K03283 HSPA1_6_8; heat shock 70kDa protein 1/6/8
D 105885170 HSPA8; heat shock cognate 71 kDa protein K03283 HSPA1_6_8; heat shock 70kDa protein 1/6/8
D 105867505 AIP; AH receptor-interacting protein isoform X1 K17767 AIP; AH receptor-interacting protein
D 105879129 AIPL1; aryl-hydrocarbon-interacting protein-like 1 K17767 AIP; AH receptor-interacting protein
D 105855142 FATE1; fetal and adult testis-expressed transcript protein K28235 FATE1; fetal and adult testis-expressed transcript protein
B Outer membrane
C Transporter outer membrane (TOM) complex
D 105873812 TOMM5; mitochondrial import receptor subunit TOM5 homolog K17773 TOM5; mitochondrial import receptor subunit TOM5
D 105875862 TOMM6; mitochondrial import receptor subunit TOM6 homolog K17772 TOM6; mitochondrial import receptor subunit TOM6
D 109731249 TOMM7; mitochondrial import receptor subunit TOM7 homolog K17771 TOM7; mitochondrial import receptor subunit TOM7
D 105856479 TOMM20; mitochondrial import receptor subunit TOM20 homolog K17770 TOM20; mitochondrial import receptor subunit TOM20
D 105866209 mitochondrial import receptor subunit TOM20 homolog K17770 TOM20; mitochondrial import receptor subunit TOM20
D 105882908 TOMM20L; TOMM20-like protein 1 K17770 TOM20; mitochondrial import receptor subunit TOM20
D 105858543 TOMM22; mitochondrial import receptor subunit TOM22 homolog K17769 TOM22; mitochondrial import receptor subunit TOM22
D 105869508 TOMM40; mitochondrial import receptor subunit TOM40 homolog K11518 TOM40; mitochondrial import receptor subunit TOM40
D 105873552 TOMM40L; mitochondrial import receptor subunit TOM40B isoform X1 K11518 TOM40; mitochondrial import receptor subunit TOM40
D 105859365 TOMM70; mitochondrial import receptor subunit TOM70 isoform X1 K17768 TOM70; mitochondrial import receptor subunit TOM70
C TOB/SAM complex
D 105867646 SAMM50; sorting and assembly machinery component 50 homolog K07277 SAM50; outer membrane protein insertion porin family
C Porin
D 105869973 VDAC1; voltage-dependent anion-selective channel protein 1 K05862 VDAC1; voltage-dependent anion channel protein 1
D 105866328 VDAC2; voltage-dependent anion-selective channel protein 2 isoform X1 K15040 VDAC2; voltage-dependent anion channel protein 2
D 105878143 VDAC3; voltage-dependent anion-selective channel protein 3 isoform X1 K15041 VDAC3; voltage-dependent anion channel protein 3
C ERMES complex
C Other outer membrane factor
D 105865071 MTX2; metaxin-2 isoform X1 K17776 MTX; metaxin
D 105872630 MTX3; metaxin-3 isoform X1 K17776 MTX; metaxin
D 105873176 MTX1; metaxin-1 K17776 MTX; metaxin
D 105878305 MYO19; unconventional myosin-XIX isoform X1 K25838 MYO19; myosin XIX
D 105872970 ARMCX1; armadillo repeat-containing X-linked protein 1 K26187 ARMCX1; armadillo repeat-containing X-linked protein 1
D 105872973 ARMCX2; armadillo repeat-containing X-linked protein 2 K26188 ARMCX2; armadillo repeat-containing X-linked protein 2
D 105872972 ARMCX3; armadillo repeat-containing X-linked protein 3 K26189 ARMCX3; armadillo repeat-containing X-linked protein 3
D 105872971 ARMCX6; protein ARMCX6 K26190 ARMCX6; armadillo repeat-containing X-linked protein 6
D 105854937 MARC1; mitochondrial amidoxime-reducing component 1 K27318 MTARC; mitochondrial amidoxime-reducing component [EC:1.7.-.-]
D 105854938 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 105858869 mitochondrial import inner membrane translocase subunit Tim9 K17777 TIM9; mitochondrial import inner membrane translocase subunit TIM9
D 105864894 mitochondrial import inner membrane translocase subunit Tim9 K17777 TIM9; mitochondrial import inner membrane translocase subunit TIM9
D 105882909 TIMM9; mitochondrial import inner membrane translocase subunit Tim9 K17777 TIM9; mitochondrial import inner membrane translocase subunit TIM9
D 105863170 TIMM10; mitochondrial import inner membrane translocase subunit Tim10 K17778 TIM10; mitochondrial import inner membrane translocase subunit TIM10
D 105883450 TIMM10B; mitochondrial import inner membrane translocase subunit Tim10 B K17779 TIM10B; mitochondrial import inner membrane translocase subunit TIM10B
C TIM8/13 complex
D 105862945 TIMM8B; mitochondrial import inner membrane translocase subunit Tim8 B K17780 TIM8; mitochondrial import inner membrane translocase subunit TIM8
D 105872963 TIMM8A; mitochondrial import inner membrane translocase subunit Tim8 A K17780 TIM8; mitochondrial import inner membrane translocase subunit TIM8
D 105873986 TIMM13; mitochondrial import inner membrane translocase subunit Tim13 K17781 TIM13; mitochondrial import inner membrane translocase subunit TIM13
C Mitochondrial intermembrane space assembly (MIA) complex
D 105861336 mitochondrial intermembrane space import and assembly protein 40 K17782 MIA40; mitochondrial intermembrane space import and assembly protein 40
D 105859616 GFER; FAD-linked sulfhydryl oxidase ALR isoform X1 K17783 ERV1; mitochondrial FAD-linked sulfhydryl oxidase [EC:1.8.3.2]
B Inner mambrane
C MICOS complex
