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| | CHCHD6-1 | MICOS complex subunit; Component of the MICOS complex, a large protein complex of the mitochondrial inner membrane that plays crucial roles in the maintenance of crista junctions, inner membrane architecture, and formation of contact sites to the outer membrane. (295 aa) |
| | SDHB | Succinate dehydrogenase [ubiquinone] iron-sulfur subunit, mitochondrial; Iron-sulfur protein (IP) subunit of succinate dehydrogenase (SDH) that is involved in complex II of the mitochondrial electron transport chain and is responsible for transferring electrons from succinate to ubiquinone (coenzyme Q). (277 aa) |
| | SLC25A34 | Solute carrier family 25 member 34; Belongs to the mitochondrial carrier (TC 2.A.29) family. (179 aa) |
| | PINK1 | Serine/threonine-protein kinase PINK1, mitochondrial; Belongs to the arginase family. (851 aa) |
| | MINOS1 | MICOS complex subunit MIC10; Component of the MICOS complex, a large protein complex of the mitochondrial inner membrane that plays crucial roles in the maintenance of crista junctions, inner membrane architecture, and formation of contact sites to the outer membrane. (77 aa) |
| | CCDC109B | Calcium uniporter regulatory subunit MCUb, mitochondrial. (330 aa) |
| | DNAJC15 | DnaJ-like protein subfamily C member 15. (145 aa) |
| | COX20 | Protein FAM36A isoform A. (181 aa) |
| | Y1Q_0013291 | Death ligand signal enhancer. (483 aa) |
| | NDUFAF5-1 | NADH dehydrogenase [ubiquinone] 1 alpha subcomplex assembly factor 5 isoform B. (338 aa) |
| | COX6B2 | Cytochrome c oxidase subunit 6B2. (107 aa) |
| | PMPCA | Mitochondrial-processing peptidase subunit alpha; Belongs to the peptidase M16 family. (525 aa) |
| | CUNH3orf18 | Uncharacterized protein. (193 aa) |
| | ATP5O | ATP synthase subunit O, mitochondrial. (82 aa) |
| | ATP5J | ATP synthase-coupling factor 6, mitochondrial; Mitochondrial membrane ATP synthase (F(1)F(0) ATP synthase or Complex V) produces ATP from ADP in the presence of a proton gradient across the membrane which is generated by electron transport complexes of the respiratory chain; Belongs to the eukaryotic ATPase subunit F6 family. (113 aa) |
| | MPC2 | Mitochondrial pyruvate carrier; Mediates the uptake of pyruvate into mitochondria. Belongs to the mitochondrial pyruvate carrier (MPC) (TC 2.A.105) family. (133 aa) |
| | NDUFB4 | NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 4. (134 aa) |
| | PHB2 | Prohibitin. (291 aa) |
| | AMPD3 | AMP deaminase; Belongs to the metallo-dependent hydrolases superfamily. Adenosine and AMP deaminases family. (809 aa) |
| | IMMP1L | Mitochondrial inner membrane protease subunit. (200 aa) |
| | NDUFS3 | NADH dehydrogenase [ubiquinone] iron-sulfur protein 3, mitochondrial; Belongs to the complex I 30 kDa subunit family. (246 aa) |
| | COX16 | COX16 cytochrome c oxidase assembly-like protein. (101 aa) |
| | Y1Q_0001631 | ATP synthase subunit O, mitochondrial-like. (85 aa) |
| | CUNH15orf48 | Normal mucosa of esophagus-specific 1 protein. (87 aa) |
| | SQRDL | Sulfide:quinone oxidoreductase, mitochondrial. (476 aa) |
| | PGPEP1L | Pyroglutamyl-peptidase 1-like protein isoform A. (200 aa) |
| | UQCRQ | Cytochrome b-c1 complex subunit 8. (82 aa) |
| | GRPEL2 | GrpE protein-like protein 2, mitochondrial; Belongs to the GrpE family. (223 aa) |
| | NDUFB5 | NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 5, mitochondrial. (154 aa) |
| | DNAJC19 | Mitochondrial import inner membrane translocase subunit TIM14. (114 aa) |
