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Mitochondrial Biogenesis - Ursus americanus (American black bear)
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AMitochondrial DNA transcription, translation, and replication factors
B Mitochondrial DNA-encoded proteins
C Mitochondrial respiratory chain complex I
D 804833 ND1; NADH dehydrogenase subunit 1 K03878 ND1; NADH-ubiquinone oxidoreductase chain 1 [EC:7.1.1.2]
D 804834 ND2; NADH dehydrogenase subunit 2 K03879 ND2; NADH-ubiquinone oxidoreductase chain 2 [EC:7.1.1.2]
D 804845 ND3; NADH dehydrogenase subunit 3 K03880 ND3; NADH-ubiquinone oxidoreductase chain 3 [EC:7.1.1.2]
D 804839 ND4; NADH dehydrogenase subunit 4 K03881 ND4; NADH-ubiquinone oxidoreductase chain 4 [EC:7.1.1.2]
D 804842 ND4L; NADH dehydrogenase subunit 4L K03882 ND4L; NADH-ubiquinone oxidoreductase chain 4L [EC:7.1.1.2]
D 804837 ND5; NADH dehydrogenase subunit 5 K03883 ND5; NADH-ubiquinone oxidoreductase chain 5 [EC:7.1.1.2]
D 804843 ND6; NADH dehydrogenase subunit 6 K03884 ND6; NADH-ubiquinone oxidoreductase chain 6 [EC:7.1.1.2]
C Mitochondrial respiratory chain complex III
D 804840 CYTB; cytochrome b K00412 CYTB; ubiquinol-cytochrome c reductase cytochrome b subunit
C Cytochrome c oxidase
D 804835 COX1; cytochrome c oxidase subunit I K02256 COX1; cytochrome c oxidase subunit 1 [EC:7.1.1.9]
D 804844 COX2; cytochrome c oxidase subunit II K02261 COX2; cytochrome c oxidase subunit 2
D 804841 COX3; cytochrome c oxidase subunit III K02262 COX3; cytochrome c oxidase subunit 3
C F-type ATPase
D 804836 ATP8; ATP synthase F0 subunit 8 K02125 ATPeF08; F-type H+-transporting ATPase subunit 8
D 804838 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 123794064 POLRMT; LOW QUALITY PROTEIN: DNA-directed RNA polymerase, mitochondrial K10908 POLRMT; DNA-directed RNA polymerase, mitochondrial [EC:2.7.7.6]
C Mitochondrial transcription factors
D 123782931 TFAM; transcription factor A, mitochondrial K11830 TFAM; transcription factor A, mitochondrial
D 123797476 TFB1M; dimethyladenosine transferase 1, mitochondrial K15266 TFB1M; dimethyladenosine transferase 1, mitochondrial [EC:2.1.1.-]
D 123791507 TFB2M; dimethyladenosine transferase 2, mitochondrial isoform X1 K17653 TFB2M; dimethyladenosine transferase 2, mitochondrial [EC:2.1.1.-]
C Mitochondrial translation factors
D 123785936 MTIF2; translation initiation factor IF-2, mitochondrial isoform X1 K02519 infB; translation initiation factor IF-2
D 123791956 MTIF3; translation initiation factor IF-3, mitochondrial K02520 infC; translation initiation factor IF-3
D 123787195 TSFM; elongation factor Ts, mitochondrial K02357 tsf; elongation factor Ts
D 123784590 TUFM; elongation factor Tu, mitochondrial K02358 tuf; elongation factor Tu
D 123800192 GFM2; ribosome-releasing factor 2, mitochondrial isoform X1 K02355 fusA; elongation factor G
D 123801629 GFM1; elongation factor G, mitochondrial K02355 fusA; elongation factor G
D 123780763 MTERF2; transcription termination factor 2, mitochondrial K15031 MTERF; mitochondrial transcription termination factor family protein
D 123789302 MTERF1; transcription termination factor 1, mitochondrial K15031 MTERF; mitochondrial transcription termination factor family protein
D 123791235 MTERF4; transcription termination factor 4, mitochondrial isoform X1 K15031 MTERF; mitochondrial transcription termination factor family protein
D 123797863 MTERF3; transcription termination factor 3, mitochondrial K15031 MTERF; mitochondrial transcription termination factor family protein
D 123804088 GUF1; translation factor GUF1, mitochondrial K21594 GUF1; translation factor GUF1, mitochondrial [EC:3.6.5.-]
C RNase MRP
D 123797851 POP1; ribonucleases P/MRP protein subunit POP1 isoform X1 K01164 POP1; ribonuclease P/MRP protein subunit POP1 [EC:3.1.26.5]
D 123776621 ribonuclease P protein subunit p29 isoform X1 K03538 POP4; ribonuclease P protein subunit POP4 [EC:3.1.26.5]
D 123788275 POP5; ribonuclease P/MRP protein subunit POP5 isoform X1 K03537 POP5; ribonuclease P/MRP protein subunit POP5 [EC:3.1.26.5]
D 123800344 RPP30; ribonuclease P protein subunit p30 K03539 RPP1; ribonuclease P/MRP protein subunit RPP1 [EC:3.1.26.5]
D 123777299 POP7; ribonuclease P protein subunit p20 K14527 RPP20; ribonuclease P/MRP protein subunit RPP20 [EC:3.1.26.5]
D 123781263 RPP25L; ribonuclease P protein subunit p25-like protein K14525 RPP25; ribonucleases P/MRP protein subunit RPP25 [EC:3.1.26.5]
D 123790673 RPP25; ribonuclease P protein subunit p25 K14525 RPP25; ribonucleases P/MRP protein subunit RPP25 [EC:3.1.26.5]
D 123785916 RPP38; ribonuclease P protein subunit p38 K14523 RPP38; ribonucleases P/MRP protein subunit RPP38 [EC:3.1.26.5]
D 123801573 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 123784102 TRMT10C; tRNA methyltransferase 10 homolog C K17654 TRMT10C; mitochondrial ribonuclease P protein 1 [EC:2.1.1.-]
D 123793530 HSD17B10; 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 123793913 mitochondrial ribonuclease P catalytic subunit isoform X1 K17655 MRPP3; mitochondrial ribonuclease P protein 3
C Other mitochondrial DNA transcription and translation factors
D 123782831 MSS51; putative protein MSS51 homolog, mitochondrial isoform X1 K17656 MSS51; mitochondrial splicing suppressor protein 51
D 123777068 TEFM; transcription elongation factor, mitochondrial K17658 TEFM; transcription elongation factor, mitochondrial
D 123777320 PTCD1; pentatricopeptide repeat-containing protein 1, mitochondrial isoform X1 K17710 PTCD1; pentatricopeptide repeat domain-containing protein 1
D 123800209 PTCD2; pentatricopeptide repeat-containing protein 2, mitochondrial isoform X1 K17711 PTCD2; pentatricopeptide repeat domain-containing protein 2
D 123799698 PTCD3; pentatricopeptide repeat domain-containing protein 3, mitochondrial isoform X1 K17659 PTCD3; pentatricopeptide repeat domain-containing protein 3
D 123779871 DHX30; ATP-dependent RNA helicase DHX30 isoform X1 K13185 DHX30; ATP-dependent RNA helicase DHX30 [EC:5.6.2.6]
D 123797950 ubiquinol-cytochrome-c reductase complex assembly factor 1 isoform X1 K17662 CBP3; cytochrome b pre-mRNA-processing protein 3
D 123798498 AFG1L; AFG1-like ATPase isoform X1 K18798 AFG1; peroxisome-assembly ATPase [EC:3.6.4.7]
D 123787191 ATP23; mitochondrial inner membrane protease ATP23 homolog isoform X1 K18156 ATP23; mitochondrial inner membrane protease ATP23 [EC:3.4.24.-]
D 123782964 SUPV3L1; ATP-dependent RNA helicase SUPV3L1, mitochondrial K17675 SUPV3L1; ATP-dependent RNA helicase SUPV3L1/SUV3 [EC:5.6.2.6]
D 123800495 LACTB2; endoribonuclease LACTB2 K16639 LACTB2; endoribonuclease LACTB2 [EC:3.1.27.-]
D 123784508 SLC25A4; ADP/ATP translocase 1 K05863 SLC25A4S; solute carrier family 25 (mitochondrial adenine nucleotide translocator), member 4/5/6/31