D 105884013 MINOS1; MICOS complex subunit MIC10 K17784 MICOS10; MICOS complex subunit MIC10
D 105876613 IMMT; MICOS complex subunit MIC60 isoform X1 K17785 IMMT; MICOS complex subunit MIC60
D 105869646 C24H19orf70; MICOS complex subunit MIC13 K24624 MICOS13; MICOS complex subunit MIC13
D 105857389 CHCHD3; MICOS complex subunit MIC19 isoform X1 K17563 CHCHD3; MICOS complex subunit MIC19
D 105864099 CHCHD6; MICOS complex subunit MIC25 K17564 CHCHD6; MICOS complex subunit MIC25
D 105876119 MICOS complex subunit MIC26 K24625 APOO; MICOS complex subunit MIC26
D 105882348 APOO; MICOS complex subunit MIC26 K24625 APOO; MICOS complex subunit MIC26
D 105884311 MICOS complex subunit MIC26 K24625 APOO; MICOS complex subunit MIC26
D 105885281 APOOL; MICOS complex subunit MIC27 isoform X1 K24626 APOOL; MICOS complex subunit MIC27
C MICOS complex (yeast)
D 105884013 MINOS1; MICOS complex subunit MIC10 K17784 MICOS10; MICOS complex subunit MIC10
D 105876613 IMMT; MICOS complex subunit MIC60 isoform X1 K17785 IMMT; MICOS complex subunit MIC60
C TIM22 complex
D 105857869 TIMM22; mitochondrial import inner membrane translocase subunit Tim22 K17790 TIM22; mitochondrial import inner membrane translocase subunit TIM22
C TIM23 complex
D 105860120 TIMM23; mitochondrial import inner membrane translocase subunit Tim23 isoform X1 K17794 TIM23; mitochondrial import inner membrane translocase subunit TIM23
D 105856396 TIMM17B; mitochondrial import inner membrane translocase subunit Tim17-B isoform X1 K17795 TIM17; mitochondrial import inner membrane translocase subunit TIM17
D 105860042 TIMM17A; mitochondrial import inner membrane translocase subunit Tim17-A K17795 TIM17; mitochondrial import inner membrane translocase subunit TIM17
D 105880293 TIMM21; mitochondrial import inner membrane translocase subunit Tim21 K17796 TIM21; mitochondrial import inner membrane translocase subunit TIM21
D 105876788 TIMM50; mitochondrial import inner membrane translocase subunit TIM50 isoform X1 K17496 TIM50; mitochondrial import inner membrane translocase subunit TIM50
C Mitochondrial calcium uniporter complex
D 105870162 MCU; calcium uniporter protein, mitochondrial K20858 MCU; calcium uniporter protein, mitochondrial
D 105870163 MICU1; calcium uptake protein 1, mitochondrial isoform X1 K22827 MICU1; calcium uptake protein 1, mitochondrial
D 105878872 MICU2; calcium uptake protein 2, mitochondrial isoform X1 K22828 MICU2; calcium uptake protein 2, mitochondrial
D 105872313 MCUB; calcium uniporter regulatory subunit MCUb, mitochondrial isoform X1 K22829 MCUB; calcium uniporter regulatory subunit MCUb, mitochondrial
D 105858590 SMDT1; essential MCU regulator, mitochondrial K22830 SMDT1; essential MCU regulator, mitochondrial
D 105865118 MCUR1; LOW QUALITY PROTEIN: mitochondrial calcium uniporter regulator 1 K22137 MCUR1; mitochondrial calcium uniporter regulator 1
D 105870240 mitochondrial calcium uniporter regulator 1 K22137 MCUR1; mitochondrial calcium uniporter regulator 1
C Other inner membrane factors
D 105856033 LETM1; LETM1 and EF-hand domain-containing protein 1, mitochondrial K17800 LETM1; LETM1 and EF-hand domain-containing protein 1, mitochondrial
D 105883063 IMMP1L; mitochondrial inner membrane protease subunit 1 K09647 IMP1; mitochondrial inner membrane protease subunit 1 [EC:3.4.21.-]
D 105861725 IMMP2L; mitochondrial inner membrane protease subunit 2 isoform X1 K09648 IMP2; mitochondrial inner membrane protease subunit 2 [EC:3.4.21.-]
D 105856803 TMEM126A; transmembrane protein 126A K18157 TMEM126A; transmembrane protein 126A
D 105877857 MPC1; mitochondrial pyruvate carrier 1 isoform X1 K22138 MPC1; mitochondrial pyruvate carrier 1
D 105880635 MPC1L; mitochondrial pyruvate carrier 1-like protein K22138 MPC1; mitochondrial pyruvate carrier 1
D 105881901 MPC2; mitochondrial pyruvate carrier 2 K22139 MPC2; mitochondrial pyruvate carrier 2
D 105877813 CHCHD10; coiled-coil-helix-coiled-coil-helix domain-containing protein 10, mitochondrial K22759 CHCHD10; coiled-coil-helix-coiled-coil-helix domain-containing protein 10
D 105873642 OCIAD1; OCIA domain-containing protein 1 isoform X1 K28215 OCIAD1; OCIA domain-containing protein 1
B Matrix
C MMC/PAM proteins
D 105885653 TIMM44; mitochondrial import inner membrane translocase subunit TIM44 isoform X1 K17804 TIM44; mitochondrial import inner membrane translocase subunit TIM44
D 105862584 HSPA9; stress-70 protein, mitochondrial K04043 dnaK; molecular chaperone DnaK
D 105858447 GRPEL2; grpE protein homolog 2, mitochondrial K03687 GRPE; molecular chaperone GrpE
D 105881684 GRPEL1; grpE protein homolog 1, mitochondrial K03687 GRPE; molecular chaperone GrpE
D 109731267 grpE protein homolog 1, mitochondrial-like K03687 GRPE; molecular chaperone GrpE
D 105855802 DNAJC19; mitochondrial import inner membrane translocase subunit TIM14 isoform X1 K09539 DNAJC19; DnaJ homolog subfamily C member 19
D 105859705 PAM16; mitochondrial import inner membrane translocase subunit TIM16 K17805 PAM16; mitochondrial import inner membrane translocase subunit TIM16
C Other matrix factors