| | TIMM17B | Mitochondrial import inner membrane translocase subunit Tim17-B isoform A. (150 aa) |
| | OMA1 | Metalloendopeptidase OMA1, mitochondrial. (504 aa) |
| | MPC1 | Mitochondrial pyruvate carrier; Mediates the uptake of pyruvate into mitochondria. Belongs to the mitochondrial pyruvate carrier (MPC) (TC 2.A.105) family. (93 aa) |
| | TMEM242 | Transmembrane protein 242. (142 aa) |
| | SLC25A12 | Calcium-binding mitochondrial carrier protein Aralar1; Belongs to the mitochondrial carrier (TC 2.A.29) family. (677 aa) |
| | DNAJC11 | DnaJ-like protein subfamily C member 11. (559 aa) |
| | NDUFA10 | NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 10, mitochondrial; Accessory subunit of the mitochondrial membrane respiratory chain NADH dehydrogenase (Complex I), that is believed not to be involved in catalysis. Complex I functions in the transfer of electrons from NADH to the respiratory chain. The immediate electron acceptor for the enzyme is believed to be ubiquinone. (357 aa) |
| | TIMM17A | Mitochondrial import inner membrane translocase subunit TIM17; Essential component of the TIM23 complex, a complex that mediates the translocation of transit peptide-containing proteins across the mitochondrial inner membrane. (166 aa) |
| | TTC19 | Tetratricopeptide repeat protein 19, mitochondrial. (227 aa) |
| | COQ8B | ABC1 domain-containing protein. (365 aa) |
| | LETM2 | LETM1 domain-containing protein LETM2, mitochondrial. (511 aa) |
| | NDUFA7-1 | NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 7 isoform B. (223 aa) |
| | TIMM44 | Mitochondrial import inner membrane translocase subunit TIM44; Essential component of the PAM complex, a complex required for the translocation of transit peptide-containing proteins from the inner membrane into the mitochondrial matrix in an ATP-dependent manner; Belongs to the Tim44 family. (447 aa) |
| | SLC25A15 | Mitochondrial ornithine transporter 1 isoform B; Belongs to the mitochondrial carrier (TC 2.A.29) family. (313 aa) |
| | NDUFS6 | NADH dehydrogenase [ubiquinone] iron-sulfur protein 6, mitochondrial. (150 aa) |
| | FAM173B | Protein FAM173B. (229 aa) |
| | Y1Q_0018516 | Ig-like domain-containing protein. (208 aa) |
| | ATP5I | ATP synthase subunit e, mitochondrial. (71 aa) |
| | NDUFB6 | NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 6. (128 aa) |
| | TYMS | Thymidylate synthase. (304 aa) |
| | NDUFV2 | NADH dehydrogenase [ubiquinone] flavoprotein 2, mitochondrial. (245 aa) |
| | TIMM21 | Mitochondrial import inner membrane translocase subunit Tim21. (187 aa) |
| | AFG3L2 | AFG3-like protein 2 isoform B. (808 aa) |
| | NDUFAF3 | NADH dehydrogenase [ubiquinone] 1 alpha subcomplex assembly factor 3. (153 aa) |
| | SDHD | Succinate dehydrogenase [ubiquinone] cytochrome b small subunit; Membrane-anchoring subunit of succinate dehydrogenase (SDH) that is involved in complex II of the mitochondrial electron transport chain and is responsible for transferring electrons from succinate to ubiquinone (coenzyme Q); Belongs to the CybS family. (163 aa) |
| | FOXRED1 | FAD-dependent oxidoreductase domain-containing protein 1. (458 aa) |
| | SMDT1 | Essential MCU regulator, mitochondrial. (93 aa) |
| | NDUFA6 | NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 6; Accessory subunit of the mitochondrial membrane respiratory chain NADH dehydrogenase (Complex I), that is believed to be not involved in catalysis. Complex I functions in the transfer of electrons from NADH to the respiratory chain. The immediate electron acceptor for the enzyme is believed to be ubiquinone. (131 aa) |