D 123787795 SLC25A5; ADP/ATP translocase 2 K05863 SLC25A4S; solute carrier family 25 (mitochondrial adenine nucleotide translocator), member 4/5/6/31
D 123793297 SLC25A31; ADP/ATP translocase 4 K05863 SLC25A4S; solute carrier family 25 (mitochondrial adenine nucleotide translocator), member 4/5/6/31
D 123802759 SLC25A6; ADP/ATP translocase 3 K05863 SLC25A4S; solute carrier family 25 (mitochondrial adenine nucleotide translocator), member 4/5/6/31
D 123777361 SLC25A37; mitoferrin-1 isoform X1 K15113 SLC25A28_37; solute carrier family 25 (mitochondrial iron transporter), member 28/37
D 123783601 SLC25A28; mitoferrin-2 isoform X1 K15113 SLC25A28_37; solute carrier family 25 (mitochondrial iron transporter), member 28/37
D 123789173 MALSU1; mitochondrial assembly of ribosomal large subunit protein 1 K25803 MALSU1; mitochondrial assembly of ribosomal large subunit protein 1
D 123777054 ERAL1; GTPase Era, mitochondrial K03595 era; GTPase
D 123782139 YARS1; tyrosine--tRNA ligase, cytoplasmic isoform X1 K01866 YARS; tyrosyl-tRNA synthetase [EC:6.1.1.1]
D 123801709 YARS2; tyrosine--tRNA ligase, mitochondrial isoform X1 K01866 YARS; tyrosyl-tRNA synthetase [EC:6.1.1.1]
D 123780081 LARS2; probable leucine--tRNA ligase, mitochondrial K01869 LARS; leucyl-tRNA synthetase [EC:6.1.1.4]
D 123790193 LARS1; leucine--tRNA ligase, cytoplasmic K01869 LARS; leucyl-tRNA synthetase [EC:6.1.1.4]
D 123801401 DARS2; aspartate--tRNA ligase, mitochondrial isoform X1 K01876 DARS2; aspartyl-tRNA synthetase [EC:6.1.1.12]
D 123789106 GARS1; glycine--tRNA ligase K01880 GARS; glycyl-tRNA synthetase [EC:6.1.1.14]
D 123776174 RARS2; probable arginine--tRNA ligase, mitochondrial isoform X1 K01887 RARS; arginyl-tRNA synthetase [EC:6.1.1.19]
D 123788575 RARS1; arginine--tRNA ligase, cytoplasmic K01887 RARS; arginyl-tRNA synthetase [EC:6.1.1.19]
D 123798514 QRSL1; LOW QUALITY PROTEIN: glutamyl-tRNA(Gln) amidotransferase subunit A, mitochondrial K02433 gatA; aspartyl-tRNA(Asn)/glutamyl-tRNA(Gln) amidotransferase subunit A [EC:6.3.5.6 6.3.5.7]
D 123793218 GATB; glutamyl-tRNA(Gln) amidotransferase subunit B, mitochondrial isoform X1 K02434 gatB; aspartyl-tRNA(Asn)/glutamyl-tRNA(Gln) amidotransferase subunit B [EC:6.3.5.6 6.3.5.7]
D 123788279 GATC; glutamyl-tRNA(Gln) amidotransferase subunit C, mitochondrial K02435 gatC; aspartyl-tRNA(Asn)/glutamyl-tRNA(Gln) amidotransferase subunit C [EC:6.3.5.6 6.3.5.7]
D 123802258 poly(A) RNA polymerase, mitochondrial K18060 MTPAP; poly(A) RNA polymerase, mitochondrial [EC:2.7.7.19]
D 123798783 PDE12; 2',5'-phosphodiesterase 12 K19612 PDE12; 2',5'-phosphodiesterase [EC:3.1.13.4 3.1.4.-]
D 123800007 MTG1; mitochondrial ribosome-associated GTPase 1 isoform X1 K19828 MTG1; mitochondrial GTPase 1
D 123804061 NOA1; nitric oxide-associated protein 1 K19832 NOA1; nitric oxide-associated protein 1
D 123784134 NSUN3; tRNA (cytosine(34)-C(5))-methyltransferase, mitochondrial isoform X1 K21969 NSUN3; tRNA (cytosine34-C5)-methyltransferase [EC:2.1.1.-]
D 123793741 NSUN4; 5-methylcytosine rRNA methyltransferase NSUN4 K21970 NSUN4; 5-methylcytosine rRNA methyltransferase NSUN4 [EC:2.1.1.-]
D 123799194 RPUSD3; mitochondrial mRNA pseudouridine synthase RPUSD3 isoform X1 K22537 RPUSD3; mitochondrial RNA pseudouridine synthase RPUSD3 [EC:5.4.99.-]
D 123792721 RPUSD4; mitochondrial RNA pseudouridine synthase RPUSD4 K22538 RPUSD4; mitochondrial RNA pseudouridine synthase RPUSD4 [EC:5.4.99.-]
D 123784722 RCC1L; RCC1-like G exchanging factor-like protein isoform X1 K23495 RCC1L; RCC1-like G exchanging factor-like protein
D 123802397 NGRN; neugrin K23496 NGRN; neugrin
D 123775659 TRUB2; mitochondrial mRNA pseudouridine synthase TRUB2 K23497 TRUB2; mitochondrial mRNA pseudouridine synthase TRUB2 [EC:5.4.99.-]
D 123783211 FASTKD1; FAST kinase domain-containing protein 1, mitochondrial K27754 FASTKD1; FAST kinase domain-containing protein 1
D 123798244 FASTKD2; FAST kinase domain-containing protein 2, mitochondrial K18190 FASTKD2; FAST kinase domain-containing protein 2
D 123791265 FASTKD3; FAST kinase domain-containing protein 3, mitochondrial isoform X1 K27755 FASTKD3; FAST kinase domain-containing protein 3
D 123789478 TBRG4; FAST kinase domain-containing protein 4 K27756 TBRG4; FAST kinase domain-containing protein 4
D 123799136 FASTKD5; FAST kinase domain-containing protein 5, mitochondrial K27757 FASTKD5; FAST kinase domain-containing protein 5
D 123779784 MTRFR; mitochondrial translation release factor in rescue K23498 COXPD7; peptide chain release factor
D 123797457 RMND1; required for meiotic nuclear division protein 1 homolog isoform X1 K23499 RMND1; required for meiotic nuclear division protein 1
D 123786898 GRSF1; LOW QUALITY PROTEIN: G-rich sequence factor 1 K24984 GRSF1; G-rich sequence factor 1
D 123799465 METTL15; 12S rRNA N4-methylcytidine (m4C) methyltransferase isoform X1 K25876 METTL15; 12S rRNA N4-methylcytidine (m4C) methyltransferase [EC:2.1.1.-]
D 123794701 MYG1; MYG1 exonuclease K25877 MYG1; MYG1 exonuclease [EC:3.1.-.-]
D 123798508 MTRES1; mitochondrial transcription rescue factor 1 isoform X1 K26186 MTRES1; mitochondrial transcription rescue factor 1
B Mitochondrial DNA replication factors
C Mitochondrial DNA polymerase
D 123802378 POLG; DNA polymerase subunit gamma-1 isoform X1 K02332 POLG; DNA polymerase gamma 1 [EC:2.7.7.7]
D 123803238 POLG2; DNA polymerase subunit gamma-2, mitochondrial K02333 POLG2; DNA polymerase gamma 2
D 123784519 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 123775761 ENDOG; endonuclease G, mitochondrial K01173 ENDOG; endonuclease G, mitochondrial
D 123800041 MGME1; mitochondrial genome maintenance exonuclease 1 K19465 MGME1; mitochondrial genome maintenance exonuclease 1 [EC:3.1.-.-]
D 123792829 EXOG; nuclease EXOG, mitochondrial K15050 EXOG; nuclease EXOG, mitochondrial
D 123795875 TWNK; twinkle mtDNA helicase isoform X1 K17680 PEO1; twinkle protein [EC:5.6.2.3]
D 123800791 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 123788864 SSBP1; single-stranded DNA-binding protein, mitochondrial K03111 ssb; single-strand DNA-binding protein
D 123796106 ATPase family AAA domain-containing protein 3 K17681 ATAD3A_B; ATPase family AAA domain-containing protein 3A/B
D 123790910 ubiquinol-cytochrome-c reductase complex assembly factor 2 K17682 MNF1; mitochondrial nucleoid factor 1
D 123803178 CUNH17orf80; uncharacterized protein C17orf80 homolog K27759 MTNAP1; mitochondrial nucleoid-associated protein 1
D 123788788 GTPase IMAP family member 5-like K27758 GIMAP1_3_5; GTPase IMAP family member 1/3/5
D 123788792 GTPase IMAP family member 5-like isoform X1 K27758 GIMAP1_3_5; GTPase IMAP family member 1/3/5
D 123788796 GTPase IMAP family member 1-like K27758 GIMAP1_3_5; GTPase IMAP family member 1/3/5