D 105880787 PMPCA; mitochondrial-processing peptidase subunit alpha isoform X1 K01412 PMPCA; mitochondrial-processing peptidase subunit alpha [EC:3.4.24.64]
D 105861550 PMPCB; mitochondrial-processing peptidase subunit beta K17732 PMPCB; mitochondrial-processing peptidase subunit beta [EC:3.4.24.64]
D 105855732 MIPEP; mitochondrial intermediate peptidase K01410 MIPEP; mitochondrial intermediate peptidase [EC:3.4.24.59]
D 105877936 HSPD1; 60 kDa heat shock protein, mitochondrial K04077 groEL; chaperonin GroEL [EC:5.6.1.7]
D 105877937 HSPE1; 10 kDa heat shock protein, mitochondrial isoform X1 K04078 groES; chaperonin GroES
D 105859717 DNAJA3; dnaJ homolog subfamily A member 3, mitochondrial isoform X1 K09504 DNAJA3; DnaJ homolog subfamily A member 3
D 105882665 TAMM41; phosphatidate cytidylyltransferase, mitochondrial isoform X1 K17807 TAM41; mitochondrial translocator assembly and maintenance protein 41
D 105880791 DNLZ; DNL-type zinc finger protein K17808 ZIM17; mitochondrial protein import protein ZIM17
D 105869459 ETHE1; persulfide dioxygenase ETHE1, mitochondrial isoform X1 K17725 ETHE1; sulfur dioxygenase [EC:1.13.11.18]
D 105857933 POLDIP2; polymerase delta-interacting protein 2 K17809 POLDIP2; polymerase delta-interacting protein 2
#
AMitochondrial quality control factors
B Regulator of mitochondrial biogenesis
C Ubiquitas transcription factors
D 105864304 CREB1; cyclic AMP-responsive element-binding protein 1 isoform X1 K05870 CREB1; cyclic AMP-responsive element-binding protein 1
D 105857415 GFM1; elongation factor G, mitochondrial K02355 fusA; elongation factor G
D 105881805 GFM2; ribosome-releasing factor 2, mitochondrial isoform X1 K02355 fusA; elongation factor G
D 105885222 ribosome-releasing factor 2, mitochondrial-like K02355 fusA; elongation factor G
D 105870777 SP1; transcription factor Sp1 K04684 SP1; transcription factor Sp1
D 105861551 YY1; transcriptional repressor protein YY1 K09201 YY; transcription factor YY
D 105882361 YY2; transcription factor YY2 K09201 YY; transcription factor YY
D 105870714 GABPB1; GA-binding protein subunit beta-1 isoform X1 K09454 GABPB; GA-binding protein transcription factor, beta
D 105873070 GABPB2; LOW QUALITY PROTEIN: GA-binding protein subunit beta-2 K09454 GABPB; GA-binding protein transcription factor, beta
D 105870281 TFAM; transcription factor A, mitochondrial K11830 TFAM; transcription factor A, mitochondrial
C Nuclear respiratory factors
D 105884861 NRF1; nuclear respiratory factor 1 isoform X1 K11831 NRF1; nuclear respiratory factor 1
D 105883030 NFE2L2; nuclear factor erythroid 2-related factor 2 isoform X1 K05638 NFE2L2; nuclear factor erythroid 2-related factor 2
D 105866476 metallothionein-2 isoform X1 K14739 MT1_2; metallothionein 1/2
D 105866477 metallothionein-2-like K14739 MT1_2; metallothionein 1/2
D 105866478 metallothionein-1E-like K14739 MT1_2; metallothionein 1/2
D 105866553 metallothionein-1X-like K14739 MT1_2; metallothionein 1/2
D 105866554 metallothionein-1E K14739 MT1_2; metallothionein 1/2
D 105866560 metallothionein-1E K14739 MT1_2; metallothionein 1/2
D 109729773 metallothionein-4 K14741 MT4; metallothionein 4
C Coactivators
D 105856056 PPARGC1A; peroxisome proliferator-activated receptor gamma coactivator 1-alpha isoform X1 K07202 PPARGC1A; alpha peroxisome proliferator-activated receptor gamma coactivator 1-alpha
D 105858430 PPARGC1B; peroxisome proliferator-activated receptor gamma coactivator 1-beta isoform X1 K17962 PPARGC1B; peroxisome proliferator-activated receptor gamma coactivator 1-beta
D 105874107 PPRC1; peroxisome proliferator-activated receptor gamma coactivator-related protein 1 isoform X1 K17963 PPRC1; peroxisome proliferator-activated receptor gamma coactivator-related protein 1
D 105854974 CRTC1; CREB-regulated transcription coactivator 1 isoform X1 K15309 CRTC1; CREB-regulated transcription coactivator 1
D 105858560 EP300; histone acetyltransferase p300 K04498 EP300; E1A/CREB-binding protein [EC:2.3.1.48]
D 105859673 CREBBP; CREB-binding protein isoform X1 K04498 EP300; E1A/CREB-binding protein [EC:2.3.1.48]
C Proteins involved in Fe-S cluster biogenesis
D 105865911 NFS1; cysteine desulfurase, mitochondrial isoform X1 K04487 iscS; cysteine desulfurase [EC:2.8.1.7]
D 105866920 ISCU; iron-sulfur cluster assembly enzyme ISCU, mitochondrial isoform X1 K22068 ISCU; iron-sulfur cluster assembly enzyme ISCU, mitochondrial
D 105856211 LYRM4; LYR motif-containing protein 4 isoform X1 K22069 LYRM4; LYR motif-containing protein 4
D 105856583 FXN; frataxin, mitochondrial K19054 FXN; frataxin [EC:1.16.3.1]
D 105876150 FDX1; adrenodoxin, mitochondrial K22070 FDX1; ferredoxin-1, mitochondrial
D 105885595 FDX1L; ferredoxin-2, mitochondrial K22071 FDX2; ferredoxin-2, mitochondrial
D 105878913 FDXR; NADPH:adrenodoxin oxidoreductase, mitochondrial K18914 FDXR; adrenodoxin-NADP+ reductase [EC:1.18.1.6]
D 105862584 HSPA9; stress-70 protein, mitochondrial K04043 dnaK; molecular chaperone DnaK
D 105870894 HSCB; iron-sulfur cluster co-chaperone protein HscB, mitochondrial isoform X1 K04082 hscB; molecular chaperone HscB
