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. Alternatively spliced transcript variants encoding different isoforms have been found for this gene.
Aliases: FP; PGL5; SDH1; SDH2; SDHF; CMD1GG; MC2DN1; NDAXOA
Entrez GeneID: 6389
Swissprot: P31040
WB Predicted band size: 72.7KDa
Host/Isotype: Mouse IgG2a
Species Reactivity: Human, Mouse
Immunogen: Purified recombinant fragment of human SDHA (AA: 516-665) expressed in E. Coli.