D 123789519 GIMAP1; GTPase IMAP family member 1 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 123776631 DNAJA2; dnaJ homolog subfamily A member 2 K09503 DNAJA2; DnaJ homolog subfamily A member 2
D 123797027 TOMM34; mitochondrial import receptor subunit TOM34 K17766 TOM34; mitochondrial import receptor subunit TOM34
D 123776840 RHOT1; mitochondrial Rho GTPase 1 isoform X1 K07870 RHOT1; mitochondrial Rho GTPase 1 [EC:3.6.5.-]
D 123783522 HSPA6; LOW QUALITY PROTEIN: heat shock 70 kDa protein 6 K03283 HSPA1_6_8; heat shock 70kDa protein 1/6/8
D 123790987 heat shock 70 kDa protein 1 K03283 HSPA1_6_8; heat shock 70kDa protein 1/6/8
D 123790988 heat shock 70 kDa protein 1 K03283 HSPA1_6_8; heat shock 70kDa protein 1/6/8
D 123790989 heat shock 70 kDa protein 1-like K03283 HSPA1_6_8; heat shock 70kDa protein 1/6/8
D 123792681 HSPA8; heat shock cognate 71 kDa protein K03283 HSPA1_6_8; heat shock 70kDa protein 1/6/8
D 123778506 AH receptor-interacting protein isoform X1 K17767 AIP; AH receptor-interacting protein
D 123797103 AIPL1; aryl-hydrocarbon-interacting protein-like 1 K17767 AIP; AH receptor-interacting protein
D 123787684 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 123781245 TOMM5; mitochondrial import receptor subunit TOM5 homolog K17773 TOM5; mitochondrial import receptor subunit TOM5
D 123779152 TOMM6; mitochondrial import receptor subunit TOM6 homolog K17772 TOM6; mitochondrial import receptor subunit TOM6
D 123789181 TOMM7; mitochondrial import receptor subunit TOM7 homolog isoform X1 K17771 TOM7; mitochondrial import receptor subunit TOM7
D 123795178 mitochondrial import receptor subunit TOM7 homolog K17771 TOM7; mitochondrial import receptor subunit TOM7
D 123782919 TOMM20; mitochondrial import receptor subunit TOM20 homolog K17770 TOM20; mitochondrial import receptor subunit TOM20
D 123800663 TOMM20L; TOMM20-like protein 1 K17770 TOM20; mitochondrial import receptor subunit TOM20
D 123780694 TOMM22; mitochondrial import receptor subunit TOM22 homolog isoform X1 K17769 TOM22; mitochondrial import receptor subunit TOM22
D 123783567 TOMM40L; mitochondrial import receptor subunit TOM40B K11518 TOM40; mitochondrial import receptor subunit TOM40
D 123802247 TOMM40; mitochondrial import receptor subunit TOM40 homolog K11518 TOM40; mitochondrial import receptor subunit TOM40
D 123784109 TOMM70; mitochondrial import receptor subunit TOM70 K17768 TOM70; mitochondrial import receptor subunit TOM70
C TOB/SAM complex
D 123780525 SAMM50; sorting and assembly machinery component 50 homolog K07277 SAM50; outer membrane protein insertion porin family
C Porin
D 123790351 VDAC1; voltage-dependent anion-selective channel protein 1 K05862 VDAC1; voltage-dependent anion channel protein 1
D 123782868 VDAC2; voltage-dependent anion-selective channel protein 2 K15040 VDAC2; voltage-dependent anion channel protein 2
D 123788811 voltage-dependent anion-selective channel protein 2 K15040 VDAC2; voltage-dependent anion channel protein 2
D 123780315 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 123783428 MTX1; metaxin-1 K17776 MTX; metaxin
D 123791531 MTX3; metaxin-3 isoform X1 K17776 MTX; metaxin
D 123798072 MTX2; metaxin-2 K17776 MTX; metaxin
D 123777035 MYO19; unconventional myosin-XIX isoform X1 K25838 MYO19; myosin XIX
D 123794082 ARMCX1; armadillo repeat-containing X-linked protein 1 K26187 ARMCX1; armadillo repeat-containing X-linked protein 1
D 123794084 ARMCX2; armadillo repeat-containing X-linked protein 2 K26188 ARMCX2; armadillo repeat-containing X-linked protein 2
D 123794086 ARMCX3; armadillo repeat-containing X-linked protein 3 K26189 ARMCX3; armadillo repeat-containing X-linked protein 3
D 123794087 ARMCX6; protein ARMCX6 K26190 ARMCX6; armadillo repeat-containing X-linked protein 6
D 123800122 MTARC2; mitochondrial amidoxime reducing component 2 K27318 MTARC; mitochondrial amidoxime-reducing component [EC:1.7.-.-]
D 123800123 MTARC1; mitochondrial amidoxime-reducing component 1 isoform X1 K27318 MTARC; mitochondrial amidoxime-reducing component [EC:1.7.-.-]
B Inter membrane space
C Transporter inner membrane (TIM) 9/10 complex
D 123783075 mitochondrial import inner membrane translocase subunit Tim9-like K17777 TIM9; mitochondrial import inner membrane translocase subunit TIM9
D 123800664 TIMM9; mitochondrial import inner membrane translocase subunit Tim9 K17777 TIM9; mitochondrial import inner membrane translocase subunit TIM9
D 123785056 TIMM10; mitochondrial import inner membrane translocase subunit Tim10 K17778 TIM10; mitochondrial import inner membrane translocase subunit TIM10
D 123782788 TIMM10B; mitochondrial import inner membrane translocase subunit Tim10 B isoform X1 K17779 TIM10B; mitochondrial import inner membrane translocase subunit TIM10B
C TIM8/13 complex
D 123792573 TIMM8B; mitochondrial import inner membrane translocase subunit Tim8 B K17780 TIM8; mitochondrial import inner membrane translocase subunit TIM8
D 123793928 TIMM8A; mitochondrial import inner membrane translocase subunit Tim8 A K17780 TIM8; mitochondrial import inner membrane translocase subunit TIM8
D 123793988 TIMM13; mitochondrial import inner membrane translocase subunit Tim13 isoform X1 K17781 TIM13; mitochondrial import inner membrane translocase subunit TIM13
C Mitochondrial intermembrane space assembly (MIA) complex
D 123802062 CHCHD4; mitochondrial intermembrane space import and assembly protein 40 K17782 MIA40; mitochondrial intermembrane space import and assembly protein 40
D 123783787 GFER; FAD-linked sulfhydryl oxidase ALR K17783 ERV1; mitochondrial FAD-linked sulfhydryl oxidase [EC:1.8.3.2]
B Inner mambrane
C MICOS complex
D 123782208 MICOS10; MICOS complex subunit MIC10 K17784 MICOS10; MICOS complex subunit MIC10
D 123800718 LOW QUALITY PROTEIN: MICOS complex subunit MIC10-like K17784 MICOS10; MICOS complex subunit MIC10
D 123799697 IMMT; MICOS complex subunit MIC60 isoform X1 K17785 IMMT; MICOS complex subunit MIC60
D 123779008 MICOS13; MICOS complex subunit MIC13 K24624 MICOS13; MICOS complex subunit MIC13
D 123788938 CHCHD3; MICOS complex subunit MIC19 K17563 CHCHD3; MICOS complex subunit MIC19
D 123801966 CHCHD6; MICOS complex subunit MIC25 isoform X1 K17564 CHCHD6; MICOS complex subunit MIC25
D 123797237 APOO; MICOS complex subunit MIC26 K24625 APOO; MICOS complex subunit MIC26
D 123793940 APOOL; LOW QUALITY PROTEIN: MICOS complex subunit MIC27 K24626 APOOL; MICOS complex subunit MIC27
C MICOS complex (yeast)
D 123782208 MICOS10; MICOS complex subunit MIC10 K17784 MICOS10; MICOS complex subunit MIC10
D 123800718 LOW QUALITY PROTEIN: MICOS complex subunit MIC10-like K17784 MICOS10; MICOS complex subunit MIC10
D 123799697 IMMT; MICOS complex subunit MIC60 isoform X1 K17785 IMMT; MICOS complex subunit MIC60
C TIM22 complex
D 123776930 TIMM22; mitochondrial import inner membrane translocase subunit Tim22 K17790 TIM22; mitochondrial import inner membrane translocase subunit TIM22