D 105862513 SIL1; nucleotide exchange factor SIL1 K14001 SIL1; nucleotide exchange factor SIL1
D 105885796 GLRX5; glutaredoxin-related protein 5, mitochondrial K07390 grxD; monothiol glutaredoxin
D 105855627 ISCA1; iron-sulfur cluster assembly 1 homolog, mitochondrial K22063 ISCA1; iron-sulfur cluster assembly 1
D 105858704 ISCA2; iron-sulfur cluster assembly 2 homolog, mitochondrial isoform X1 K22072 ISCA2; iron-sulfur cluster assembly 2
D 105878823 IBA57; putative transferase CAF17, mitochondrial isoform X1 K22073 IBA57; transferase CAF17, mitochondrial [EC:2.1.-.-]
D 105863269 NFU1; NFU1 iron-sulfur cluster scaffold homolog, mitochondrial K22074 NFU1; NFU1 iron-sulfur cluster scaffold homolog, mitochondrial
D 105865500 BOLA1; bolA-like protein 1 K22066 BOLA1; BolA-like protein 1
D 105884597 BOLA3; bolA-like protein 3 K22075 BOLA3; BolA-like protein 3
D 105873810 SFXN3; sideroflexin-3 isoform X1 K23500 SFXN1_3; sideroflexin-1/3
D 105880476 SFXN1; sideroflexin-1 K23500 SFXN1_3; sideroflexin-1/3
D 105874319 SFXN2; sideroflexin-2 isoform X1 K23501 SFXN2; sideroflexin-2
D 105857045 SFXN4; sideroflexin-4 K23502 SFXN4; sideroflexin-4
D 105863242 SFXN5; sideroflexin-5 isoform X1 K23503 SFXN5; sideroflexin-5
C Other regulator of mitochondrial biogenesis
D 105867480 CPT1A; carnitine O-palmitoyltransferase 1, liver isoform K08765 CPT1A; carnitine O-palmitoyltransferase 1, liver isoform [EC:2.3.1.21]
D 105884997 CPT1B; carnitine O-palmitoyltransferase 1, muscle isoform K19523 CPT1B; carnitine O-palmitoyltransferase 1, muscle isoform [EC:2.3.1.21]
D 105865647 CPT1C; LOW QUALITY PROTEIN: carnitine O-palmitoyltransferase 1, brain isoform K19524 CPT1C; carnitine O-palmitoyltransferase 1, brain isoform [EC:2.3.1.21]
D 105857730 DHTKD1; probable 2-oxoglutarate dehydrogenase E1 component DHKTD1, mitochondrial K15791 DHKTD1; 2-oxoadipate dehydrogenase E1 component [EC:1.2.4.-]
D 105874009 FBXL4; F-box/LRR-repeat protein 4 isoform X1 K10270 FBXL4; F-box and leucine-rich repeat protein 4
D 105867313 HCFC1; host cell factor 1 isoform X1 K14966 HCFC1; host cell factor 1
D 105875585 HCFC2; host cell factor 2 isoform X1 K27390 HCFC2; host cell factor 2
D 105856002 HTT; huntingtin isoform X1 K04533 HD; huntingtin
D 105878476 ESRRA; steroid hormone receptor ERR1 isoform X1 K08552 NR3B1; estrogen-related receptor alpha
D 105859036 ESRRB; steroid hormone receptor ERR2 isoform X1 K08553 NR3B2; estrogen-related receptor beta
D 105860671 ESRRG; estrogen-related receptor gamma isoform X1 K08554 NR3B3; estrogen-related receptor gamma
D 105883108 NRIP1; nuclear receptor-interacting protein 1 K17965 NRIP1; nuclear receptor-interacting protein 1
D 105878394 PRKAA1; 5'-AMP-activated protein kinase catalytic subunit alpha-1 isoform X2 K07198 PRKAA; 5'-AMP-activated protein kinase, catalytic alpha subunit [EC:2.7.11.31]
D 105882576 PRKAA2; 5'-AMP-activated protein kinase catalytic subunit alpha-2 K07198 PRKAA; 5'-AMP-activated protein kinase, catalytic alpha subunit [EC:2.7.11.31]
D 105869644 LONP1; lon protease homolog, mitochondrial K08675 PRSS15; ATP-dependent Lon protease [EC:3.4.21.53]
D 105870235 SIRT1; NAD-dependent protein deacetylase sirtuin-1 isoform X1 K11411 SIRT1; NAD-dependent protein deacetylase sirtuin 1 [EC:2.3.1.286]
D 105868568 SERAC1; protein SERAC1 isoform X1 K23504 SERAC1; protein SERAC1
D 105862166 UXT; protein UXT K26537 UXT; protein UXT
D 105877628 PERM1; PGC-1 and ERR-induced regulator in muscle protein 1 K26738 PERM1; PGC-1 and ERR-induced regulator in muscle protein 1
D 105879100 C31H21orf33; ES1 protein homolog, mitochondrial K27835 GATD3; glutamine amidotransferase-like class 1 domain-containing protein 3, mitochondrial
D 105877723 TANGO2; transport and Golgi organization protein 2 homolog isoform X1 K28098 TANGO2; transport and Golgi organization protein 2
D 105856987 C8H2orf69; UPF0565 protein C2orf69 homolog K28271 C2ORF69; mitochondrial protein C2orf69
D 105870690 FAM214A; protein FAM214A isoform X1 K28528 ATOS; protein Atossa
D 105874318 FAM214B; protein FAM214B isoform X1 K28528 ATOS; protein Atossa
D 105872236 LOW QUALITY PROTEIN: probable ATP-dependent RNA helicase DDX47 K14777 DDX47; ATP-dependent RNA helicase DDX47/RRP3 [EC:5.6.2.7]
D 105876711 DDX47; probable ATP-dependent RNA helicase DDX47 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 105872473 NDUFAF1; complex I intermediate-associated protein 30, mitochondrial K18159 NDUFAF1; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex assembly factor 1
D 105877260 NDUFAF2; mimitin, mitochondrial K18160 NDUFAF2; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex assembly factor 2
D 105874946 NDUFAF3; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex assembly factor 3 isoform X1 K09008 NDUFAF3; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex assembly factor 3
D 105874016 NDUFAF4; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex assembly factor 4 K18161 NDUFAF4; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex assembly factor 4
