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| UQCRC1 | Cytochrome b-c1 complex subunit 1, mitochondrial; This is a component of the ubiquinol-cytochrome c reductase complex (complex III or cytochrome b-c1 complex), which is part of the mitochondrial respiratory chain. This protein may mediate formation of the complex between cytochromes c and c1; M16 metallopeptidases (480 aa) | |||
| PMPCB | Mitochondrial-processing peptidase subunit beta; Cleaves presequences (transit peptides) from mitochondrial protein precursors; M16 metallopeptidases (489 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; NADH-ubiquinone oxidoreductase supernumerary subunits (355 aa) | |||
| DGUOK | Deoxyguanosine kinase, mitochondrial; Phosphorylates deoxyguanosine and deoxyadenosine in the mitochondrial matrix, with the highest efficiency for deoxyguanosine. In non-replicating cells, where cytosolic dNTP synthesis is down-regulated, mtDNA synthesis depends solely on DGUOK and TK2. Phosphorylates certain nucleoside analogs. Widely used as target of antiviral and chemotherapeutic agents (277 aa) | |||
| NDUFA9 | NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 9, 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; NADH-ubiquinone oxidoreductase supernumerary subunits (377 aa) | |||
| UQCRC2 | Cytochrome b-c1 complex subunit 2, mitochondrial; This is a component of the ubiquinol-cytochrome c reductase complex (complex III or cytochrome b-c1 complex), which is part of the mitochondrial respiratory chain. The core protein 2 is required for the assembly of the complex; M16 metallopeptidases (453 aa) | |||
| NDUFS6 | NADH dehydrogenase [ubiquinone] iron-sulfur protein 6, 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 (124 aa) | |||
| NDUFB11 | NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 11, 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; NADH-ubiquinone oxidoreductase supernumerary subunits (163 aa) | |||
| DCK | Deoxycytidine kinase; Required for the phosphorylation of the deoxyribonucleosides deoxycytidine (dC), deoxyguanosine (dG) and deoxyadenosine (dA). Has broad substrate specificity, and does not display selectivity based on the chirality of the substrate. It is also an essential enzyme for the phosphorylation of numerous nucleoside analogs widely employed as antiviral and chemotherapeutic agents; Belongs to the DCK/DGK family (260 aa) | |||
| TK2 | Thymidine kinase 2, mitochondrial; Phosphorylates thymidine, deoxycytidine, and deoxyuridine in the mitochondrial matrix. In non-replicating cells, where cytosolic dNTP synthesis is down-regulated, mtDNA synthesis depends solely on TK2 and DGUOK. Widely used as target of antiviral and chemotherapeutic agents; Belongs to the DCK/DGK family (265 aa) | |||
| UQCRFS1 | Cytochrome b-c1 complex subunit Rieske, mitochondrial; Cytochrome b-c1 complex subunit Rieske, mitochondrial- Component of the mitochondrial ubiquinol-cytochrome c reductase complex dimer (complex III dimer), which is a respiratory chain that generates an electrochemical potential coupled to ATP synthesis. Incorporation of UQCRFS1 is the penultimate step in complex III assembly (By similarity) (274 aa) | |||
| CYCS | Cytochrome c; Electron carrier protein. The oxidized form of the cytochrome c heme group can accept an electron from the heme group of the cytochrome c1 subunit of cytochrome reductase. Cytochrome c then transfers this electron to the cytochrome oxidase complex, the final protein carrier in the mitochondrial electron-transport chain (105 aa) | |||
| CYC1 | Cytochrome c1, heme protein, mitochondrial; This is the heme-containing component of the cytochrome b-c1 complex, which accepts electrons from Rieske protein and transfers electrons to cytochrome c in the mitochondrial respiratory chain; Apoptosome (325 aa) | |||
| COX5A | Cytochrome c oxidase subunit 5A, mitochondrial; This is the heme A-containing chain of cytochrome c oxidase, the terminal oxidase in mitochondrial electron transport (150 aa) | |||
| UQCR10 | Cytochrome b-c1 complex subunit 9; This is a component of the ubiquinol-cytochrome c reductase complex (complex III or cytochrome b-c1 complex), which is part of the mitochondrial respiratory chain. This subunit interacts with cytochrome c1 (By similarity); Belongs to the UQCR10/QCR9 family (63 aa) | |||
| MT-CYB | Cytochrome b; Component of the ubiquinol-cytochrome c reductase complex (complex III or cytochrome b-c1 complex) that is part of the mitochondrial respiratory chain. The b-c1 complex mediates electron transfer from ubiquinol to cytochrome c. Contributes to the generation of a proton gradient across the mitochondrial membrane that is then used for ATP synthesis (380 aa) | |||
| PMPCA | Mitochondrial-processing peptidase subunit alpha; Cleaves presequences (transit peptides) from mitochondrial protein precursors; Belongs to the peptidase M16 family (525 aa) | |||
| UQCRQ | Cytochrome b-c1 complex subunit 8; This is a component of the ubiquinol-cytochrome c reductase complex (complex III or cytochrome b-c1 complex), which is part of the mitochondrial respiratory chain. This subunit, together with cytochrome b, binds to ubiquinone; Belongs to the UQCRQ/QCR8 family (82 aa) | |||
| NDUFA11 | NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 11; 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; NADH-ubiquinone oxidoreductase supernumerary subunits (228 aa) | |||
| BCS1L | Mitochondrial chaperone BCS1; Chaperone necessary for the assembly of mitochondrial respiratory chain complex III. Plays an important role in the maintenance of mitochondrial tubular networks, respiratory chain assembly and formation of the LETM1 complex; AAA ATPases (419 aa) | |||
| NDUFA5 | NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 5; 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 (116 aa) | |||
| UQCRB | Cytochrome b-c1 complex subunit 7; This is a component of the ubiquinol-cytochrome c reductase complex (complex III or cytochrome b-c1 complex), which is part of the mitochondrial respiratory chain. This component is involved in redox-linked proton pumping; Belongs to the UQCRB/QCR7 family (140 aa) | |||
| NDUFAB1 | Acyl carrier protein, mitochondrial; Carrier of the growing fatty acid chain in fatty acid biosynthesis (By similarity). Accessory and non-catalytic subunit of the mitochondrial membrane respiratory chain NADH dehydrogenase (Complex I), which functions in the transfer of electrons from NADH to the respiratory chain; Belongs to the acyl carrier protein (ACP) family (156 aa) | |||
| ENSG00000267740 | Uncharacterized protein (169 aa) | |||
| UQCR11 | Cytochrome b-c1 complex subunit 10; This is a component of the ubiquinol-cytochrome c reductase complex (complex III or cytochrome b-c1 complex), which is part of the mitochondrial respiratory chain; Belongs to the UQCR11/QCR10 family (56 aa) | |||
| UQCRHL | Cytochrome b-c1 complex subunit 6-like, mitochondrial; May be a component of the ubiquinol-cytochrome c reductase complex (complex III or cytochrome b-c1 complex), which is part of the mitochondrial respiratory chain. This protein may mediate formation of the complex between cytochromes c and c1 (91 aa) | |||