| | SLC25A18 | Mitochondrial glutamate carrier 2 isoform B; Belongs to the mitochondrial carrier (TC 2.A.29) family. (323 aa) |
| | LDHB | L-lactate dehydrogenase; Belongs to the LDH/MDH superfamily. (375 aa) |
| | COQ6 | Ubiquinone biosynthesis monooxygenase COQ6, mitochondrial; FAD-dependent monooxygenase required for the C5-ring hydroxylation during ubiquinone biosynthesis. Catalyzes the hydroxylation of 3-polyprenyl-4-hydroxybenzoic acid to 3-polyprenyl- 4,5-dihydroxybenzoic acid. The electrons required for the hydroxylation reaction may be funneled indirectly from NADPH via a ferredoxin/ferredoxin reductase system to COQ6. (424 aa) |
| | HEATR5A-2 | Uncharacterized protein. (2030 aa) |
| | APOOL | MICOS complex subunit; Component of the MICOS complex, a large protein complex of the mitochondrial inner membrane that plays crucial roles in the maintenance of crista junctions, inner membrane architecture, and formation of contact sites to the outer membrane. (326 aa) |
| | Y1Q_0015686 | Uncharacterized protein. (282 aa) |
| | ABCB7 | ATP-binding cassette sub-family B member 7, mitochondrial. (724 aa) |
| | CHCHD3-1 | MICOS complex subunit; Component of the MICOS complex, a large protein complex of the mitochondrial inner membrane that plays crucial roles in the maintenance of crista junctions, inner membrane architecture, and formation of contact sites to the outer membrane. (246 aa) |
| | NDUFV3-1 | NADH dehydrogenase [ubiquinone] flavoprotein 3, mitochondrial isoform B. (136 aa) |
| | Y1Q_0006674 | Mitochondrial ornithine transporter 1-like; Belongs to the mitochondrial carrier (TC 2.A.29) family. (309 aa) |
| | OPA1 | Dynamin-like protein, mitochondrial isoform A; Belongs to the TRAFAC class dynamin-like GTPase superfamily. Dynamin/Fzo/YdjA family. (905 aa) |
| | COQ3 | Ubiquinone biosynthesis O-methyltransferase, mitochondrial; O-methyltransferase that catalyzes the 2 O-methylation steps in the ubiquinone biosynthetic pathway; Belongs to the class I-like SAM-binding methyltransferase superfamily. UbiG/COQ3 family. (341 aa) |
| | TIMM29 | Uncharacterized protein. (243 aa) |
| | UQCC | Ubiquinol-cytochrome c reductase complex chaperone. (302 aa) |
| | PRODH2 | Proline dehydrogenase; Converts proline to delta-1-pyrroline-5-carboxylate. (449 aa) |
| | ROMO1 | Reactive oxygen species modulator 1. (102 aa) |
| | NDUFB11 | NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 11, mitochondrial. (93 aa) |
| | FAM83C | Protein FAM83C. (581 aa) |
| | SLC25A22 | Mitochondrial glutamate carrier 1; Belongs to the mitochondrial carrier (TC 2.A.29) family. (320 aa) |
| | CUNH20orf24 | Uncharacterized protein. (135 aa) |
| | MCU | Calcium uniporter protein, mitochondrial. (309 aa) |
| | MICU1 | Calcium uptake protein 1, mitochondrial. (526 aa) |
| | CUNH19orf70 | MICOS complex subunit MIC13; Component of the MICOS complex, a large protein complex of the mitochondrial inner membrane that plays crucial roles in the maintenance of crista junctions, inner membrane architecture, and formation of contact sites to the outer membrane. (111 aa) |
| | COQ2 | 4-hydroxybenzoate polyprenyltransferase, mitochondrial. (200 aa) |
| | Y1Q_0020518 | Cytochrome c oxidase subunit 6A2, mitochondrial; Belongs to the cytochrome c oxidase subunit 6A family. (107 aa) |
