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Mitochondrial Biogenesis - Mesitornis unicolor (brown roatelo)
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AMitochondrial DNA transcription, translation, and replication factors
B Mitochondrial DNA-encoded proteins
C Mitochondrial respiratory chain complex I
C Mitochondrial respiratory chain complex III
C Cytochrome c oxidase
C F-type ATPase
B Mitochondrial transcription and translation factors
C Mitochondrial RNA polymerase
D 104544473 POLRMT; DNA-directed RNA polymerase, mitochondrial K10908 POLRMT; DNA-directed RNA polymerase, mitochondrial [EC:2.7.7.6]
C Mitochondrial transcription factors
D 104536543 TFB1M; dimethyladenosine transferase 1, mitochondrial K15266 TFB1M; dimethyladenosine transferase 1, mitochondrial [EC:2.1.1.-]
D 104536620 TFB2M; dimethyladenosine transferase 2, mitochondrial K17653 TFB2M; dimethyladenosine transferase 2, mitochondrial [EC:2.1.1.-]
C Mitochondrial translation factors
D 104547024 MTIF2; translation initiation factor IF-2, mitochondrial K02519 infB; translation initiation factor IF-2
D 104541234 MTIF3; translation initiation factor IF-3, mitochondrial K02520 infC; translation initiation factor IF-3
D 104535897 elongation factor G, mitochondrial-like K02355 fusA; elongation factor G
D 104547843 GFM2; ribosome-releasing factor 2, mitochondrial K02355 fusA; elongation factor G
D 104541308 MTERF2; transcription termination factor 2, mitochondrial K15031 MTERF; mitochondrial transcription termination factor family protein
D 104534772 MTERF3; transcription termination factor 3, mitochondrial K15031 MTERF; mitochondrial transcription termination factor family protein
D 104547477 GUF1; translation factor GUF1, mitochondrial K21594 GUF1; translation factor GUF1, mitochondrial [EC:3.6.5.-]
C RNase MRP
D 104537544 POP1; ribonucleases P/MRP protein subunit POP1 K01164 POP1; ribonuclease P/MRP protein subunit POP1 [EC:3.1.26.5]
D 104537732 POP4; ribonuclease P protein subunit p29 K03538 POP4; ribonuclease P protein subunit POP4 [EC:3.1.26.5]
D 104537940 POP5; ribonuclease P/MRP protein subunit POP5 K03537 POP5; ribonuclease P/MRP protein subunit POP5 [EC:3.1.26.5]
D 104539856 RPP30; ribonuclease P protein subunit p30 K03539 RPP1; ribonuclease P/MRP protein subunit RPP1 [EC:3.1.26.5]
D 104541288 RPP25L; ribonuclease P protein subunit p25-like protein K14525 RPP25; ribonucleases P/MRP protein subunit RPP25 [EC:3.1.26.5]
D 104535859 RPP25; ribonuclease P protein subunit p25 K14525 RPP25; ribonucleases P/MRP protein subunit RPP25 [EC:3.1.26.5]
D 104533802 RPP38; ribonuclease P protein subunit p38 K14523 RPP38; ribonucleases P/MRP protein subunit RPP38 [EC:3.1.26.5]
C Mitochondrial ribonuclease P
D 104535503 TRMT10C; mitochondrial ribonuclease P protein 1 K17654 TRMT10C; mitochondrial ribonuclease P protein 1 [EC:2.1.1.-]
D 104538959 KIAA0391; mitochondrial ribonuclease P protein 3 K17655 MRPP3; mitochondrial ribonuclease P protein 3
C Other mitochondrial DNA transcription and translation factors
D 104539454 MSS51; putative protein MSS51 homolog, mitochondrial K17656 MSS51; mitochondrial splicing suppressor protein 51
D 104534245 LOW QUALITY PROTEIN: pentatricopeptide repeat-containing protein 1, mitochondrial-like K17710 PTCD1; pentatricopeptide repeat domain-containing protein 1
D 104539998 PTCD2; pentatricopeptide repeat-containing protein 2, mitochondrial K17711 PTCD2; pentatricopeptide repeat domain-containing protein 2
D 104543208 PTCD3; pentatricopeptide repeat domain-containing protein 3, mitochondrial K17659 PTCD3; pentatricopeptide repeat domain-containing protein 3
D 104540286 LOW QUALITY PROTEIN: putative ATP-dependent RNA helicase DHX30 K13185 DHX30; ATP-dependent RNA helicase DHX30 [EC:5.6.2.6]
D 104548159 ubiquinol-cytochrome-c reductase complex assembly factor 1 K17662 CBP3; cytochrome b pre-mRNA-processing protein 3
D 104540159 LACE1; lactation elevated protein 1 K18798 AFG1; peroxisome-assembly ATPase [EC:3.6.4.7]
D 104547029 XRCC6BP1; mitochondrial inner membrane protease ATP23 homolog isoform X1 K18156 ATP23; mitochondrial inner membrane protease ATP23 [EC:3.4.24.-]
D 104542826 SUPV3L1; ATP-dependent RNA helicase SUPV3L1, mitochondrial K17675 SUPV3L1; ATP-dependent RNA helicase SUPV3L1/SUV3 [EC:5.6.2.6]
D 104545377 LACTB2; beta-lactamase-like protein 2 K16639 LACTB2; endoribonuclease LACTB2 [EC:3.1.27.-]
D 104535384 ADP/ATP translocase 3-like K05863 SLC25A4S; solute carrier family 25 (mitochondrial adenine nucleotide translocator), member 4/5/6/31