C TIM23 complex
D 123800275 mitochondrial import inner membrane translocase subunit Tim23 isoform X1 K17794 TIM23; mitochondrial import inner membrane translocase subunit TIM23
D 123785748 TIMM17A; mitochondrial import inner membrane translocase subunit Tim17-A K17795 TIM17; mitochondrial import inner membrane translocase subunit TIM17
D 123787476 TIMM21; mitochondrial import inner membrane translocase subunit Tim21 K17796 TIM21; mitochondrial import inner membrane translocase subunit TIM21
D 123784178 TIMM50; mitochondrial import inner membrane translocase subunit TIM50 K17496 TIM50; mitochondrial import inner membrane translocase subunit TIM50
C Mitochondrial calcium uniporter complex
D 123782996 MCU; calcium uniporter protein, mitochondrial K20858 MCU; calcium uniporter protein, mitochondrial
D 123783012 MICU1; calcium uptake protein 1, mitochondrial isoform X1 K22827 MICU1; calcium uptake protein 1, mitochondrial
D 123791978 MICU2; calcium uptake protein 2, mitochondrial isoform X1 K22828 MICU2; calcium uptake protein 2, mitochondrial
D 123793365 MCUB; calcium uniporter regulatory subunit MCUb, mitochondrial isoform X1 K22829 MCUB; calcium uniporter regulatory subunit MCUb, mitochondrial
D 123780548 SMDT1; essential MCU regulator, mitochondrial K22830 SMDT1; essential MCU regulator, mitochondrial
D 123786580 LOW QUALITY PROTEIN: mitochondrial calcium uniporter regulator 1-like K22137 MCUR1; mitochondrial calcium uniporter regulator 1
D 123801501 MCUR1; mitochondrial calcium uniporter regulator 1 isoform X1 K22137 MCUR1; mitochondrial calcium uniporter regulator 1
C Other inner membrane factors
D 123777828 LETM1; mitochondrial proton/calcium exchanger protein K17800 LETM1; LETM1 and EF-hand domain-containing protein 1, mitochondrial
D 123799455 IMMP1L; mitochondrial inner membrane protease subunit 1 isoform X1 K09647 IMP1; mitochondrial inner membrane protease subunit 1 [EC:3.4.21.-]
D 123789057 IMMP2L; mitochondrial inner membrane protease subunit 2 isoform X1 K09648 IMP2; mitochondrial inner membrane protease subunit 2 [EC:3.4.21.-]
D 123803834 TMEM126A; transmembrane protein 126A isoform X1 K18157 TMEM126A; transmembrane protein 126A
D 123794544 MPC1L; mitochondrial pyruvate carrier 1-like protein K22138 MPC1; mitochondrial pyruvate carrier 1
D 123797527 MPC1; mitochondrial pyruvate carrier 1 K22138 MPC1; mitochondrial pyruvate carrier 1
D 123792182 MPC2; mitochondrial pyruvate carrier 2 K22139 MPC2; mitochondrial pyruvate carrier 2
D 123795634 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 123803995 OCIAD1; OCIA domain-containing protein 1 isoform X1 K28215 OCIAD1; OCIA domain-containing protein 1
B Matrix
C MMC/PAM proteins
D 123777606 TIMM44; mitochondrial import inner membrane translocase subunit TIM44 K17804 TIM44; mitochondrial import inner membrane translocase subunit TIM44
D 123790302 HSPA9; stress-70 protein, mitochondrial K04043 dnaK; molecular chaperone DnaK
D 123787075 GRPEL1; grpE protein homolog 1, mitochondrial K03687 GRPE; molecular chaperone GrpE
D 123803657 GRPEL2; grpE protein homolog 2, mitochondrial K03687 GRPE; molecular chaperone GrpE
D 123786425 DNAJC19; mitochondrial import inner membrane translocase subunit TIM14 K09539 DNAJC19; DnaJ homolog subfamily C member 19
D 123787100 LOW QUALITY PROTEIN: mitochondrial import inner membrane translocase subunit TIM14-like K09539 DNAJC19; DnaJ homolog subfamily C member 19
D 123783694 PAM16; mitochondrial import inner membrane translocase subunit TIM16 isoform X1 K17805 PAM16; mitochondrial import inner membrane translocase subunit TIM16
C Other matrix factors
D 123796351 PMPCA; mitochondrial-processing peptidase subunit alpha isoform X1 K01412 PMPCA; mitochondrial-processing peptidase subunit alpha [EC:3.4.24.64]
D 123789376 PMPCB; mitochondrial-processing peptidase subunit beta K17732 PMPCB; mitochondrial-processing peptidase subunit beta [EC:3.4.24.64]
D 123791971 mitochondrial intermediate peptidase isoform X1 K01410 MIPEP; mitochondrial intermediate peptidase [EC:3.4.24.59]
D 123798166 HSPD1; 60 kDa heat shock protein, mitochondrial K04077 groEL; chaperonin GroEL [EC:5.6.1.7]
D 123783331 DNAJA3; dnaJ homolog subfamily A member 3, mitochondrial isoform X1 K09504 DNAJA3; DnaJ homolog subfamily A member 3
D 123802023 TAMM41; phosphatidate cytidylyltransferase, mitochondrial isoform X1 K17807 TAM41; mitochondrial translocator assembly and maintenance protein 41
D 123796356 DNLZ; DNL-type zinc finger protein K17808 ZIM17; mitochondrial protein import protein ZIM17
D 123777835 ETHE1; persulfide dioxygenase ETHE1, mitochondrial isoform X1 K17725 ETHE1; sulfur dioxygenase [EC:1.13.11.18]
D 123776893 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 123798247 CREB1; cyclic AMP-responsive element-binding protein 1 isoform X1 K05870 CREB1; cyclic AMP-responsive element-binding protein 1
D 123800192 GFM2; ribosome-releasing factor 2, mitochondrial isoform X1 K02355 fusA; elongation factor G
D 123801629 GFM1; elongation factor G, mitochondrial K02355 fusA; elongation factor G
D 123794698 SP1; transcription factor Sp1 K04684 SP1; transcription factor Sp1
D 123778160 YY1; transcriptional repressor protein YY1 K09201 YY; transcription factor YY
D 123787724 GABPB2; GA-binding protein subunit beta-2 isoform X1 K09454 GABPB; GA-binding protein transcription factor, beta
D 123801791 GABPB1; GA-binding protein subunit beta-1 isoform X1 K09454 GABPB; GA-binding protein transcription factor, beta
D 123782931 TFAM; transcription factor A, mitochondrial K11830 TFAM; transcription factor A, mitochondrial
C Nuclear respiratory factors
D 123788962 NRF1; nuclear respiratory factor 1 isoform X1 K11831 NRF1; nuclear respiratory factor 1
D 123798077 NFE2L2; nuclear factor erythroid 2-related factor 2 isoform X1 K05638 NFE2L2; nuclear factor erythroid 2-related factor 2
D 123798427 metallothionein-2 K14739 MT1_2; metallothionein 1/2
D 123798428 metallothionein-1 K14739 MT1_2; metallothionein 1/2
D 123798429 metallothionein-1 K14739 MT1_2; metallothionein 1/2
D 123800247 metallothionein-2E-like K14739 MT1_2; metallothionein 1/2
D 123798366 metallothionein-3 K14740 MT3; metallothionein 3
D 123798365 MT4; metallothionein-4 K14741 MT4; metallothionein 4
C Coactivators
D 123775818 PPARGC1A; peroxisome proliferator-activated receptor gamma coactivator 1-alpha isoform X1 K07202 PPARGC1A; alpha peroxisome proliferator-activated receptor gamma coactivator 1-alpha
D 123803693 PPARGC1B; peroxisome proliferator-activated receptor gamma coactivator 1-beta isoform X1 K17962 PPARGC1B; peroxisome proliferator-activated receptor gamma coactivator 1-beta
D 123795897 PPRC1; peroxisome proliferator-activated receptor gamma coactivator-related protein 1 isoform X1 K17963 PPRC1; peroxisome proliferator-activated receptor gamma coactivator-related protein 1
