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UQCRC1 | ubiquinol-cytochrome c reductase core protein I; 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 (480 aa) | |||
NDUFA10 | NADH dehydrogenase (ubiquinone) 1 alpha subcomplex, 10, 42kDa; 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 (355 aa) | |||
FAH | fumarylacetoacetate hydrolase (fumarylacetoacetase) (419 aa) | |||
SDHA | succinate dehydrogenase complex, subunit A, flavoprotein (Fp); 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). Can act as a tumor suppressor (664 aa) | |||
UQCRC2 | ubiquinol-cytochrome c reductase core protein II; 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 (453 aa) | |||
OXA1L | oxidase (cytochrome c) assembly 1-like; Required for the insertion of integral membrane proteins into the mitochondrial inner membrane. Essential for the activity and assembly of cytochrome oxidase. Required for the correct biogenesis of ATP synthase and complex I in mitochondria (495 aa) | |||
UQCRB | ubiquinol-cytochrome c reductase binding protein; 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 (111 aa) | |||
CYCS | cytochrome c, somatic; 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 (By similarity) (105 aa) | |||
UQCRH | ubiquinol-cytochrome c reductase hinge protein; 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 (91 aa) | |||
CYC1 | cytochrome c-1; 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 (325 aa) | |||
UQCR10 | ubiquinol-cytochrome c reductase, complex III subunit X; 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) (63 aa) | |||
UBC | ubiquitin C (685 aa) | |||
MT-CYB | mitochondrially encoded cytochrome b; Component of the ubiquinol-cytochrome c reductase complex (complex III or cytochrome b-c1 complex), which is a respiratory chain that generates an electrochemical potential coupled to ATP synthesis (By similarity) (380 aa) | |||
FH | fumarate hydratase; Also acts as a tumor suppressor (510 aa) | |||
SDHC | succinate dehydrogenase complex, subunit C, integral membrane protein, 15kDa; 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) (169 aa) | |||
HOGA1 | 4-hydroxy-2-oxoglutarate aldolase 1; Catalyzes the final step in the metabolic pathway of hydroxyproline (Probable) (327 aa) | |||
SDHB | succinate dehydrogenase complex, subunit B, iron sulfur (Ip); 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) (280 aa) | |||
SDHD | succinate dehydrogenase complex, subunit D, integral membrane protein; 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) (By similarity) (159 aa) | |||
FXN | frataxin; Promotes the biosynthesis of heme and assembly and repair of iron-sulfur clusters by delivering Fe(2+) to proteins involved in these pathways. May play a role in the protection against iron-catalyzed oxidative stress through its ability to catalyze the oxidation of Fe(2+) to Fe(3+); the oligomeric form but not the monomeric form has in vitro ferroxidase activity. May be able to store large amounts of iron in the form of a ferrihydrite mineral by oligomerization; however, the physiological relevance is unsure as reports are conflicting and the function has only been shown usin [...] (210 aa) | |||
SUCLA2 | succinate-CoA ligase, ADP-forming, beta subunit; Catalyzes the ATP-dependent ligation of succinate and CoA to form succinyl-CoA (By similarity) (463 aa) | |||
UQCRQ | ubiquinol-cytochrome c reductase, complex III subunit VII, 9.5kDa; 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 (82 aa) | |||
FAHD1 | fumarylacetoacetate hydrolase domain containing 1; Probable mitochondrial acylpyruvase which is able to hydrolyze acetylpyruvate and fumarylpyruvate in vitro (248 aa) | |||
SUCLG1 | succinate-CoA ligase, alpha subunit; Catalyzes the ATP- or GTP-dependent ligation of succinate and CoA to form succinyl-CoA. The nature of the beta subunit determines the nucleotide specificity (By similarity) (346 aa) | |||
ENSG00000249319 | Uncharacterized protein (293 aa) | |||
SUCLG2 | succinate-CoA ligase, GDP-forming, beta subunit; Catalyzes the GTP-dependent ligation of succinate and CoA to form succinyl-CoA (By similarity) (440 aa) | |||
ENSG00000255292 | Uncharacterized protein (140 aa) |