D 104535406 ADP/ATP translocase 1-like K05863 SLC25A4S; solute carrier family 25 (mitochondrial adenine nucleotide translocator), member 4/5/6/31
D 104538442 ADP/ATP translocase 2 K05863 SLC25A4S; solute carrier family 25 (mitochondrial adenine nucleotide translocator), member 4/5/6/31
D 104533471 SLC25A37; mitoferrin-1 K15113 SLC25A28_37; solute carrier family 25 (mitochondrial iron transporter), member 28/37
D 104536268 mitoferrin-2 K15113 SLC25A28_37; solute carrier family 25 (mitochondrial iron transporter), member 28/37
D 104543541 MALSU1; mitochondrial assembly of ribosomal large subunit protein 1 K25803 MALSU1; mitochondrial assembly of ribosomal large subunit protein 1
D 104534303 ERAL1; GTPase Era, mitochondrial K03595 era; GTPase
D 104546073 YARS; tyrosine--tRNA ligase, cytoplasmic K01866 YARS; tyrosyl-tRNA synthetase [EC:6.1.1.1]
D 104543065 YARS2; tyrosine--tRNA ligase, mitochondrial K01866 YARS; tyrosyl-tRNA synthetase [EC:6.1.1.1]
D 104542772 LARS2; probable leucine--tRNA ligase, mitochondrial K01869 LARS; leucyl-tRNA synthetase [EC:6.1.1.4]
D 104537489 LARS; leucine--tRNA ligase, cytoplasmic K01869 LARS; leucyl-tRNA synthetase [EC:6.1.1.4]
D 104533823 DARS2; aspartate--tRNA ligase, mitochondrial K01876 DARS2; aspartyl-tRNA synthetase [EC:6.1.1.12]
D 104540303 GARS; glycine--tRNA ligase K01880 GARS; glycyl-tRNA synthetase [EC:6.1.1.14]
D 104540829 RARS; arginine--tRNA ligase, cytoplasmic K01887 RARS; arginyl-tRNA synthetase [EC:6.1.1.19]
D 104543368 RARS2; LOW QUALITY PROTEIN: probable arginine--tRNA ligase, mitochondrial K01887 RARS; arginyl-tRNA synthetase [EC:6.1.1.19]
D 104534128 QRSL1; 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 104536433 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 104535757 GATC; LOW QUALITY PROTEIN: 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 104538415 MTPAP; poly(A) RNA polymerase, mitochondrial K18060 MTPAP; poly(A) RNA polymerase, mitochondrial [EC:2.7.7.19]
D 104534018 PDE12; 2',5'-phosphodiesterase 12 K19612 PDE12; 2',5'-phosphodiesterase [EC:3.1.13.4 3.1.4.-]
D 104533885 MTG1; mitochondrial ribosome-associated GTPase 1 K19828 MTG1; mitochondrial GTPase 1
D 104541272 NSUN3; putative methyltransferase NSUN3 K21969 NSUN3; tRNA (cytosine34-C5)-methyltransferase [EC:2.1.1.-]
D 104539548 NSUN4; 5-methylcytosine rRNA methyltransferase NSUN4 K21970 NSUN4; 5-methylcytosine rRNA methyltransferase NSUN4 [EC:2.1.1.-]
D 104541979 RPUSD4; RNA pseudouridylate synthase domain-containing protein 4 K22538 RPUSD4; mitochondrial RNA pseudouridine synthase RPUSD4 [EC:5.4.99.-]
D 104533457 WBSCR16; Williams-Beuren syndrome chromosomal region 16 protein K23495 RCC1L; RCC1-like G exchanging factor-like protein
D 104540183 TRUB2; probable tRNA pseudouridine synthase 2 K23497 TRUB2; mitochondrial mRNA pseudouridine synthase TRUB2 [EC:5.4.99.-]
D 104542748 FASTKD2; FAST kinase domain-containing protein 2 K18190 FASTKD2; FAST kinase domain-containing protein 2
D 104538365 probable peptide chain release factor C12orf65 homolog, mitochondrial K23498 COXPD7; peptide chain release factor
D 104542079 RMND1; required for meiotic nuclear division protein 1 homolog K23499 RMND1; required for meiotic nuclear division protein 1
D 104534298 GRSF1; LOW QUALITY PROTEIN: G-rich sequence factor 1 K24984 GRSF1; G-rich sequence factor 1
D 104548310 METTL15; probable methyltransferase-like protein 15 K25876 METTL15; 12S rRNA N4-methylcytidine (m4C) methyltransferase [EC:2.1.1.-]
D 104535402 UPF0160 protein MYG1, mitochondrial K25877 MYG1; MYG1 exonuclease [EC:3.1.-.-]
D 104544683 uncharacterized protein C6orf203 homolog K26186 MTRES1; mitochondrial transcription rescue factor 1
B Mitochondrial DNA replication factors
C Mitochondrial DNA polymerase
D 104539957 POLG; DNA polymerase subunit gamma-1 K02332 POLG; DNA polymerase gamma 1 [EC:2.7.7.7]
D 104536677 POLG2; DNA polymerase subunit gamma-2, mitochondrial K02333 POLG2; DNA polymerase gamma 2
D 104545929 PRIMPOL; DNA-directed primase/polymerase protein K22761 PRIMPOL; DNA-directed primase/polymerase protein [EC:2.7.7.102 2.7.7.7]
C Mitochondrial DNA maintenance factors
D 104548475 ENDOG; endonuclease G, mitochondrial K01173 ENDOG; endonuclease G, mitochondrial
D 104539379 MGME1; mitochondrial genome maintenance exonuclease 1 K19465 MGME1; mitochondrial genome maintenance exonuclease 1 [EC:3.1.-.-]
D 104544055 EXOG; nuclease EXOG, mitochondrial K15050 EXOG; nuclease EXOG, mitochondrial
D 104548152 twinkle protein, mitochondrial K17680 PEO1; twinkle protein [EC:5.6.2.3]
C Other Mitochondrial DNA replication factors
D 104538088 SSBP1; single-stranded DNA-binding protein, mitochondrial K03111 ssb; single-strand DNA-binding protein