| | COA1 | Cytochrome c oxidase assembly factor 1-like protein. (179 aa) |
| | ACAD10 | Acyl-CoA dehydrogenase family member 10. (779 aa) |
| | SLC25A38 | Mitochondrial glycine transporter; Mitochondrial glycine transporter that imports glycine into the mitochondrial matrix. Plays an important role in providing glycine for the first enzymatic step in heme biosynthesis, the condensation of glycine with succinyl-CoA to produce 5-aminolevulinate (ALA) in the miochondrial matrix. Required during erythropoiesis. Belongs to the mitochondrial carrier (TC 2.A.29) family. SLC25A38 subfamily. (320 aa) |
| | ADCK3 | Atypical kinase ADCK3, mitochondrial. (682 aa) |
| | ATP5SL | ATP synthase subunit s-like protein. (202 aa) |
| | SPG7 | 60S ribosomal protein L13; Belongs to the eukaryotic ribosomal protein eL13 family. (1069 aa) |
| | SLC25A34L | Solute carrier family 25 member 34; Belongs to the mitochondrial carrier (TC 2.A.29) family. (300 aa) |
| | PHB | Prohibitin. (273 aa) |
| | NDUFA9 | NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 9, mitochondrial. (382 aa) |
| | ATP5D | ATP synthase subunit delta, mitochondrial. (137 aa) |
| | Y1Q_0022959 | EF-hand domain-containing protein. (115 aa) |
| | SCO1 | SCO1-like protein, mitochondrial. (256 aa) |
| | MICU2 | Calcium uptake protein 2, mitochondrial. (388 aa) |
| | BDH1 | D-beta-hydroxybutyrate dehydrogenase, mitochondrial; Belongs to the short-chain dehydrogenases/reductases (SDR) family. (372 aa) |
| | ACADVL | Very long-chain specific acyl-CoA dehydrogenase, mitochondrial. (631 aa) |
| | IMMT | MICOS complex subunit MIC60; Component of the MICOS complex, a large protein complex of the mitochondrial inner membrane that plays crucial roles in the maintenance of crista junctions, inner membrane architecture, and formation of contact sites to the outer membrane. (807 aa) |
| | LETM1 | LETM1 and EF-hand domain-containing protein 1, mitochondrial. (754 aa) |
| | GRPEL1 | GrpE protein homolog; Essential component of the PAM complex, a complex required for the translocation of transit peptide-containing proteins from the inner membrane into the mitochondrial matrix in an ATP-dependent manner; Belongs to the GrpE family. (218 aa) |
| | SMIM20 | Small integral membrane protein 20. (67 aa) |
| | MICU3 | Palmitoyltransferase; Belongs to the DHHC palmitoyltransferase family. (869 aa) |
| | ETFDH | Electron transfer flavoprotein-ubiquinone oxidoreductase; Accepts electrons from ETF and reduces ubiquinone. (618 aa) |
| | CCDC56 | Cytochrome c oxidase assembly factor 3-like protein, mitochondrial. (100 aa) |
| | YME1L1 | ATP-dependent zinc metalloprotease YME1L1 isoform A. (724 aa) |
| | HIGD1B | HIG1 domain family member 1B isoform A. (98 aa) |
| | NDUFA5 | NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 5. (116 aa) |
| | IMMP2LL | Mitochondrial inner membrane protease subunit 2. (90 aa) |
| | IMMP2L | Mitochondrial inner membrane protease subunit 2 isoform A. (129 aa) |
| | XRCC6BP1 | Mitochondrial inner membrane protease ATP23; Belongs to the peptidase M76 family. (193 aa) |
| | TYMP | Thymidine phosphorylase. (275 aa) |
| | NDUFS7 | NADH dehydrogenase [ubiquinone] iron-sulfur protein 7, mitochondrial; Belongs to the complex I 20 kDa subunit family. (204 aa) |
| | ATP5L | ATP synthase subunit g, mitochondrial. (163 aa) |
| | MTG1 | Mitochondrial GTPase 1; Plays a role in the regulation of the mitochondrial ribosome assembly and of translational activity. Displays mitochondrial GTPase activity; Belongs to the TRAFAC class YlqF/YawG GTPase family. MTG1 subfamily. (315 aa) |