D 105880070 NDUFAF5; arginine-hydroxylase NDUFAF5, mitochondrial isoform X1 K18162 NDUFAF5; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex assembly factor 5 [EC:2.1.1.-]
D 105875660 NDUFAF6; NADH dehydrogenase (ubiquinone) complex I, assembly factor 6 isoform X1 K18163 NDUFAF6; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex assembly factor 6
D 105884501 NDUFAF7; protein arginine methyltransferase NDUFAF7, mitochondrial isoform X1 K18164 NDUFAF7; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex assembly factor 7
D 105884974 NDUFAF8; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex assembly factor 8 isoform X1 K24726 NDUFAF8; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex assembly factor 8
D 105856799 TMEM126B; complex I assembly factor TMEM126B, mitochondrial K18165 TMEM126B; complex I assembly factor TMEM126B
D 105885556 ECSIT; evolutionarily conserved signaling intermediate in Toll pathway, mitochondrial isoform X1 K04405 ECSIT; evolutionarily conserved signaling intermediate in Toll pathway
D 105882649 ACAD9; acyl-CoA dehydrogenase family member 9, mitochondrial K15980 ACAD9; acyl-CoA dehydrogenase family member 9 [EC:1.3.99.-]
D 105875960 NUBPL; iron-sulfur protein NUBPL isoform X1 K03593 mrp; ATP-binding protein involved in chromosome partitioning
D 105875357 FOXRED1; FAD-dependent oxidoreductase domain-containing protein 1 K18166 FOXRED1; FAD-dependent oxidoreductase domain-containing protein 1
D 105864948 RTN4IP1; reticulon-4-interacting protein 1, mitochondrial isoform X1 K23164 RTN4IP1; reticulon-4-interacting protein 1, mitochondrial
D 105879925 TIMMDC1; complex I assembly factor TIMMDC1, mitochondrial K23505 TIMMDC1; complex I assembly factor TIMMDC1
C Complex-II assembly factors
D 105876898 SDHAF1; succinate dehydrogenase assembly factor 1, mitochondrial K18167 SDHAF1; succinate dehydrogenase assembly factor 1
D 105878633 SDHAF2; succinate dehydrogenase assembly factor 2, mitochondrial isoform X1 K18168 SDHAF2; succinate dehydrogenase assembly factor 2
C Complex-III assembly factors
D 105857695 mitochondrial chaperone BCS1 K08900 BCS1; mitochondrial chaperone BCS1
D 105856311 TTC19; tetratricopeptide repeat protein 19, mitochondrial isoform X2 K18169 TTC19; tetratricopeptide repeat protein 19, mitochondrial
D 105869936 LYRM7; complex III assembly factor LYRM7 isoform X1 K18170 LYRM7; complex III assembly factor LYRM7
D 105865869 ubiquinol-cytochrome-c reductase complex assembly factor 1 isoform X1 K17662 CBP3; cytochrome b pre-mRNA-processing protein 3
D 105861403 ubiquinol-cytochrome-c reductase complex assembly factor 2 K17682 MNF1; mitochondrial nucleoid factor 1
D 105878590 ubiquinol-cytochrome-c reductase complex assembly factor 3 K23165 UQCC3; ubiquinol-cytochrome-c reductase complex assembly factor 3
D 105859623 C17H16orf91; protein CCSMST1 K27339 UQCC4; ubiquinol-cytochrome c reductase complex assembly factor 4
D 105875056 SMIM4; small integral membrane protein 4 K27340 UQCC5; ubiquinol-cytochrome c reductase complex assembly factor 5
D 105875582 C7H12orf73; uncharacterized protein C12orf73 homolog K27341 UQCC6; ubiquinol-cytochrome c reductase complex assembly factor 6
D 105860204 C11H7orf73; uncharacterized protein C7orf73 homolog K27319 STMP1; short transmembrane mitochondrial protein 1
C Complex-IV assembly factors
D 105883581 CMC1; COX assembly mitochondrial protein homolog isoform X1 K18171 CMC1; COX assembly mitochondrial protein 1
D 105861206 CMC2; COX assembly mitochondrial protein 2 homolog K18172 CMC2; COX assembly mitochondrial protein 2
D 105858410 cytochrome c oxidase assembly factor 1 homolog isoform X1 K18173 COA1; cytochrome c oxidase assembly factor 1
D 105859447 cytochrome c oxidase assembly factor 3 homolog, mitochondrial K18175 CCDC56; cytochrome c oxidase assembly factor 3, animal type
D 105860383 cytochrome c oxidase assembly factor 4 homolog, mitochondrial isoform X1 K18177 COA4; cytochrome c oxidase assembly factor 4
D 105875822 cytochrome c oxidase assembly factor 5 K18178 COA5; cytochrome c oxidase assembly factor 5
D 105883673 cytochrome c oxidase assembly factor 5 isoform X1 K18178 COA5; cytochrome c oxidase assembly factor 5
D 105856473 cytochrome c oxidase assembly factor 6 homolog isoform X1 K18179 COA6; cytochrome c oxidase assembly factor 6
D 105877563 cytochrome c oxidase assembly factor 7 isoform X1 K18180 COA7; cytochrome c oxidase assembly factor 7
D 105861972 protoheme IX farnesyltransferase, mitochondrial K02257 COX10; heme o synthase [EC:2.5.1.141]
D 105876415 cytochrome c oxidase assembly protein COX11, mitochondrial K02258 COX11; cytochrome c oxidase assembly protein subunit 11
D 105869867 cytochrome c oxidase assembly protein COX14 K18181 COX14; cytochrome c oxidase assembly factor 14
D 105873394 cytochrome c oxidase assembly protein COX15 homolog isoform X1 K02259 COX15; heme a synthase [EC:1.17.99.9]
D 105880279 cytochrome c oxidase assembly protein COX16 homolog, mitochondrial isoform X1 K18182 COX16; cytochrome c oxidase assembly protein subunit 16