D 104533927 ATPase family AAA domain-containing protein 3-like K17681 ATAD3A_B; ATPase family AAA domain-containing protein 3A/B
D 104543363 ubiquinol-cytochrome-c reductase complex assembly factor 2 K17682 MNF1; mitochondrial nucleoid factor 1
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AMitochondrial protein import machinery
B Endoplasmic reticulum membrane and cytosol
C ER-mitochondria encounter structure (ERMES) complex
C Other ER-memrane and cytosol factors
D 104540089 DNAJA2; dnaJ homolog subfamily A member 2 K09503 DNAJA2; DnaJ homolog subfamily A member 2
D 104534175 TOMM34; mitochondrial import receptor subunit TOM34 K17766 TOM34; mitochondrial import receptor subunit TOM34
D 104547493 RHOT1; LOW QUALITY PROTEIN: mitochondrial Rho GTPase 1 K07870 RHOT1; mitochondrial Rho GTPase 1 [EC:3.6.5.-]
D 104534263 HSPA8; heat shock cognate 71 kDa protein isoform X1 K03283 HSPA1_6_8; heat shock 70kDa protein 1/6/8
D 104539117 aryl-hydrocarbon-interacting protein-like 1 K17767 AIP; AH receptor-interacting protein
D 104536190 LOW QUALITY PROTEIN: AH receptor-interacting protein-like K17767 AIP; AH receptor-interacting protein
B Outer membrane
C Transporter outer membrane (TOM) complex
D 104536397 TOMM70A; mitochondrial import receptor subunit TOM70 K17768 TOM70; mitochondrial import receptor subunit TOM70
D 104535154 TOMM20; mitochondrial import receptor subunit TOM20 homolog K17770 TOM20; mitochondrial import receptor subunit TOM20
C TOB/SAM complex
D 104534060 SAMM50; sorting and assembly machinery component 50 homolog K07277 SAM50; outer membrane protein insertion porin family
C Porin
D 104534556 VDAC1; voltage-dependent anion-selective channel protein 1 K05862 VDAC1; voltage-dependent anion channel protein 1
D 104535115 VDAC2; voltage-dependent anion-selective channel protein 2 isoform X1 K15040 VDAC2; voltage-dependent anion channel protein 2
D 104534789 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 104544871 MTX3; metaxin-3 K17776 MTX; metaxin
D 104537115 MTX2; metaxin-2 K17776 MTX; metaxin
D 104533695 MYO19; unconventional myosin-XIX K25838 MYO19; myosin XIX
D 104544844 mitochondrial amidoxime reducing component 2-like K27318 MTARC; mitochondrial amidoxime-reducing component [EC:1.7.-.-]
B Inter membrane space
C Transporter inner membrane (TIM) 9/10 complex
D 104538197 TIMM9; mitochondrial import inner membrane translocase subunit Tim9 K17777 TIM9; mitochondrial import inner membrane translocase subunit TIM9
C TIM8/13 complex
D 104533563 TIMM8A; mitochondrial import inner membrane translocase subunit Tim8 A K17780 TIM8; mitochondrial import inner membrane translocase subunit TIM8
D 104534510 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 104547308 CHCHD4; mitochondrial intermembrane space import and assembly protein 40 K17782 MIA40; mitochondrial intermembrane space import and assembly protein 40
D 104537445 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 104539788 MINOS1; MICOS complex subunit MIC10 K17784 MICOS10; MICOS complex subunit MIC10
D 104543210 IMMT; MICOS complex subunit MIC60 K17785 IMMT; MICOS complex subunit MIC60
D 104535285 protein QIL1 K24624 MICOS13; MICOS complex subunit MIC13
D 104542804 APOO; apolipoprotein O K24625 APOO; MICOS complex subunit MIC26
D 104533523 APOOL; MICOS complex subunit MIC27 K24626 APOOL; MICOS complex subunit MIC27
C MICOS complex (yeast)
D 104539788 MINOS1; MICOS complex subunit MIC10 K17784 MICOS10; MICOS complex subunit MIC10
D 104543210 IMMT; MICOS complex subunit MIC60 K17785 IMMT; MICOS complex subunit MIC60
C TIM22 complex
D 104543009 TIMM22; mitochondrial import inner membrane translocase subunit Tim22 K17790 TIM22; mitochondrial import inner membrane translocase subunit TIM22
C TIM23 complex
D 104543055 TIMM23; mitochondrial import inner membrane translocase subunit Tim23 K17794 TIM23; mitochondrial import inner membrane translocase subunit TIM23
D 104546499 TIMM17A; mitochondrial import inner membrane translocase subunit Tim17-A K17795 TIM17; mitochondrial import inner membrane translocase subunit TIM17
D 104533944 TIMM21; mitochondrial import inner membrane translocase subunit Tim21 K17796 TIM21; mitochondrial import inner membrane translocase subunit TIM21
C Mitochondrial calcium uniporter complex
D 104544564 MCU; LOW QUALITY PROTEIN: calcium uniporter protein, mitochondrial K20858 MCU; calcium uniporter protein, mitochondrial
D 104544645 MICU2; calcium uptake protein 2, mitochondrial K22828 MICU2; calcium uptake protein 2, mitochondrial