D 123779850 CRTC1; CREB-regulated transcription coactivator 1 isoform X1 K15309 CRTC1; CREB-regulated transcription coactivator 1
D 123780918 EP300; histone acetyltransferase p300 isoform X1 K04498 EP300; E1A/CREB-binding protein [EC:2.3.1.48]
D 123783593 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 123797824 NFS1; cysteine desulfurase, mitochondrial isoform X1 K04487 iscS; cysteine desulfurase [EC:2.8.1.7]
D 123788231 ISCU; iron-sulfur cluster assembly enzyme ISCU, mitochondrial K22068 ISCU; iron-sulfur cluster assembly enzyme ISCU, mitochondrial
D 123801553 LYRM4; LYR motif-containing protein 4 K22069 LYRM4; LYR motif-containing protein 4
D 123776485 FXN; frataxin, mitochondrial K19054 FXN; frataxin [EC:1.16.3.1]
D 123799513 LOW QUALITY PROTEIN: frataxin, mitochondrial-like K19054 FXN; frataxin [EC:1.16.3.1]
D 123792548 FDX1; adrenodoxin, mitochondrial K22070 FDX1; ferredoxin-1, mitochondrial
D 123777562 FDX2; ferredoxin-2, mitochondrial K22071 FDX2; ferredoxin-2, mitochondrial
D 123803103 FDXR; NADPH:adrenodoxin oxidoreductase, mitochondrial isoform X1 K18914 FDXR; adrenodoxin-NADP+ reductase [EC:1.18.1.6]
D 123790302 HSPA9; stress-70 protein, mitochondrial K04043 dnaK; molecular chaperone DnaK
D 123788137 HSCB; iron-sulfur cluster co-chaperone protein HscB isoform X1 K04082 hscB; molecular chaperone HscB
D 123790300 SIL1; nucleotide exchange factor SIL1 isoform X1 K14001 SIL1; nucleotide exchange factor SIL1
D 123803937 GLRX5; glutaredoxin-related protein 5, mitochondrial K07390 grxD; monothiol glutaredoxin
D 123781460 ISCA1; iron-sulfur cluster assembly 1 homolog, mitochondrial K22063 ISCA1; iron-sulfur cluster assembly 1
D 123798986 ISCA2; iron-sulfur cluster assembly 2 homolog, mitochondrial isoform X1 K22072 ISCA2; iron-sulfur cluster assembly 2
D 123796500 IBA57; putative transferase CAF17, mitochondrial isoform X1 K22073 IBA57; transferase CAF17, mitochondrial [EC:2.1.-.-]
D 123786019 NFU1; NFU1 iron-sulfur cluster scaffold homolog, mitochondrial isoform X1 K22074 NFU1; NFU1 iron-sulfur cluster scaffold homolog, mitochondrial
D 123779207 BOLA1; bolA-like protein 1 K22066 BOLA1; BolA-like protein 1
D 123786067 BOLA3; bolA-like protein 3 K22075 BOLA3; BolA-like protein 3
D 123795879 SFXN3; sideroflexin-3 isoform X1 K23500 SFXN1_3; sideroflexin-1/3
D 123796176 SFXN1; sideroflexin-1 isoform X1 K23500 SFXN1_3; sideroflexin-1/3
D 123795913 SFXN2; sideroflexin-2 K23501 SFXN2; sideroflexin-2
D 123796006 SFXN4; sideroflexin-4 isoform X1 K23502 SFXN4; sideroflexin-4
C Other regulator of mitochondrial biogenesis
D 123778360 CPT1A; carnitine O-palmitoyltransferase 1, liver isoform isoform X1 K08765 CPT1A; carnitine O-palmitoyltransferase 1, liver isoform [EC:2.3.1.21]
D 123803781 CPT1B; carnitine O-palmitoyltransferase 1, muscle isoform K19523 CPT1B; carnitine O-palmitoyltransferase 1, muscle isoform [EC:2.3.1.21]
D 123776346 CPT1C; carnitine O-palmitoyltransferase 1, brain isoform K19524 CPT1C; carnitine O-palmitoyltransferase 1, brain isoform [EC:2.3.1.21]
D 123789980 DHTKD1; probable 2-oxoglutarate dehydrogenase E1 component DHKTD1, mitochondrial isoform X1 K15791 DHKTD1; 2-oxoadipate dehydrogenase E1 component [EC:1.2.4.-]
D 123798538 FBXL4; F-box/LRR-repeat protein 4 isoform X1 K10270 FBXL4; F-box and leucine-rich repeat protein 4
D 123786235 HCFC1; host cell factor 1 K14966 HCFC1; host cell factor 1
D 123780564 HCFC2; host cell factor 2 isoform X1 K27390 HCFC2; host cell factor 2
D 123775561 HTT; huntingtin K04533 HD; huntingtin
D 123778305 ESRRA; steroid hormone receptor ERR1 K08552 NR3B1; estrogen-related receptor alpha
D 123798955 ESRRB; steroid hormone receptor ERR2 isoform X1 K08553 NR3B2; estrogen-related receptor beta
D 123800107 ESRRG; estrogen-related receptor gamma isoform X1 K08554 NR3B3; estrogen-related receptor gamma
D 123784159 NRIP1; nuclear receptor-interacting protein 1 K17965 NRIP1; nuclear receptor-interacting protein 1
D 123785508 PRKAA1; 5'-AMP-activated protein kinase catalytic subunit alpha-1 isoform X1 K07198 PRKAA; 5'-AMP-activated protein kinase, catalytic alpha subunit [EC:2.7.11.31]
D 123795384 PRKAA2; 5'-AMP-activated protein kinase catalytic subunit alpha-2 isoform X1 K07198 PRKAA; 5'-AMP-activated protein kinase, catalytic alpha subunit [EC:2.7.11.31]
D 123778997 LONP1; LOW QUALITY PROTEIN: lon protease homolog, mitochondrial K08675 PRSS15; ATP-dependent Lon protease [EC:3.4.21.53]
D 123782872 SIRT1; NAD-dependent protein deacetylase sirtuin-1 isoform X1 K11411 SIRT1; NAD-dependent protein deacetylase sirtuin 1 [EC:2.3.1.286]
D 123797489 SERAC1; protein SERAC1 isoform X1 K23504 SERAC1; protein SERAC1
D 123787941 UXT; protein UXT K26537 UXT; protein UXT
D 123792775 PERM1; PGC-1 and ERR-induced regulator in muscle protein 1 K26738 PERM1; PGC-1 and ERR-induced regulator in muscle protein 1
D 123777474 GATD3; glutamine amidotransferase-like class 1 domain-containing protein 3, mitochondrial isoform X1 K27835 GATD3; glutamine amidotransferase-like class 1 domain-containing protein 3, mitochondrial
D 123803551 TANGO2; transport and Golgi organization protein 2 homolog isoform X1 K28098 TANGO2; transport and Golgi organization protein 2
D 123798177 CUNH2orf69; mitochondrial protein C2orf69 homolog K28271 C2ORF69; mitochondrial protein C2orf69
D 123781106 FAM214B; protein FAM214B K28528 ATOS; protein Atossa
D 123801862 FAM214A; protein FAM214A isoform X1 K28528 ATOS; protein Atossa
D 123781736 DDX47; probable ATP-dependent RNA helicase DDX47 isoform X1 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 123775669 NDUFAF1; complex I intermediate-associated protein 30, mitochondrial K18159 NDUFAF1; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex assembly factor 1
D 123790167 NDUFAF2; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex assembly factor 2 K18160 NDUFAF2; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex assembly factor 2
D 123780020 NDUFAF3; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex assembly factor 3 K09008 NDUFAF3; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex assembly factor 3
D 123798542 NDUFAF4; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex assembly factor 4 K18161 NDUFAF4; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex assembly factor 4
D 123799926 NDUFAF5; arginine-hydroxylase NDUFAF5, mitochondrial isoform X1 K18162 NDUFAF5; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex assembly factor 5 [EC:2.1.1.-]
D 123797868 NDUFAF6; NADH dehydrogenase (ubiquinone) complex I, assembly factor 6 isoform X1 K18163 NDUFAF6; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex assembly factor 6
D 123799830 NDUFAF7; protein arginine methyltransferase NDUFAF7, mitochondrial K18164 NDUFAF7; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex assembly factor 7