D 105879919 cytochrome c oxidase copper chaperone K02260 COX17; cytochrome c oxidase assembly protein subunit 17
D 105860331 mitochondrial inner membrane protein COX18 isoform X1 K17797 COX18; mitochondrial inner membrane protein COX18
D 105868356 cytochrome c oxidase assembly protein COX19 K18183 COX19; cytochrome c oxidase assembly protein subunit 19
D 105863786 cytochrome c oxidase protein 20 homolog K18184 COX20; cytochrome c oxidase assembly protein subunit 20
D 105868800 MSS51; putative protein MSS51 homolog, mitochondrial K17656 MSS51; mitochondrial splicing suppressor protein 51
D 105885639 PET100; protein PET100 homolog, mitochondrial K18186 PET100; protein PET100, animal type
D 105879701 PET117; protein PET117 homolog, mitochondrial K18188 PET117; protein PET117
D 105864717 OXA1L; mitochondrial inner membrane protein OXA1L K03217 yidC; YidC/Oxa1 family membrane protein insertase
D 105880764 surfeit locus protein 1 isoform X1 K14998 SURF1; surfeit locus 1 family protein
D 105878333 protein SCO1 homolog, mitochondrial isoform X1 K07152 SCO1; protein SCO1
D 105885006 protein SCO2 homolog, mitochondrial isoform X1 K23755 SCO2; protein SCO2
D 105861701 LOW QUALITY PROTEIN: translational activator of cytochrome c oxidase 1 K18189 TACO1; translational activator of cytochrome c oxidase 1
D 105866800 LRPPRC; leucine-rich PPR motif-containing protein, mitochondrial K17964 LRPPRC; leucine-rich PPR motif-containing protein, mitochondrial
D 105861078 APOPT1; apoptogenic protein 1, mitochondrial isoform X1 K23506 APOPT1; apoptogenic protein 1
D 105860204 C11H7orf73; uncharacterized protein C7orf73 homolog K27319 STMP1; short transmembrane mitochondrial protein 1
D 105875056 SMIM4; small integral membrane protein 4 K27340 UQCC5; ubiquinol-cytochrome c reductase complex assembly factor 5
D 105878579 TMEM223; transmembrane protein 223 K27342 TMEM223; transmembrane protein 223
D 105870041 HIGD1C; HIG1 domain family member 1C K28216 HIGD1A_C; HIG1 domain family member 1A/C, mitochondrial
D 105874431 HIGD1A; HIG1 domain family member 1A, mitochondrial K28216 HIGD1A_C; HIG1 domain family member 1A/C, mitochondrial
D 105880467 HIGD2A; HIG1 domain family member 2A, mitochondrial K28217 HIGD2A; HIG1 domain family member 2A, mitochondrial
C Complex-V assembly factors
D 105877505 ATPAF1; ATP synthase mitochondrial F1 complex assembly factor 1 isoform X1 K07555 ATPeAF1; ATP synthase mitochondrial F1 complex assembly factor 1
D 105861961 ATPAF2; ATP synthase mitochondrial F1 complex assembly factor 2 isoform X1 K07556 ATPeAF2; ATP synthase mitochondrial F1 complex assembly factor 2
D 105854797 TMEM70; transmembrane protein 70, mitochondrial K17966 TMEM70; transmembrane protein 70, mitochondrial
D 105861134 C3H14orf2; 6.8 kDa mitochondrial proteolipid isoform X1 K18193 MP68; 6.8 kDa mitochondrial proteolipid
D 105878347 up-regulated during skeletal muscle growth protein 5 K18194 USMG5; up-regulated during skeletal muscle growth protein 5
D 109729413 USMG5; up-regulated during skeletal muscle growth protein 5 K18194 USMG5; up-regulated during skeletal muscle growth protein 5
B Mitochondrial dynamics
C Fission and Fusion factors
D 105855819 MFN1; mitofusin-1 K21356 MFN1; mitofusin 1 [EC:3.6.5.-]
D 105882711 MFN2; mitofusin-2 K06030 MFN2; mitofusin 2 [EC:3.6.5.-]
D 105879775 OPA1; dynamin-like 120 kDa protein, mitochondrial isoform X1 K17079 OPA1; optic atrophy protein 1 [EC:3.6.5.5]
D 105869539 OPA3; optic atrophy 3 protein isoform X1 K23166 OPA3; optic atrophy 3 protein
D 105866932 TRIAP1; TP53-regulated inhibitor of apoptosis 1 K17968 TRIAP1; TRIAP1/MDM35 family protein
D 105855781 PARL; presenilins-associated rhomboid-like protein, mitochondrial isoform X1 K09650 PARL; rhomboid-like protein [EC:3.4.21.105]
D 105861442 BAK1; bcl-2 homologous antagonist/killer isoform X1 K14021 BAK; Bcl-2 homologous antagonist/killer
D 105865602 BAX; apoptosis regulator BAX isoform X2 K02159 BAX; apoptosis regulator BAX
D 105884879 DNM1L; dynamin-1-like protein isoform X1 K17065 DNM1L; dynamin 1-like protein [EC:3.6.5.5]
D 105868402 FIS1; mitochondrial fission 1 protein K17969 FIS1; mitochondrial fission 1 protein
D 105856947 SUMO1; small ubiquitin-related modifier 1 K12160 SUMO; small ubiquitin-related modifier
D 105859346 uncharacterized protein LOC105859346 K12160 SUMO; small ubiquitin-related modifier
D 105878934 SUMO2; small ubiquitin-related modifier 2 K12160 SUMO; small ubiquitin-related modifier
D 105879655 SUMO3; small ubiquitin-related modifier 3 K12160 SUMO; small ubiquitin-related modifier
D 105861773 SH3GLB1; endophilin-B1 isoform X1 K11248 SH3GLB1; endophilin-B1
D 105861406 MTCH1; mitochondrial carrier homolog 1 isoform X1 K17885 MTCH; mitochondrial carrier
D 105884594 MTCH2; mitochondrial carrier homolog 2 K17885 MTCH; mitochondrial carrier
D 105862835 MFF; mitochondrial fission factor isoform X1 K22076 MFF; mitochondrial fission factor
D 105854791 GDAP1; ganglioside-induced differentiation-associated protein 1 isoform X1 K22077 GDAP1; ganglioside-induced differentiation-associated protein 1