D 104544709 CCDC109B; mitochondrial calcium uniporter regulatory subunit MCUb K22829 MCUB; calcium uniporter regulatory subunit MCUb, mitochondrial
D 104536930 mitochondrial calcium uniporter regulator 1-like K22137 MCUR1; mitochondrial calcium uniporter regulator 1
C Other inner membrane factors
D 104543686 LETM1; LETM1 and EF-hand domain-containing protein 1, mitochondrial K17800 LETM1; LETM1 and EF-hand domain-containing protein 1, mitochondrial
D 104546162 IMMP1L; mitochondrial inner membrane protease subunit 1 K09647 IMP1; mitochondrial inner membrane protease subunit 1 [EC:3.4.21.-]
D 104541395 mitochondrial inner membrane protease subunit 2-like K09648 IMP2; mitochondrial inner membrane protease subunit 2 [EC:3.4.21.-]
D 104534010 TMEM126A; transmembrane protein 126A K18157 TMEM126A; transmembrane protein 126A
D 104537714 MPC1; mitochondrial pyruvate carrier 1 K22138 MPC1; mitochondrial pyruvate carrier 1
D 104535255 MPC2; mitochondrial pyruvate carrier 2 K22139 MPC2; mitochondrial pyruvate carrier 2
B Matrix
C MMC/PAM proteins
D 104545886 TIMM44; mitochondrial import inner membrane translocase subunit TIM44 K17804 TIM44; mitochondrial import inner membrane translocase subunit TIM44
D 104537406 HSPA9; stress-70 protein, mitochondrial K04043 dnaK; molecular chaperone DnaK
D 104534866 GRPEL2; grpE protein homolog 2, mitochondrial K03687 GRPE; molecular chaperone GrpE
D 104548063 GRPEL1; grpE protein homolog 1, mitochondrial K03687 GRPE; molecular chaperone GrpE
D 104542820 DNAJC19; mitochondrial import inner membrane translocase subunit TIM14 K09539 DNAJC19; DnaJ homolog subfamily C member 19
D 104545468 PAM16; mitochondrial import inner membrane translocase subunit TIM16 K17805 PAM16; mitochondrial import inner membrane translocase subunit TIM16
C Other matrix factors
D 104533750 PMPCA; mitochondrial-processing peptidase subunit alpha K01412 PMPCA; mitochondrial-processing peptidase subunit alpha [EC:3.4.24.64]
D 104539831 PMPCB; mitochondrial-processing peptidase subunit beta K17732 PMPCB; mitochondrial-processing peptidase subunit beta [EC:3.4.24.64]
D 104536330 MIPEP; mitochondrial intermediate peptidase K01410 MIPEP; mitochondrial intermediate peptidase [EC:3.4.24.59]
D 104534161 HSPD1; 60 kDa heat shock protein, mitochondrial K04077 groEL; chaperonin GroEL [EC:5.6.1.7]
D 104541645 HSPE1; 10 kDa heat shock protein, mitochondrial K04078 groES; chaperonin GroES
D 104534537 DNAJA3; dnaJ homolog subfamily A member 3, mitochondrial K09504 DNAJA3; DnaJ homolog subfamily A member 3
D 104545547 TAMM41; phosphatidate cytidylyltransferase, mitochondrial K17807 TAM41; mitochondrial translocator assembly and maintenance protein 41
D 104543551 DNLZ; DNL-type zinc finger protein K17808 ZIM17; mitochondrial protein import protein ZIM17
D 104540763 POLDIP2; polymerase delta-interacting protein 2 K17809 POLDIP2; polymerase delta-interacting protein 2
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AMitochondrial quality control factors
B Regulator of mitochondrial biogenesis
C Ubiquitas transcription factors
D 104547803 CREB1; cyclic AMP-responsive element-binding protein 1 isoform X1 K05870 CREB1; cyclic AMP-responsive element-binding protein 1
D 104535897 elongation factor G, mitochondrial-like K02355 fusA; elongation factor G
D 104547843 GFM2; ribosome-releasing factor 2, mitochondrial K02355 fusA; elongation factor G
D 104541243 SP1; transcription factor Sp1 K04684 SP1; transcription factor Sp1
D 104533469 GA-binding protein subunit beta-1 K09454 GABPB; GA-binding protein transcription factor, beta
C Nuclear respiratory factors
D 104548884 nuclear factor erythroid 2-related factor 2-like K05638 NFE2L2; nuclear factor erythroid 2-related factor 2
D 104536991 metallothionein-2 K14739 MT1_2; metallothionein 1/2
C Coactivators
D 104538605 PPARGC1A; peroxisome proliferator-activated receptor gamma coactivator 1-alpha K07202 PPARGC1A; alpha peroxisome proliferator-activated receptor gamma coactivator 1-alpha
D 104541951 PPARGC1B; peroxisome proliferator-activated receptor gamma coactivator 1-beta K17962 PPARGC1B; peroxisome proliferator-activated receptor gamma coactivator 1-beta
D 104534482 CREB-regulated transcription coactivator 1-like K15309 CRTC1; CREB-regulated transcription coactivator 1
D 104541933 CREBBP; CREB-binding protein K04498 EP300; E1A/CREB-binding protein [EC:2.3.1.48]
D 104543391 EP300; LOW QUALITY PROTEIN: histone acetyltransferase p300 K04498 EP300; E1A/CREB-binding protein [EC:2.3.1.48]
C Proteins involved in Fe-S cluster biogenesis
D 104534276 NFS1; cysteine desulfurase, mitochondrial K04487 iscS; cysteine desulfurase [EC:2.8.1.7]