D 123803849 TMEM126B; complex I assembly factor TMEM126B, mitochondrial isoform X1 K18165 TMEM126B; complex I assembly factor TMEM126B
D 123777708 ECSIT; evolutionarily conserved signaling intermediate in Toll pathway, mitochondrial isoform X1 K04405 ECSIT; evolutionarily conserved signaling intermediate in Toll pathway
D 123801978 ACAD9; complex I assembly factor ACAD9, mitochondrial K15980 ACAD9; acyl-CoA dehydrogenase family member 9 [EC:1.3.99.-]
D 123795706 NUBPL; iron-sulfur protein NUBPL isoform X1 K03593 mrp; ATP-binding protein involved in chromosome partitioning
D 123792725 FOXRED1; FAD-dependent oxidoreductase domain-containing protein 1 isoform X1 K18166 FOXRED1; FAD-dependent oxidoreductase domain-containing protein 1
D 123798515 RTN4IP1; reticulon-4-interacting protein 1, mitochondrial isoform X1 K23164 RTN4IP1; reticulon-4-interacting protein 1, mitochondrial
D 123784032 TIMMDC1; complex I assembly factor TIMMDC1, mitochondrial K23505 TIMMDC1; complex I assembly factor TIMMDC1
C Complex-II assembly factors
D 123793909 SDHAF1; succinate dehydrogenase assembly factor 1, mitochondrial K18167 SDHAF1; succinate dehydrogenase assembly factor 1
D 123785118 SDHAF2; succinate dehydrogenase assembly factor 2, mitochondrial isoform X1 K18168 SDHAF2; succinate dehydrogenase assembly factor 2
C Complex-III assembly factors
D 123779425 mitochondrial chaperone BCS1 isoform X1 K08900 BCS1; mitochondrial chaperone BCS1
D 123779777 TTC19; LOW QUALITY PROTEIN: tetratricopeptide repeat protein 19, mitochondrial K18169 TTC19; tetratricopeptide repeat protein 19, mitochondrial
D 123790384 LYRM7; complex III assembly factor LYRM7 K18170 LYRM7; complex III assembly factor LYRM7
D 123797950 ubiquinol-cytochrome-c reductase complex assembly factor 1 isoform X1 K17662 CBP3; cytochrome b pre-mRNA-processing protein 3
D 123790910 ubiquinol-cytochrome-c reductase complex assembly factor 2 K17682 MNF1; mitochondrial nucleoid factor 1
D 123789824 ubiquinol-cytochrome-c reductase complex assembly factor 3 K23165 UQCC3; ubiquinol-cytochrome-c reductase complex assembly factor 3
D 123783794 CUNH16orf91; protein CCSMST1 K27339 UQCC4; ubiquinol-cytochrome c reductase complex assembly factor 4
D 123798744 SMIM4; small integral membrane protein 4 K27340 UQCC5; ubiquinol-cytochrome c reductase complex assembly factor 5
D 123780749 CUNH12orf73; protein BRAWNIN K27341 UQCC6; ubiquinol-cytochrome c reductase complex assembly factor 6
D 123788918 STMP1; short transmembrane mitochondrial protein 1 K27319 STMP1; short transmembrane mitochondrial protein 1
C Complex-IV assembly factors
D 123777683 CMC1; COX assembly mitochondrial protein homolog K18171 CMC1; COX assembly mitochondrial protein 1
D 123791336 CMC2; COX assembly mitochondrial protein 2 homolog K18172 CMC2; COX assembly mitochondrial protein 2
D 123780650 LOW QUALITY PROTEIN: cytochrome c oxidase assembly factor 1 homolog K18173 COA1; cytochrome c oxidase assembly factor 1
D 123789448 cytochrome c oxidase assembly factor 1 homolog K18173 COA1; cytochrome c oxidase assembly factor 1
D 123781840 cytochrome c oxidase assembly factor 3 homolog, mitochondrial K18175 CCDC56; cytochrome c oxidase assembly factor 3, animal type
D 123804182 cytochrome c oxidase assembly factor 4 homolog, mitochondrial isoform X1 K18177 COA4; cytochrome c oxidase assembly factor 4
D 123776042 cytochrome c oxidase assembly factor 5 K18178 COA5; cytochrome c oxidase assembly factor 5
D 123782994 cytochrome c oxidase assembly factor 6 homolog K18179 COA6; cytochrome c oxidase assembly factor 6
D 123793688 cytochrome c oxidase assembly factor 7 K18180 COA7; cytochrome c oxidase assembly factor 7
D 123779781 protoheme IX farnesyltransferase, mitochondrial K02257 COX10; heme o synthase [EC:2.5.1.141]
D 123776786 cytochrome c oxidase assembly protein COX11, mitochondrial K02258 COX11; cytochrome c oxidase assembly protein subunit 11
D 123794759 cytochrome c oxidase assembly protein COX14 K18181 COX14; cytochrome c oxidase assembly factor 14
D 123783570 cytochrome c oxidase assembly protein COX15 homolog isoform X1 K02259 COX15; heme a synthase [EC:1.17.99.9]
D 123799031 cytochrome c oxidase assembly protein COX16 homolog, mitochondrial isoform X1 K18182 COX16; cytochrome c oxidase assembly protein subunit 16
D 123784026 cytochrome c oxidase copper chaperone K02260 COX17; cytochrome c oxidase assembly protein subunit 17
D 123786975 cytochrome c oxidase assembly protein COX18, mitochondrial isoform X1 K17797 COX18; mitochondrial inner membrane protein COX18
D 123802548 cytochrome c oxidase assembly protein COX19 K18183 COX19; cytochrome c oxidase assembly protein subunit 19
D 123791450 cytochrome c oxidase assembly protein COX20, mitochondrial K18184 COX20; cytochrome c oxidase assembly protein subunit 20
D 123782831 MSS51; putative protein MSS51 homolog, mitochondrial isoform X1 K17656 MSS51; mitochondrial splicing suppressor protein 51
D 123777557 protein PET100 homolog, mitochondrial isoform X1 K18186 PET100; protein PET100, animal type
D 123800061 protein PET117 homolog, mitochondrial K18188 PET117; protein PET117
D 123795804 OXA1L; mitochondrial inner membrane protein OXA1L K03217 yidC; YidC/Oxa1 family membrane protein insertase
D 123775622 surfeit locus protein 1 isoform X1 K14998 SURF1; surfeit locus 1 family protein
D 123792078 protein SCO1 homolog, mitochondrial K07152 SCO1; protein SCO1
D 123803769 protein SCO2 homolog, mitochondrial K23755 SCO2; protein SCO2
D 123803065 translational activator of cytochrome c oxidase 1 isoform X1 K18189 TACO1; translational activator of cytochrome c oxidase 1
D 123776073 LRPPRC; leucine-rich PPR motif-containing protein, mitochondrial K17964 LRPPRC; leucine-rich PPR motif-containing protein, mitochondrial
D 123779247 cytochrome c oxidase assembly factor 8 K23506 APOPT1; apoptogenic protein 1
D 123788918 STMP1; short transmembrane mitochondrial protein 1 K27319 STMP1; short transmembrane mitochondrial protein 1
D 123798744 SMIM4; small integral membrane protein 4 K27340 UQCC5; ubiquinol-cytochrome c reductase complex assembly factor 5
D 123789809 TMEM223; transmembrane protein 223 K27342 TMEM223; transmembrane protein 223
D 123778634 HIGD1A; HIG1 domain family member 1A, mitochondrial K28216 HIGD1A_C; HIG1 domain family member 1A/C, mitochondrial
D 123794747 HIGD1C; HIG1 domain family member 1C K28216 HIGD1A_C; HIG1 domain family member 1A/C, mitochondrial
D 123795880 HIGD2A; HIG1 domain family member 2A, mitochondrial K28217 HIGD2A; HIG1 domain family member 2A, mitochondrial
C Complex-V assembly factors
D 123793734 ATPAF1; ATP synthase mitochondrial F1 complex assembly factor 1 isoform X1 K07555 ATPeAF1; ATP synthase mitochondrial F1 complex assembly factor 1