| | STOML2 | Stomatin-like protein 2, mitochondrial. (326 aa) |
| | Y1Q_0017874 | Tetraspanin; Belongs to the tetraspanin (TM4SF) family. (478 aa) |
| | FH | Succinate dehydrogenase [ubiquinone] flavoprotein subunit, mitochondrial; Flavoprotein (FP) subunit of succinate dehydrogenase (SDH) that is involved in complex II of the mitochondrial electron transport chain and is responsible for transferring electrons from succinate to ubiquinone (coenzyme Q). (1165 aa) |
| | UCP3 | Mitochondrial uncoupling protein 3; Belongs to the mitochondrial carrier (TC 2.A.29) family. (760 aa) |
| | TAMM41 | Phosphatidate cytidylyltransferase, mitochondrial; Catalyzes the formation of CDP-diacylglycerol (CDP-DAG) from phosphatidic acid (PA) in the mitochondrial inner membrane. Required for the biosynthesis of the dimeric phospholipid cardiolipin, which stabilizes supercomplexes of the mitochondrial respiratory chain in the mitochondrial inner membrane. (330 aa) |
| | NDUFA4L2 | NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 4-like 2. (146 aa) |
| | HSP75 | Heat shock protein, mitochondrial. (708 aa) |
| | SLC25A26 | S-adenosylmethionine mitochondrial carrier protein; Belongs to the mitochondrial carrier (TC 2.A.29) family. (267 aa) |
| | SLC25A20 | Mitochondrial carnitine/acylcarnitine carrier protein; Belongs to the mitochondrial carrier (TC 2.A.29) family. (326 aa) |
| | NDUFB8 | NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 8, mitochondrial; Accessory subunit of the mitochondrial membrane respiratory chain NADH dehydrogenase (Complex I), that is believed not to be involved in catalysis. Complex I functions in the transfer of electrons from NADH to the respiratory chain. The immediate electron acceptor for the enzyme is believed to be ubiquinone. (184 aa) |
| | NDUFA13 | NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 13. (144 aa) |
| | TMEM70 | Transmembrane protein 70, mitochondrial. (222 aa) |
| | MCUR1 | Mitochondrial calcium uniporter regulator 1. (304 aa) |
| | ACAD9L | Acyl-CoA dehydrogenase family member 9, mitochondrial. (651 aa) |
| | SLC25A13 | Uncharacterized protein; Belongs to the mitochondrial carrier (TC 2.A.29) family. (497 aa) |
| | NDUFA4 | Cytochrome c oxidase subunit NDUFA4. (82 aa) |
| | TCTN3 | Tectonic-3. (488 aa) |
| | COQ9 | Ubiquinone biosynthesis protein; Lipid-binding protein involved in the biosynthesis of coenzyme Q, also named ubiquinone, an essential lipid-soluble electron transporter for aerobic cellular respiration. (311 aa) |
| | GHITM | Growth hormone-inducible transmembrane protein; Belongs to the BI1 family. (275 aa) |
| | TIMM23 | Mitochondrial import inner membrane translocase subunit TIM23; Essential component of the TIM23 complex, a complex that mediates the translocation of transit peptide-containing proteins across the mitochondrial inner membrane. (207 aa) |
| | Y1Q_0020984 | Uncharacterized protein. (152 aa) |
| | ATP5F1 | ATP synthase F(0) complex subunit B1, mitochondrial. (252 aa) |
| | COX7A2 | Cytochrome c oxidase subunit 7A2, mitochondrial. (89 aa) |
| | SLC25A10 | Mitochondrial dicarboxylate carrier; Belongs to the mitochondrial carrier (TC 2.A.29) family. (286 aa) |
| | FECH | Ferrochelatase; Catalyzes the ferrous insertion into protoporphyrin IX. (412 aa) |
| | NDUFS4 | NADH dehydrogenase [ubiquinone] iron-sulfur protein 4, mitochondrial. (165 aa) |
| | FAM83A | Protein FAM83A. (441 aa) |