D 104534122 ISCU; iron-sulfur cluster assembly enzyme ISCU, mitochondrial K22068 ISCU; iron-sulfur cluster assembly enzyme ISCU, mitochondrial
D 104534333 FXN; frataxin, mitochondrial K19054 FXN; frataxin [EC:1.16.3.1]
D 104537802 FDX1; adrenodoxin, mitochondrial K22070 FDX1; ferredoxin-1, mitochondrial
D 104540349 adrenodoxin-like K22071 FDX2; ferredoxin-2, mitochondrial
D 104537406 HSPA9; stress-70 protein, mitochondrial K04043 dnaK; molecular chaperone DnaK
D 104546598 HSCB; iron-sulfur cluster co-chaperone protein HscB, mitochondrial K04082 hscB; molecular chaperone HscB
D 104539931 SIL1; nucleotide exchange factor SIL1 K14001 SIL1; nucleotide exchange factor SIL1
D 104538782 ISCA1; iron-sulfur cluster assembly 1 homolog, mitochondrial K22063 ISCA1; iron-sulfur cluster assembly 1
D 104538924 ISCA2; iron-sulfur cluster assembly 2 homolog, mitochondrial K22072 ISCA2; iron-sulfur cluster assembly 2
D 104539307 IBA57; putative transferase CAF17, mitochondrial K22073 IBA57; transferase CAF17, mitochondrial [EC:2.1.-.-]
D 104533379 sideroflexin-3-like K23500 SFXN1_3; sideroflexin-1/3
D 104534658 SFXN1; sideroflexin-1 K23500 SFXN1_3; sideroflexin-1/3
D 104533933 SFXN2; sideroflexin-2 K23501 SFXN2; sideroflexin-2
C Other regulator of mitochondrial biogenesis
D 104536096 CPT1A; carnitine O-palmitoyltransferase 1, liver isoform K08765 CPT1A; carnitine O-palmitoyltransferase 1, liver isoform [EC:2.3.1.21]
D 104533890 DHTKD1; probable 2-oxoglutarate dehydrogenase E1 component DHKTD1, mitochondrial K15791 DHKTD1; 2-oxoadipate dehydrogenase E1 component [EC:1.2.4.-]
D 104534489 FBXL4; F-box/LRR-repeat protein 4 K10270 FBXL4; F-box and leucine-rich repeat protein 4
D 104534839 HCFC2; host cell factor 2 K27390 HCFC2; host cell factor 2
D 104547922 HTT; LOW QUALITY PROTEIN: huntingtin K04533 HD; huntingtin
D 104540036 ESRRB; steroid hormone receptor ERR2 isoform X1 K08553 NR3B2; estrogen-related receptor beta
D 104547925 estrogen-related receptor gamma K08554 NR3B3; estrogen-related receptor gamma
D 104533153 NRIP1; nuclear receptor-interacting protein 1 K17965 NRIP1; nuclear receptor-interacting protein 1
D 104534340 LOW QUALITY PROTEIN: 5'-AMP-activated protein kinase catalytic subunit alpha-1-like K07198 PRKAA; 5'-AMP-activated protein kinase, catalytic alpha subunit [EC:2.7.11.31]
D 104538944 PRKAA2; LOW QUALITY PROTEIN: 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 104539892 LONP1; lon protease homolog, mitochondrial K08675 PRSS15; ATP-dependent Lon protease [EC:3.4.21.53]
D 104534358 SIRT1; NAD-dependent protein deacetylase sirtuin-1 K11411 SIRT1; NAD-dependent protein deacetylase sirtuin 1 [EC:2.3.1.286]
D 104542069 SERAC1; protein SERAC1 K23504 SERAC1; protein SERAC1
D 104537937 PGC-1 and ERR-induced regulator in muscle protein 1-like K26738 PERM1; PGC-1 and ERR-induced regulator in muscle protein 1
B Mitochondrial respiratory chain complex assembly factors
C Complex-I assembly factors
D 104544851 NDUFAF1; complex I intermediate-associated protein 30, mitochondrial K18159 NDUFAF1; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex assembly factor 1
D 104542367 NDUFAF2; mimitin, mitochondrial K18160 NDUFAF2; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex assembly factor 2
D 104541122 NDUFAF4; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex assembly factor 4 K18161 NDUFAF4; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex assembly factor 4
D 104533696 NDUFAF5; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex assembly factor 5 K18162 NDUFAF5; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex assembly factor 5 [EC:2.1.1.-]
D 104543821 NDUFAF6; NADH dehydrogenase (ubiquinone) complex I, assembly factor 6 K18163 NDUFAF6; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex assembly factor 6
D 104542150 NDUFAF7; NADH dehydrogenase [ubiquinone] complex I, assembly factor 7 K18164 NDUFAF7; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex assembly factor 7
D 104546384 ACAD9; acyl-CoA dehydrogenase family member 9, mitochondrial K15980 ACAD9; acyl-CoA dehydrogenase family member 9 [EC:1.3.99.-]
D 104546513 cytosolic Fe-S cluster assembly factor NUBP2-like K03593 mrp; ATP-binding protein involved in chromosome partitioning
D 104538246 iron-sulfur protein NUBPL-like K03593 mrp; ATP-binding protein involved in chromosome partitioning
D 104544458 RTN4IP1; reticulon-4-interacting protein 1, mitochondrial K23164 RTN4IP1; reticulon-4-interacting protein 1, mitochondrial
D 104548050 TIMMDC1; complex I assembly factor TIMMDC1, mitochondrial K23505 TIMMDC1; complex I assembly factor TIMMDC1