D 123779737 ATPAF2; ATP synthase mitochondrial F1 complex assembly factor 2 isoform X1 K07556 ATPeAF2; ATP synthase mitochondrial F1 complex assembly factor 2
D 123800467 TMEM70; transmembrane protein 70, mitochondrial K17966 TMEM70; transmembrane protein 70, mitochondrial
D 123778657 ATP5MJ; ATP synthase subunit ATP5MJ, mitochondrial K18193 MP68; 6.8 kDa mitochondrial proteolipid
D 123783302 ATP synthase subunit ATP5MJ, mitochondrial-like K18193 MP68; 6.8 kDa mitochondrial proteolipid
D 123784573 ATP synthase subunit ATP5MJ, mitochondrial-like K18193 MP68; 6.8 kDa mitochondrial proteolipid
D 123792564 ATP synthase subunit ATP5MJ, mitochondrial-like K18193 MP68; 6.8 kDa mitochondrial proteolipid
D 123783113 LOW QUALITY PROTEIN: ATP synthase membrane subunit K, mitochondrial-like K18194 USMG5; up-regulated during skeletal muscle growth protein 5
D 123795925 ATP5MK; ATP synthase membrane subunit K, mitochondrial K18194 USMG5; up-regulated during skeletal muscle growth protein 5
B Mitochondrial dynamics
C Fission and Fusion factors
D 123786410 MFN1; mitofusin-1 K21356 MFN1; mitofusin 1 [EC:3.6.5.-]
D 123779618 MFN2; mitofusin-2 K06030 MFN2; mitofusin 2 [EC:3.6.5.-]
D 123802205 OPA1; dynamin-like 120 kDa protein, mitochondrial isoform X1 K17079 OPA1; optic atrophy protein 1 [EC:3.6.5.5]
D 123799488 OPA3; optic atrophy 3 protein isoform X1 K23166 OPA3; optic atrophy 3 protein
D 123788282 TRIAP1; TP53-regulated inhibitor of apoptosis 1 K17968 TRIAP1; TRIAP1/MDM35 family protein
D 123801083 TP53-regulated inhibitor of apoptosis 1-like K17968 TRIAP1; TRIAP1/MDM35 family protein
D 123778931 PARL; presenilins-associated rhomboid-like protein, mitochondrial K09650 PARL; rhomboid-like protein [EC:3.4.21.105]
D 123790912 BAK1; bcl-2 homologous antagonist/killer K14021 BAK; Bcl-2 homologous antagonist/killer
D 123776228 BAX; apoptosis regulator BAX isoform X1 K02159 BAX; apoptosis regulator BAX
D 123801708 DNM1L; dynamin-1-like protein isoform X1 K17065 DNM1L; dynamin 1-like protein [EC:3.6.5.5]
D 123777283 FIS1; mitochondrial fission 1 protein K17969 FIS1; mitochondrial fission 1 protein
D 123779138 SUMO3; small ubiquitin-related modifier 3 K12160 SUMO; small ubiquitin-related modifier
D 123793458 small ubiquitin-related modifier 2-like K12160 SUMO; small ubiquitin-related modifier
D 123796266 small ubiquitin-related modifier 1-like K12160 SUMO; small ubiquitin-related modifier
D 123796973 small ubiquitin-related modifier 2-like K12160 SUMO; small ubiquitin-related modifier
D 123798212 SUMO1; small ubiquitin-related modifier 1 K12160 SUMO; small ubiquitin-related modifier
D 123800683 small ubiquitin-related modifier 2-like K12160 SUMO; small ubiquitin-related modifier
D 123803046 SUMO2; small ubiquitin-related modifier 2 K12160 SUMO; small ubiquitin-related modifier
D 123795252 SH3GLB1; endophilin-B1 isoform X1 K11248 SH3GLB1; endophilin-B1
D 123779566 MTCH1; LOW QUALITY PROTEIN: mitochondrial carrier homolog 1 K17885 MTCH; mitochondrial carrier
D 123784831 mitochondrial carrier homolog 1-like K17885 MTCH; mitochondrial carrier
D 123785038 MTCH2; mitochondrial carrier homolog 2 isoform X1 K17885 MTCH; mitochondrial carrier
D 123778095 MFF; mitochondrial fission factor isoform X1 K22076 MFF; mitochondrial fission factor
D 123797011 GDAP1L1; ganglioside-induced differentiation-associated protein 1-like 1 isoform X1 K22077 GDAP1; ganglioside-induced differentiation-associated protein 1
D 123800473 GDAP1; ganglioside-induced differentiation-associated protein 1 isoform X1 K22077 GDAP1; ganglioside-induced differentiation-associated protein 1
D 123781867 VAT1; synaptic vesicle membrane protein VAT-1 homolog K23167 VAT1; synaptic vesicle membrane protein VAT-1
D 123779761 MIEF2; mitochondrial dynamics protein MID49 K23507 MIEF1_2; mitochondrial dynamics protein MID51/49
D 123780648 MIEF1; mitochondrial dynamics protein MID51 isoform X1 K23507 MIEF1_2; mitochondrial dynamics protein MID51/49
D 123779766 PLD6; mitochondrial cardiolipin hydrolase K16862 PLD6; mitochondrial cardiolipin hydrolase [EC:3.1.4.-]
D 123775665 MIGA2; mitoguardin 2 K27289 MIGA; mitoguardin
D 123795291 MIGA1; mitoguardin 1 K27289 MIGA; mitoguardin
C Actin cytoskeleton organization
D 123777926 ACTG1; actin, cytoplasmic 2 K05692 ACTB_G1; actin beta/gamma 1
D 123802532 ACTB; actin, cytoplasmic 1 K05692 ACTB_G1; actin beta/gamma 1
D 123800273 NAA20; N-alpha-acetyltransferase 20 K17972 NAA20; N-terminal acetyltransferase B complex catalytic subunit [EC:2.3.1.254]
D 123778911 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 123802851 PHB1; prohibitin K17080 PHB1; prohibitin 1
D 123781537 PHB2; LOW QUALITY PROTEIN: prohibitin-2 K17081 PHB2; prohibitin 2
D 123800727 KTN1; kinectin isoform X1 K17975 KTN1; kinectin
C Retention of mitochondria
D 123801708 DNM1L; dynamin-1-like protein isoform X1 K17065 DNM1L; dynamin 1-like protein [EC:3.6.5.5]
D 123787383 MYO5B; unconventional myosin-Vb isoform X1 K10357 MYO5; myosin V
D 123801798 MYO5A; unconventional myosin-Va isoform X1 K10357 MYO5; myosin V
D 123801870 MYO5C; unconventional myosin-Vc isoform X1 K10357 MYO5; myosin V
D 123792333 E3 ubiquitin-protein ligase XIAP isoform X1 K04725 XIAP; E3 ubiquitin-protein ligase XIAP [EC:2.3.2.27]
D 123779506 DIABLO; diablo homolog, mitochondrial isoform X1 K10522 DIABLO; diablo
C Morphogenesis factors
D 123784506 SNX25; sorting nexin-25 isoform X1 K17887 SNX25; sorting nexin-25
D 123776840 RHOT1; mitochondrial Rho GTPase 1 isoform X1 K07870 RHOT1; mitochondrial Rho GTPase 1 [EC:3.6.5.-]
D 123799697 IMMT; MICOS complex subunit MIC60 isoform X1 K17785 IMMT; MICOS complex subunit MIC60
D 123788165 MTFP1; mitochondrial fission process protein 1 isoform X1 K17981 MTFP1; mitochondrial fission process protein 1
D 123783429 DAP3; 28S ribosomal protein S29, mitochondrial isoform X1 K17408 DAP3; small subunit ribosomal protein S29
D 123787970 NEDD4; E3 ubiquitin-protein ligase NEDD4 isoform X1 K10591 NEDD4; E3 ubiquitin-protein ligase NEDD4 [EC:2.3.2.26]
D 123797804 OXR1; oxidation resistance protein 1 isoform X1 K25437 OXR1; oxidation resistance protein 1
D 123783407 MSTO1; protein misato homolog 1 isoform X1 K27320 MSTO1; protein misato
D 123793197 PRELID1; PRELI domain-containing protein 1, mitochondrial K27964 PRELID1; PRELI domain-containing protein 1, mitochondrial
D 123790197 PRELID2; PRELI domain-containing protein 2 isoform X1 K27965 PRELID2; PRELI domain-containing protein 2 mitochondrial
D 123803294 PRELID3B; PRELI domain containing protein 3B K27966 PRELID3; PRELI domain-containing protein 3, mitochondrial
D 123803574 PRELID3A; PRELI domain containing protein 3A isoform X1 K27966 PRELID3; PRELI domain-containing protein 3, mitochondrial
B Mitophagy factors
C Autophagy-related proteins
