SDHA Antibody

Complex II of the mitochondrial respiratory chain, also known as succinate dehydrogenase or succinate:ubiquinone oxidoreductase, consists of 4 nuclear-encoded polypeptides, these are the flavoprotein subunit (SDHA), the iron sulfur protein subunit (SDHB), and the integral membrane protein subunits SDHC and SDHD. SDHA is an acronym for succinate dehydrogenase complex subunit A. The succinate dehydrogenase (SDH) protein complex catalyzes the oxidation of succinate (succinate + ubiquinone => fumarate + ubiquinol) . The SDHA subunit is connected to the SDHB subunit on the hydrophilic, catalytic end of the complex, and weighs 72.7 kDA. Mutations in the SDHA subunit have a distinct pathology from mutations in the SDHB/SDHC/SDHD subunits; it is the only subunit to never have shown tumor suppressor behaviour. Heterozygous carriers of an SDHA mutation do not develop paragangliomas as has been seen for mutations in the other subunits. This appears to be due to the expression of two similar SDHA genes (Types I and II) in the paraganglia system.