C Complex-II assembly factors
D 104545463 SDHAF2; succinate dehydrogenase assembly factor 2, mitochondrial K18168 SDHAF2; succinate dehydrogenase assembly factor 2
C Complex-III assembly factors
D 104541606 mitochondrial chaperone BCS1 K08900 BCS1; mitochondrial chaperone BCS1
D 104548287 TTC19; tetratricopeptide repeat protein 19, mitochondrial K18169 TTC19; tetratricopeptide repeat protein 19, mitochondrial
D 104542988 LYRM7; LOW QUALITY PROTEIN: complex III assembly factor LYRM7 K18170 LYRM7; complex III assembly factor LYRM7
D 104548159 ubiquinol-cytochrome-c reductase complex assembly factor 1 K17662 CBP3; cytochrome b pre-mRNA-processing protein 3
D 104543363 ubiquinol-cytochrome-c reductase complex assembly factor 2 K17682 MNF1; mitochondrial nucleoid factor 1
D 104538995 protein CCSMST1 K27339 UQCC4; ubiquinol-cytochrome c reductase complex assembly factor 4
D 104534069 SMIM4; small integral membrane protein 4 K27340 UQCC5; ubiquinol-cytochrome c reductase complex assembly factor 5
D 104542744 uncharacterized protein C12orf73 homolog K27341 UQCC6; ubiquinol-cytochrome c reductase complex assembly factor 6
D 104546593 uncharacterized protein C7orf73 homolog K27319 STMP1; short transmembrane mitochondrial protein 1
C Complex-IV assembly factors
D 104539535 CMC2; COX assembly mitochondrial protein 2 homolog K18172 CMC2; COX assembly mitochondrial protein 2
D 104547785 cytochrome c oxidase assembly factor 1 homolog K18173 COA1; cytochrome c oxidase assembly factor 1
D 104540014 LOW QUALITY PROTEIN: cytochrome c oxidase assembly factor 4 homolog, mitochondrial K18177 COA4; cytochrome c oxidase assembly factor 4
D 104548020 cytochrome c oxidase assembly factor 5 K18178 COA5; cytochrome c oxidase assembly factor 5
D 104536421 cytochrome c oxidase assembly factor 7A isoform X1 K18180 COA7; cytochrome c oxidase assembly factor 7
D 104545990 protoheme IX farnesyltransferase, mitochondrial K02257 COX10; heme o synthase [EC:2.5.1.141]
D 104541883 cytochrome c oxidase assembly protein COX11, mitochondrial K02258 COX11; cytochrome c oxidase assembly protein subunit 11
D 104534167 cytochrome c oxidase assembly protein COX15 homolog K02259 COX15; heme a synthase [EC:1.17.99.9]
D 104535608 cytochrome c oxidase assembly protein COX16 homolog, mitochondrial-like K18182 COX16; cytochrome c oxidase assembly protein subunit 16
D 104544716 cytochrome c oxidase copper chaperone K02260 COX17; cytochrome c oxidase assembly protein subunit 17
D 104534036 mitochondrial inner membrane protein COX18 K17797 COX18; mitochondrial inner membrane protein COX18
D 104533515 cytochrome c oxidase protein 20 homolog K18184 COX20; cytochrome c oxidase assembly protein subunit 20
D 104539454 MSS51; putative protein MSS51 homolog, mitochondrial K17656 MSS51; mitochondrial splicing suppressor protein 51
D 104538423 PET117; protein PET117 homolog, mitochondrial K18188 PET117; protein PET117
D 104541082 SURF1; surfeit locus protein 1 K14998 SURF1; surfeit locus 1 family protein
D 104540818 protein SCO1 homolog, mitochondrial K07152 SCO1; protein SCO1
D 104545783 LRPPRC; leucine-rich PPR motif-containing protein, mitochondrial K17964 LRPPRC; leucine-rich PPR motif-containing protein, mitochondrial
D 104536757 APOPT1; apoptogenic protein 1, mitochondrial K23506 APOPT1; apoptogenic protein 1
D 104546593 uncharacterized protein C7orf73 homolog K27319 STMP1; short transmembrane mitochondrial protein 1
D 104534069 SMIM4; small integral membrane protein 4 K27340 UQCC5; ubiquinol-cytochrome c reductase complex assembly factor 5
C Complex-V assembly factors
D 104536304 ATPAF2; ATP synthase mitochondrial F1 complex assembly factor 2 K07556 ATPeAF2; ATP synthase mitochondrial F1 complex assembly factor 2
D 104535345 TMEM70; transmembrane protein 70, mitochondrial K17966 TMEM70; transmembrane protein 70, mitochondrial
D 104537268 6.8 kDa mitochondrial proteolipid K18193 MP68; 6.8 kDa mitochondrial proteolipid
D 104536290 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 104541968 MFN1; LOW QUALITY PROTEIN: mitofusin-1 K21356 MFN1; mitofusin 1 [EC:3.6.5.-]
D 104545988 MFN2; mitofusin-2 isoform X1 K06030 MFN2; mitofusin 2 [EC:3.6.5.-]
D 104533399 OPA1; dynamin-like 120 kDa protein, mitochondrial K17079 OPA1; optic atrophy protein 1 [EC:3.6.5.5]
D 104537883 TRIAP1; TP53-regulated inhibitor of apoptosis 1 K17968 TRIAP1; TRIAP1/MDM35 family protein
D 104534899 PARL; presenilins-associated rhomboid-like protein, mitochondrial isoform X1 K09650 PARL; rhomboid-like protein [EC:3.4.21.105]
