This gene encodes a major catalytic subunit of succinate-ubiquinone oxidoreductase, a complex of the mitochondrial respiratory chain. The complex is composed of four nuclear-encoded subunits and is localized in the mitochondrial inner membrane. Mutations in this gene have been associated with a form of mitochondrial respiratory chain deficiency known as Leigh Syndrome. A pseudogene has been identified on chromosome 3q29.
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Protein Aliases: Flavoprotein subunit of complex II; Fp; succinate dehydrogenase [ubiquinone] flavoprotein subunit; Succinate dehydrogenase [ubiquinone] flavoprotein subunit, mitochondrial; succinate dehydrogenase complex subunit A, flavoprotein (Fp); succinate dehydrogenase complex, subunit A, flavoprotein (Fp)
Gene Aliases: 1500032O14Rik; 2310034D06Rik; 4921513A11; C81073; CMD1GG; FP; PGL5; SDH1; SDH2; SDHA; SDHF