D 123778272 ATG2A; autophagy-related protein 2 homolog A isoform X1 K17906 ATG2; autophagy-related protein 2
D 123803919 ATG2B; autophagy-related protein 2 homolog B isoform X1 K17906 ATG2; autophagy-related protein 2
D 123784066 ATG3; ubiquitin-like-conjugating enzyme ATG3 K08343 ATG3; ubiquitin-like-conjugating enzyme ATG3
D 123777724 ATG4D; cysteine protease ATG4D isoform X1 K08342 ATG4; cysteine protease ATG4 [EC:3.4.22.-]
D 123784799 ATG4A; cysteine protease ATG4A K08342 ATG4; cysteine protease ATG4 [EC:3.4.22.-]
D 123791225 ATG4B; cysteine protease ATG4B K08342 ATG4; cysteine protease ATG4 [EC:3.4.22.-]
D 123794206 cysteine protease ATG4B-like K08342 ATG4; cysteine protease ATG4 [EC:3.4.22.-]
D 123795361 ATG4C; cysteine protease ATG4C isoform X1 K08342 ATG4; cysteine protease ATG4 [EC:3.4.22.-]
D 123798518 ATG5; autophagy protein 5 isoform X1 K08339 ATG5; autophagy-related protein 5
D 123781873 BECN1; beclin-1 isoform X1 K08334 BECN; beclin
D 123790998 BECN2; beclin-2 K08334 BECN; beclin
D 123801971 ATG7; ubiquitin-like modifier-activating enzyme ATG7 K08337 ATG7; ubiquitin-like modifier-activating enzyme ATG7
D 123781641 GABARAPL1; gamma-aminobutyric acid receptor-associated protein-like 1 K08341 GABARAP; GABA(A) receptor-associated protein
D 123791319 GABARAPL2; gamma-aminobutyric acid receptor-associated protein-like 2 K08341 GABARAP; GABA(A) receptor-associated protein
D 123792166 LOW QUALITY PROTEIN: gamma-aminobutyric acid receptor-associated protein K08341 GABARAP; GABA(A) receptor-associated protein
D 123779479 ATG9A; autophagy-related protein 9A isoform X1 K17907 ATG9; autophagy-related protein 9
D 123788782 ATG9B; autophagy-related protein 9B K17907 ATG9; autophagy-related protein 9
D 123791511 ATG10; ubiquitin-like-conjugating enzyme ATG10 isoform X1 K17888 ATG10L; ubiquitin-like-conjugating enzyme ATG10
D 123790443 ATG12; ubiquitin-like protein ATG12 isoform X1 K08336 ATG12; ubiquitin-like protein ATG12
D 123800612 ATG14; beclin 1-associated autophagy-related key regulator K17889 ATG14L; beclin 1-associated autophagy-related key regulator
D 123790051 ATG16L1; autophagy-related protein 16-1 isoform X1 K17890 ATG16L1; autophagy-related protein 16-1
D 123804197 ATG16L2; protein Atg16l2 isoform X1 K20868 ATG16L2; autophagy-related protein 16-2
D 123802503 WIPI2; WD repeat domain phosphoinositide-interacting protein 2 isoform X1 K17908 WIPI1_2; autophagy-related protein 18
D 123802964 WIPI1; WD repeat domain phosphoinositide-interacting protein 1 isoform X1 K17908 WIPI1_2; autophagy-related protein 18
C Parkin-dependent mechanism factors
D 123797535 PRKN; E3 ubiquitin-protein ligase parkin isoform X1 K04556 PARK2; parkin [EC:2.3.2.31]
D 123787256 RNF41; E3 ubiquitin-protein ligase NRDP1 isoform X1 K11981 RNF41; E3 ubiquitin-protein ligase NRDP1 [EC:2.3.2.27]
D 123783558 CLEC16A; protein CLEC16A isoform X1 K19513 CLEC16A; protein CLEC16A
D 123782135 PINK1; serine/threonine-protein kinase PINK1, mitochondrial K05688 PINK1; PTEN induced putative kinase 1 [EC:2.7.11.1]
D 123778931 PARL; presenilins-associated rhomboid-like protein, mitochondrial K09650 PARL; rhomboid-like protein [EC:3.4.21.105]
D 123790491 SQSTM1; sequestosome-1 K14381 SQSTM1; sequestosome 1
D 123789129 TAX1BP1; tax1-binding protein 1 isoform X1 K21347 TAX1BP1; Tax1-binding protein 1
D 123802839 CALCOCO2; calcium-binding and coiled-coil domain-containing protein 2 K21348 CALCOCO2; calcium-binding and coiled-coil domain-containing protein 2
D 123799572 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 123785004 ATG13; autophagy-related protein 13 isoform X1 K08331 ATG13; autophagy-related protein 13
D 123803317 ULK1; serine/threonine-protein kinase ULK1 isoform X1 K21357 ULK1; serine/threonine-protein kinase ULK1 [EC:2.7.11.1]
D 123779782 ULK2; serine/threonine-protein kinase ULK2 K08269 ULK2; serine/threonine-protein kinase ULK2 [EC:2.7.11.1]
D 123786734 RB1CC1; RB1-inducible coiled-coil protein 1 isoform X1 K17589 RB1CC1; RB1-inducible coiled-coil protein 1
D 123778170 HSP90AA1; heat shock protein HSP 90-alpha K04079 HSP90A; molecular chaperone HtpG
D 123795038 HSP90AB1; heat shock protein HSP 90-beta K04079 HSP90A; molecular chaperone HtpG
D 123777589 CDC37; hsp90 co-chaperone Cdc37 K09554 CDC37; cell division cycle protein 37
D 123802558 SMURF1; E3 ubiquitin-protein ligase SMURF1 isoform X1 K04678 SMURF; E3 ubiquitin ligase SMURF1/2 [EC:2.3.2.26]
D 123803252 SMURF2; E3 ubiquitin-protein ligase SMURF2 K04678 SMURF; E3 ubiquitin ligase SMURF1/2 [EC:2.3.2.26]
D 123777393 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 123794549 FUNDC1; FUN14 domain-containing protein 1 K17986 FUNDC1; FUN14 domain-containing protein 1
C Other mitophagy factors
D 123785508 PRKAA1; 5'-AMP-activated protein kinase catalytic subunit alpha-1 isoform X1 K07198 PRKAA; 5'-AMP-activated protein kinase, catalytic alpha subunit [EC:2.7.11.31]
D 123795384 PRKAA2; 5'-AMP-activated protein kinase catalytic subunit alpha-2 isoform X1 K07198 PRKAA; 5'-AMP-activated protein kinase, catalytic alpha subunit [EC:2.7.11.31]
D 123776674 MAP1LC3B; microtubule-associated proteins 1A/1B light chain 3B K10435 MAP1LC; microtubule-associated protein 1 light chain
D 123790991 microtubule-associated proteins 1A/1B light chain 3C-like K10435 MAP1LC; microtubule-associated protein 1 light chain
D 123791286 microtubule-associated proteins 1A/1B light chain 3C-like K10435 MAP1LC; microtubule-associated protein 1 light chain
D 123798215 MAP1LC3A; microtubule-associated proteins 1A/1B light chain 3A K10435 MAP1LC; microtubule-associated protein 1 light chain
D 123781965 NBR1; next to BRCA1 gene 1 protein isoform X1 K17987 NBR1; next to BRCA1 gene 1 protein
D 123802525 TECPR1; tectonin beta-propeller repeat-containing protein 1 K17988 TECPR1; tectonin beta-propeller repeat-containing protein 1
D 123799017 ZFYVE1; zinc finger FYVE domain-containing protein 1 K17603 ZFYVE1; zinc finger FYVE domain-containing protein 1
D 123781955 COASY; bifunctional coenzyme A synthase isoform X1 K02318 COASY; phosphopantetheine adenylyltransferase / dephospho-CoA kinase [EC:2.7.7.3 2.7.1.24]
D 123780588 PLA2G6; 85/88 kDa calcium-independent phospholipase A2 isoform X1 K16343 PLA2G6; calcium-independent phospholipase A2 [EC:3.1.1.4]
D 123791366 protein C19orf12 homolog K23168 MPAN; protein MPAN
D 123791368 protein C19orf12 homolog K23168 MPAN; protein MPAN
D 123791369 protein C19orf12 homolog K23168 MPAN; protein MPAN
D 123791372 CUNH19orf12; protein C19orf12 homolog isoform X1 K23168 MPAN; protein MPAN
D 123791419 uncharacterized protein LOC123791419 K23168 MPAN; protein MPAN
D 123786664 FUNDC2; FUN14 domain-containing protein 2 isoform X1 K24308 FUNDC2; FUN14 domain-containing protein 2
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#[ BRITE | KEGG2 | KEGG ]
#Last updated: September 5, 2025