D 104538866 BAK1; bcl-2 homologous antagonist/killer K14021 BAK; Bcl-2 homologous antagonist/killer
D 104533958 DNM1L; dynamin-1-like protein K17065 DNM1L; dynamin 1-like protein [EC:3.6.5.5]
D 104535136 SUMO1; small ubiquitin-related modifier 1 K12160 SUMO; small ubiquitin-related modifier
D 104535439 small ubiquitin-related modifier 2 K12160 SUMO; small ubiquitin-related modifier
D 104534962 SH3GLB1; endophilin-B1 K11248 SH3GLB1; endophilin-B1
D 104538771 MTCH1; mitochondrial carrier homolog 1 K17885 MTCH; mitochondrial carrier
D 104534637 MTCH2; mitochondrial carrier homolog 2 K17885 MTCH; mitochondrial carrier
D 104539261 MFF; LOW QUALITY PROTEIN: mitochondrial fission factor K22076 MFF; mitochondrial fission factor
D 104537175 FATE1; fetal and adult testis-expressed transcript protein K22076 MFF; mitochondrial fission factor
D 104544194 GDAP1L1; ganglioside-induced differentiation-associated protein 1-like 1 isoform X1 K22077 GDAP1; ganglioside-induced differentiation-associated protein 1
D 104546377 ganglioside-induced differentiation-associated protein 1 K22077 GDAP1; ganglioside-induced differentiation-associated protein 1
D 104544499 VAT1; synaptic vesicle membrane protein VAT-1 homolog K23167 VAT1; synaptic vesicle membrane protein VAT-1
D 104538240 MIEF1; mitochondrial dynamics protein MID51 K23507 MIEF1_2; mitochondrial dynamics protein MID51/49
D 104538657 MIEF2; mitochondrial dynamics protein MID49 K23507 MIEF1_2; mitochondrial dynamics protein MID51/49
D 104546618 PLD6; mitochondrial cardiolipin hydrolase K16862 PLD6; mitochondrial cardiolipin hydrolase [EC:3.1.4.-]
D 104537503 FAM73A; protein FAM73A K27289 MIGA; mitoguardin
D 104538012 FAM73B; LOW QUALITY PROTEIN: protein FAM73B K27289 MIGA; mitoguardin
C Actin cytoskeleton organization
D 104535615 actin, cytoplasmic type 5 isoform X1 K05692 ACTB_G1; actin beta/gamma 1
D 104539636 LOW QUALITY PROTEIN: actin, cytoplasmic 1-like K05692 ACTB_G1; actin beta/gamma 1
D 104538689 actin, cytoplasmic 1-like K05692 ACTB_G1; actin beta/gamma 1
D 104544635 NAA25; N-alpha-acetyltransferase 25, NatB auxiliary subunit K17973 NAA25; N-terminal acetyltransferase B complex non-catalytic subunit
C Mitochondrial movement
D 104545214 PHB; prohibitin isoform X1 K17080 PHB1; prohibitin 1
D 104535072 PHB2; prohibitin-2 K17081 PHB2; prohibitin 2
D 104539882 KTN1; kinectin K17975 KTN1; kinectin
C Retention of mitochondria
D 104533958 DNM1L; dynamin-1-like protein K17065 DNM1L; dynamin 1-like protein [EC:3.6.5.5]
D 104546803 MYO5B; unconventional myosin-Vb K10357 MYO5; myosin V
D 104538549 MYO5C; unconventional myosin-Vc K10357 MYO5; myosin V
D 104538550 MYO5A; unconventional myosin-Va K10357 MYO5; myosin V
D 104537250 XIAP; E3 ubiquitin-protein ligase XIAP K04725 XIAP; E3 ubiquitin-protein ligase XIAP [EC:2.3.2.27]
D 104533575 DIABLO; diablo homolog, mitochondrial K10522 DIABLO; diablo
C Morphogenesis factors
D 104538162 SNX25; LOW QUALITY PROTEIN: sorting nexin-25 K17887 SNX25; sorting nexin-25
D 104547493 RHOT1; LOW QUALITY PROTEIN: mitochondrial Rho GTPase 1 K07870 RHOT1; mitochondrial Rho GTPase 1 [EC:3.6.5.-]
D 104543210 IMMT; MICOS complex subunit MIC60 K17785 IMMT; MICOS complex subunit MIC60
D 104536902 DAP3; 28S ribosomal protein S29, mitochondrial K17408 DAP3; small subunit ribosomal protein S29
D 104546349 NEDD4; E3 ubiquitin-protein ligase NEDD4 isoform X1 K10591 NEDD4; E3 ubiquitin-protein ligase NEDD4 [EC:2.3.2.26]
D 104539511 oxidation resistance protein 1-like K25437 OXR1; oxidation resistance protein 1
D 104548782 oxidation resistance protein 1-like K25437 OXR1; oxidation resistance protein 1
D 104533250 MSTO1; protein misato homolog 1 K27320 MSTO1; protein misato
B Mitophagy factors
C Autophagy-related proteins
D 104537082 LOW QUALITY PROTEIN: autophagy-related protein 2 homolog B-like K17906 ATG2; autophagy-related protein 2
D 104541524 ATG3; ubiquitin-like-conjugating enzyme ATG3 K08343 ATG3; ubiquitin-like-conjugating enzyme ATG3
D 104536612 ATG4B; cysteine protease ATG4B K08342 ATG4; cysteine protease ATG4 [EC:3.4.22.-]
D 104548434 ATG4A; cysteine protease ATG4A K08342 ATG4; cysteine protease ATG4 [EC:3.4.22.-]
D 104548824 ATG4C; cysteine protease ATG4C K08342 ATG4; cysteine protease ATG4 [EC:3.4.22.-]
D 104539913 ATG5; autophagy protein 5 K08339 ATG5; autophagy-related protein 5
D 104534788 BECN1; beclin-1 K08334 BECN; beclin
D 104540602 ATG7; ubiquitin-like modifier-activating enzyme ATG7 K08337 ATG7; ubiquitin-like modifier-activating enzyme ATG7
D 104535364 GABARAPL2; gamma-aminobutyric acid receptor-associated protein-like 2 K08341 GABARAP; GABA(A) receptor-associated protein