D 105880117 GDAP1L1; ganglioside-induced differentiation-associated protein 1-like 1 K22077 GDAP1; ganglioside-induced differentiation-associated protein 1
D 105859428 VAT1; synaptic vesicle membrane protein VAT-1 homolog K23167 VAT1; synaptic vesicle membrane protein VAT-1
D 105858534 MIEF1; mitochondrial dynamics protein MID51 isoform X3 K23507 MIEF1_2; mitochondrial dynamics protein MID51/49
D 105861969 MIEF2; mitochondrial dynamics protein MID49 K23507 MIEF1_2; mitochondrial dynamics protein MID51/49
D 105856330 PLD6; mitochondrial cardiolipin hydrolase isoform X1 K16862 PLD6; mitochondrial cardiolipin hydrolase [EC:3.1.4.-]
D 105875275 MIGA2; mitoguardin 2 isoform X1 K27289 MIGA; mitoguardin
D 105883893 MIGA1; mitoguardin 1 K27289 MIGA; mitoguardin
C Actin cytoskeleton organization
D 105868294 ACTB; actin, cytoplasmic 1 K05692 ACTB_G1; actin beta/gamma 1
D 105884969 ACTG1; actin, cytoplasmic 2 K05692 ACTB_G1; actin beta/gamma 1
D 105867384 NAA20; N-alpha-acetyltransferase 20 K17972 NAA20; N-terminal acetyltransferase B complex catalytic subunit [EC:2.3.1.254]
D 105878700 NAA25; LOW QUALITY PROTEIN: N-alpha-acetyltransferase 25, NatB auxiliary subunit K17973 NAA25; N-terminal acetyltransferase B complex non-catalytic subunit
C Mitochondrial movement
D 105867761 prohibitin-like K17080 PHB1; prohibitin 1
D 105870320 prohibitin-like K17080 PHB1; prohibitin 1
D 105885091 PHB; prohibitin K17080 PHB1; prohibitin 1
D 105868987 PHB2; prohibitin-2 K17081 PHB2; prohibitin 2
D 105867666 KTN1; kinectin isoform X1 K17975 KTN1; kinectin
C Retention of mitochondria
D 105884879 DNM1L; dynamin-1-like protein isoform X1 K17065 DNM1L; dynamin 1-like protein [EC:3.6.5.5]
D 105870692 MYO5A; unconventional myosin-Va isoform X1 K10357 MYO5; myosin V
D 105870694 MYO5C; unconventional myosin-Vc isoform X1 K10357 MYO5; myosin V
D 105874244 MYO5B; unconventional myosin-Vb isoform X1 K10357 MYO5; myosin V
D 105860713 XIAP; E3 ubiquitin-protein ligase XIAP isoform X1 K04725 XIAP; E3 ubiquitin-protein ligase XIAP [EC:2.3.2.27]
D 105880894 DIABLO; diablo homolog, mitochondrial isoform X1 K10522 DIABLO; diablo
C Morphogenesis factors
D 105882487 SNX25; sorting nexin-25 isoform X1 K17887 SNX25; sorting nexin-25
D 105855903 RHOT1; mitochondrial Rho GTPase 1 isoform X1 K07870 RHOT1; mitochondrial Rho GTPase 1 [EC:3.6.5.-]
D 105876613 IMMT; MICOS complex subunit MIC60 isoform X1 K17785 IMMT; MICOS complex subunit MIC60
D 105870931 MTFP1; mitochondrial fission process protein 1 K17981 MTFP1; mitochondrial fission process protein 1
D 105873209 DAP3; 28S ribosomal protein S29, mitochondrial K17408 DAP3; small subunit ribosomal protein S29
D 105881413 NEDD4; E3 ubiquitin-protein ligase NEDD4 isoform X1 K10591 NEDD4; E3 ubiquitin-protein ligase NEDD4 [EC:2.3.2.26]
D 105882133 OXR1; oxidation resistance protein 1 isoform X1 K25437 OXR1; oxidation resistance protein 1
D 105873159 MSTO1; protein misato homolog 1 isoform X1 K27320 MSTO1; protein misato
D 105883850 PRELID1; PRELI domain-containing protein 1, mitochondrial K27964 PRELID1; PRELI domain-containing protein 1, mitochondrial
D 105862618 PRELID2; PRELI domain-containing protein 2 isoform X1 K27965 PRELID2; PRELI domain-containing protein 2 mitochondrial
D 105874512 PRELID3B; PRELI domain containing protein 3B K27966 PRELID3; PRELI domain-containing protein 3, mitochondrial
B Mitophagy factors
C Autophagy-related proteins
D 105878494 ATG2A; autophagy-related protein 2 homolog A isoform X1 K17906 ATG2; autophagy-related protein 2
D 105885803 ATG2B; autophagy-related protein 2 homolog B isoform X1 K17906 ATG2; autophagy-related protein 2
D 105883910 ATG3; ubiquitin-like-conjugating enzyme ATG3 K08343 ATG3; ubiquitin-like-conjugating enzyme ATG3
D 105863441 ATG4A; cysteine protease ATG4A isoform X1 K08342 ATG4; cysteine protease ATG4 [EC:3.4.22.-]
D 105866388 ATG4B; cysteine protease ATG4B isoform X1 K08342 ATG4; cysteine protease ATG4 [EC:3.4.22.-]
D 105872807 ATG4C; cysteine protease ATG4C isoform X1 K08342 ATG4; cysteine protease ATG4 [EC:3.4.22.-]
D 105885584 ATG4D; cysteine protease ATG4D K08342 ATG4; cysteine protease ATG4 [EC:3.4.22.-]
D 105864958 ATG5; autophagy protein 5 isoform X1 K08339 ATG5; autophagy-related protein 5
D 105859442 BECN1; beclin-1 isoform X1 K08334 BECN; beclin
D 105882655 ATG7; ubiquitin-like modifier-activating enzyme ATG7 isoform X1 K08337 ATG7; ubiquitin-like modifier-activating enzyme ATG7
D 105866301 GABARAPL1; gamma-aminobutyric acid receptor-associated protein-like 1 K08341 GABARAP; GABA(A) receptor-associated protein
D 105875460 GABARAPL2; gamma-aminobutyric acid receptor-associated protein-like 2 K08341 GABARAP; GABA(A) receptor-associated protein
D 105877898 GABARAP; gamma-aminobutyric acid receptor-associated protein K08341 GABARAP; GABA(A) receptor-associated protein
D 105857669 ATG9A; autophagy-related protein 9A isoform X1 K17907 ATG9; autophagy-related protein 9
D 105883170 ATG9B; autophagy-related protein 9B isoform X2 K17907 ATG9; autophagy-related protein 9