| | NDUFAF6-1 | NADH dehydrogenase (Ubiquinone) complex I, assembly factor 6 isoform B. (278 aa) |
| | COX6C | Cytochrome c oxidase subunit 6C. (105 aa) |
| | SLC25A32 | Mitochondrial folate transporter/carrier; Belongs to the mitochondrial carrier (TC 2.A.29) family. (323 aa) |
| | PRODH | Proline dehydrogenase; Converts proline to delta-1-pyrroline-5-carboxylate. (585 aa) |
| | CHDH | Choline dehydrogenase, mitochondrial isoform A. (428 aa) |
| | ACAD9 | Acyl-CoA dehydrogenase family member 9, mitochondrial. (611 aa) |
| | APOO | MICOS complex subunit; Component of the MICOS complex, a large protein complex of the mitochondrial inner membrane that plays crucial roles in the maintenance of crista junctions, inner membrane architecture, and formation of contact sites to the outer membrane. (233 aa) |
| | COQ7 | 5-demethoxyubiquinone hydroxylase, mitochondrial; Catalyzes the hydroxylation of 2-polyprenyl-3-methyl-6- methoxy-1,4-benzoquinol (DMQH2) during ubiquinone biosynthesis. Has also a structural role in the COQ enzyme complex, stabilizing other COQ polypeptides. Involved in lifespan determination in a ubiquinone- independent manner. (207 aa) |
| | UQCRC2 | Cytochrome b-c1 complex subunit 2, mitochondrial. (457 aa) |
| | TIMM50 | Mitochondrial import inner membrane translocase subunit TIM50; Essential component of the TIM23 complex, a complex that mediates the translocation of transit peptide-containing proteins across the mitochondrial inner membrane. (338 aa) |
| | COX11 | Cytochrome c oxidase assembly protein COX11, mitochondrial. (171 aa) |
| | ATP5H | ATP synthase subunit d, mitochondrial; Mitochondrial membrane ATP synthase (F(1)F(0) ATP synthase or Complex V) produces ATP from ADP in the presence of a proton gradient across the membrane which is generated by electron transport complexes of the respiratory chain. F-type ATPases consist of two structural domains, F(1) - containing the extramembraneous catalytic core, and F(0) - containing the membrane proton channel, linked together by a central stalk and a peripheral stalk. During catalysis, ATP synthesis in the catalytic domain of F(1) is coupled via a rotary mechanism of the cent [...] (185 aa) |
| | NDUFV1 | NADH dehydrogenase [ubiquinone] flavoprotein 1, mitochondrial. (792 aa) |
| | SLC8B1 | Sodium/potassium/calcium exchanger 6, mitochondrial; Belongs to the Ca(2+):cation antiporter (CaCA) (TC 2.A.19) family. (468 aa) |
| | MTFP1 | Mitochondrial fission process protein 1. (167 aa) |
| | Y1Q_0022874 | Acyl-CoA dehydrogenase family member 11-like. (630 aa) |
| | DUSP18 | Dual specificity protein phosphatase 18; Belongs to the protein-tyrosine phosphatase family. Non- receptor class dual specificity subfamily. (188 aa) |
| | COX6A1 | Cytochrome c oxidase subunit 6A, mitochondrial. (95 aa) |
| | COX18 | Mitochondrial inner membrane protein COX18. (267 aa) |
| | Y1Q_0022847 | Uncharacterized protein. (144 aa) |
| | PAM16 | Mitochondrial import inner membrane translocase subunit TIM16. (125 aa) |
| | CUNH19orf43 | Uncharacterized protein. (118 aa) |
| | Y1Q_0017325 | Peptidase_M41 domain-containing protein. (125 aa) |
| | AMPD2 | AMP deaminase; Belongs to the metallo-dependent hydrolases superfamily. Adenosine and AMP deaminases family. (857 aa) |
| | NDUFS1 | NADH-ubiquinone oxidoreductase subunit, mitochondrial; Belongs to the complex I 75 kDa subunit family. (766 aa) |
| | MPV17L2 | Mpv17-like protein 2; Belongs to the peroxisomal membrane protein PXMP2/4 family. (148 aa) |