D 104533507 gamma-aminobutyric acid receptor-associated protein-like 1 K08341 GABARAP; GABA(A) receptor-associated protein
D 104534538 LOW QUALITY PROTEIN: autophagy-related protein 9A-like K17907 ATG9; autophagy-related protein 9
D 104536128 ATG12; ubiquitin-like protein ATG12 K08336 ATG12; ubiquitin-like protein ATG12
D 104534653 ATG14; beclin 1-associated autophagy-related key regulator K17889 ATG14L; beclin 1-associated autophagy-related key regulator
D 104533952 ATG16L1; autophagy-related protein 16-1 isoform X1 K17890 ATG16L1; autophagy-related protein 16-1
D 104544393 WIPI1; WD repeat domain phosphoinositide-interacting protein 1 K17908 WIPI1_2; autophagy-related protein 18
D 104545410 WIPI2; WD repeat domain phosphoinositide-interacting protein 2 K17908 WIPI1_2; autophagy-related protein 18
D 104537974 vacuolar protein 8-like K08332 VAC8; vacuolar protein 8
C Parkin-dependent mechanism factors
D 104540181 RNF41; E3 ubiquitin-protein ligase NRDP1 K11981 RNF41; E3 ubiquitin-protein ligase NRDP1 [EC:2.3.2.27]
D 104542066 PINK1; serine/threonine-protein kinase PINK1, mitochondrial K05688 PINK1; PTEN induced putative kinase 1 [EC:2.7.11.1]
D 104534899 PARL; presenilins-associated rhomboid-like protein, mitochondrial isoform X1 K09650 PARL; rhomboid-like protein [EC:3.4.21.105]
D 104536546 SQSTM1; sequestosome-1 isoform X1 K14381 SQSTM1; sequestosome 1
D 104542213 TAX1BP1; tax1-binding protein 1 isoform X1 K21347 TAX1BP1; Tax1-binding protein 1
D 104539359 CALCOCO2; calcium-binding and coiled-coil domain-containing protein 2 K21348 CALCOCO2; calcium-binding and coiled-coil domain-containing protein 2
D 104538315 AMBRA1; activating molecule in BECN1-regulated autophagy protein 1 K17985 AMBRA1; activating molecule in BECN1-regulated autophagy protein 1
C Parkin-independent mechanism factors
D 104541263 ATG13; autophagy-related protein 13 isoform X1 K08331 ATG13; autophagy-related protein 13
D 104538658 ULK1; LOW QUALITY PROTEIN: serine/threonine-protein kinase ULK1 K21357 ULK1; serine/threonine-protein kinase ULK1 [EC:2.7.11.1]
D 104545019 ULK2; serine/threonine-protein kinase ULK2 K08269 ULK2; serine/threonine-protein kinase ULK2 [EC:2.7.11.1]
D 104539992 RB1CC1; RB1-inducible coiled-coil protein 1 K17589 RB1CC1; RB1-inducible coiled-coil protein 1
D 104539063 HSP90AB1; LOW QUALITY PROTEIN: heat shock protein HSP 90-beta K04079 HSP90A; molecular chaperone HtpG
D 104534415 heat shock protein HSP 90-alpha K04079 HSP90A; molecular chaperone HtpG
D 104539221 SMURF2; E3 ubiquitin-protein ligase SMURF2 K04678 SMURF; E3 ubiquitin ligase SMURF1/2 [EC:2.3.2.26]
D 104534219 SMURF1; E3 ubiquitin-protein ligase SMURF1 K04678 SMURF; E3 ubiquitin ligase SMURF1/2 [EC:2.3.2.26]
D 104547626 BNIP3L; BCL2/adenovirus E1B 19 kDa protein-interacting protein 3-like K15465 BNIP3L; BCL2/adenovirus E1B 19 kDa protein-interacting protein 3-like
D 104536669 FUN14 domain-containing protein 1-like K17986 FUNDC1; FUN14 domain-containing protein 1
C Other mitophagy factors
D 104534340 LOW QUALITY PROTEIN: 5'-AMP-activated protein kinase catalytic subunit alpha-1-like K07198 PRKAA; 5'-AMP-activated protein kinase, catalytic alpha subunit [EC:2.7.11.31]
D 104538944 PRKAA2; LOW QUALITY PROTEIN: 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 104539404 microtubule-associated proteins 1A/1B light chain 3C-like K10435 MAP1LC; microtubule-associated protein 1 light chain
D 104535282 microtubule-associated proteins 1A/1B light chain 3C-like K10435 MAP1LC; microtubule-associated protein 1 light chain
D 104534943 MAP1LC3B; microtubule-associated proteins 1A/1B light chain 3B K10435 MAP1LC; microtubule-associated protein 1 light chain
D 104533612 microtubule-associated proteins 1A/1B light chain 3A K10435 MAP1LC; microtubule-associated protein 1 light chain
D 104548043 NBR1; next to BRCA1 gene 1 protein K17987 NBR1; next to BRCA1 gene 1 protein
D 104547356 TECPR1; tectonin beta-propeller repeat-containing protein 1 K17988 TECPR1; tectonin beta-propeller repeat-containing protein 1
D 104546991 COASY; LOW QUALITY PROTEIN: bifunctional coenzyme A synthase K02318 COASY; phosphopantetheine adenylyltransferase / dephospho-CoA kinase [EC:2.7.7.3 2.7.1.24]
D 104540004 PLA2G6; 85/88 kDa calcium-independent phospholipase A2 K16343 PLA2G6; calcium-independent phospholipase A2 [EC:3.1.1.4]
D 104533176 protein C19orf12 homolog K23168 MPAN; protein MPAN
D 104538483 FUN14 domain-containing protein 2-like K24308 FUNDC2; FUN14 domain-containing protein 2
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#[ BRITE | KEGG2 | KEGG ]
#Last updated: March 14, 2024