D 105882205 ATG10; ubiquitin-like-conjugating enzyme ATG10 isoform X1 K17888 ATG10L; ubiquitin-like-conjugating enzyme ATG10
D 105859520 ATG12; ubiquitin-like protein ATG12 K08336 ATG12; ubiquitin-like protein ATG12
D 105867661 ATG14; beclin 1-associated autophagy-related key regulator isoform X1 K17889 ATG14L; beclin 1-associated autophagy-related key regulator
D 105886147 ATG16L1; autophagy-related protein 16-1 isoform X1 K17890 ATG16L1; autophagy-related protein 16-1
D 105860298 ATG16L2; autophagy-related protein 16-2 isoform X1 K20868 ATG16L2; autophagy-related protein 16-2
D 105861685 WIPI1; WD repeat domain phosphoinositide-interacting protein 1 K17908 WIPI1_2; autophagy-related protein 18
D 105868428 WIPI2; WD repeat domain phosphoinositide-interacting protein 2 isoform X1 K17908 WIPI1_2; autophagy-related protein 18
C Parkin-dependent mechanism factors
D 105865182 RNF41; E3 ubiquitin-protein ligase NRDP1 isoform X1 K11981 RNF41; E3 ubiquitin-protein ligase NRDP1 [EC:2.3.2.27]
D 105882434 CLEC16A; protein CLEC16A isoform X1 K19513 CLEC16A; protein CLEC16A
D 105883977 PINK1; LOW QUALITY PROTEIN: serine/threonine-protein kinase PINK1, mitochondrial K05688 PINK1; PTEN induced putative kinase 1 [EC:2.7.11.1]
D 105855781 PARL; presenilins-associated rhomboid-like protein, mitochondrial isoform X1 K09650 PARL; rhomboid-like protein [EC:3.4.21.105]
D 105885196 SQSTM1; sequestosome-1 isoform X1 K14381 SQSTM1; sequestosome 1
D 105875934 TAX1BP1; tax1-binding protein 1 isoform X2 K21347 TAX1BP1; Tax1-binding protein 1
D 105872219 CALCOCO2; calcium-binding and coiled-coil domain-containing protein 2 K21348 CALCOCO2; calcium-binding and coiled-coil domain-containing protein 2
D 105885026 AMBRA1; activating molecule in BECN1-regulated autophagy protein 1 isoform X1 K17985 AMBRA1; activating molecule in BECN1-regulated autophagy protein 1
C Parkin-independent mechanism factors
D 105885072 ATG13; autophagy-related protein 13 isoform X1 K08331 ATG13; autophagy-related protein 13
D 105879455 ULK1; serine/threonine-protein kinase ULK1 K21357 ULK1; serine/threonine-protein kinase ULK1 [EC:2.7.11.1]
D 105856303 ULK2; serine/threonine-protein kinase ULK2 isoform X1 K08269 ULK2; serine/threonine-protein kinase ULK2 [EC:2.7.11.1]
D 105885261 RB1CC1; RB1-inducible coiled-coil protein 1 isoform X1 K17589 RB1CC1; RB1-inducible coiled-coil protein 1
D 105859524 HSP90AB1; LOW QUALITY PROTEIN: heat shock protein HSP 90-beta K04079 HSP90A; molecular chaperone HtpG
D 105861065 HSP90AA1; heat shock protein HSP 90-alpha K04079 HSP90A; molecular chaperone HtpG
D 105885590 CDC37; hsp90 co-chaperone Cdc37 K09554 CDC37; cell division cycle protein 37
D 105861697 SMURF2; E3 ubiquitin-protein ligase SMURF2 isoform X1 K04678 SMURF; E3 ubiquitin ligase SMURF1/2 [EC:2.3.2.26]
D 105868303 SMURF1; E3 ubiquitin-protein ligase SMURF1 isoform X1 K04678 SMURF; E3 ubiquitin ligase SMURF1/2 [EC:2.3.2.26]
D 105877117 BNIP3L; BCL2/adenovirus E1B 19 kDa protein-interacting protein 3-like isoform X1 K15465 BNIP3L; BCL2/adenovirus E1B 19 kDa protein-interacting protein 3-like
D 105882213 FUNDC1; FUN14 domain-containing protein 1 K17986 FUNDC1; FUN14 domain-containing protein 1
C Other mitophagy factors
D 105878394 PRKAA1; 5'-AMP-activated protein kinase catalytic subunit alpha-1 isoform X2 K07198 PRKAA; 5'-AMP-activated protein kinase, catalytic alpha subunit [EC:2.7.11.31]
D 105882576 PRKAA2; 5'-AMP-activated protein kinase catalytic subunit alpha-2 K07198 PRKAA; 5'-AMP-activated protein kinase, catalytic alpha subunit [EC:2.7.11.31]
D 105855051 MAP1LC3B; microtubule-associated proteins 1A/1B light chain 3B K10435 MAP1LC; microtubule-associated protein 1 light chain
D 105863722 microtubule-associated proteins 1A/1B light chain 3C-like K10435 MAP1LC; microtubule-associated protein 1 light chain
D 105863733 MAP1LC3C; microtubule-associated proteins 1A/1B light chain 3C K10435 MAP1LC; microtubule-associated protein 1 light chain
D 105865824 MAP1LC3A; microtubule-associated proteins 1A/1B light chain 3A K10435 MAP1LC; microtubule-associated protein 1 light chain
D 105859425 NBR1; next to BRCA1 gene 1 protein isoform X1 K17987 NBR1; next to BRCA1 gene 1 protein
D 105868478 TECPR1; tectonin beta-propeller repeat-containing protein 1 K17988 TECPR1; tectonin beta-propeller repeat-containing protein 1
D 105858144 ZFYVE1; zinc finger FYVE domain-containing protein 1 isoform X1 K17603 ZFYVE1; zinc finger FYVE domain-containing protein 1
D 105859403 COASY; bifunctional coenzyme A synthase isoform X1 K02318 COASY; phosphopantetheine adenylyltransferase / dephospho-CoA kinase [EC:2.7.7.3 2.7.1.24]
D 105858610 PLA2G6; 85/88 kDa calcium-independent phospholipase A2 isoform X1 K16343 PLA2G6; calcium-independent phospholipase A2 [EC:3.1.1.4]
D 105877008 C22H19orf12; protein C19orf12 homolog K23168 MPAN; protein MPAN
D 105864590 FUNDC2; FUN14 domain-containing protein 2 K24308 FUNDC2; FUN14 domain-containing protein 2
!
#
#[ BRITE | KEGG2 | KEGG ]
#Last updated: September 5, 2025