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SDHA is the catalytic subunit of succinate dehydrogenase (SDH) complex II of the mitochondrial electron transport chain. Complex II contains four subunits: the flavoprotein catalytic subunit SDHA, iron-sulfur protein SDHB, and a cytochrome b560 composed of SDHC and SDHD. Interaction with SDH5 is required for FAD attachment. Responsible for transferring electrons from succinate to ubiquinone (coenzyme Q). Defects in SDHA cause defective mitochondrial oxidative phosphorylation, giving rise to heterogeneous clinical symptoms ranging from isolated organ dysfunction to multisystem disorder. Acetylation of SDHA may control entry of the substrate into the active site, thus regulating its enzymatic activity. Acetylated SDHA may be a SIRT3 substrate SIRT3 is a mitochondrial NAD(+)-dependent deacetylase. Increased succinate levels as a consequence of SDH deficiency inhibit hypoxia inducible factor-1alpha (HIF-1alpha) prolyl hydroxylases leading to sustained HIF-1alpha expression in tumours. Defects in SDHA cause of Leigh syndrome, a severe disorder characterized by bilaterally symmetrical necrotic lesions in subcortical brain regions. Note: This description may include information from UniProtKB.
Protein type: Carbohydrate Metabolism - citrate (TCA) cycle; EC; Energy Metabolism - oxidative phosphorylation; Mitochondrial; Oxidoreductase; Tumor suppressor
Chromosomal Location of Human Ortholog: 5p15.33
Cellular Component: mitochondrial inner membrane; mitochondrial respiratory chain complex II; mitochondrion; myelin sheath; nucleolus
Molecular Function: electron transfer activity; FAD binding; protein binding; succinate dehydrogenase (ubiquinone) activity; succinate dehydrogenase activity
Biological Process: anaerobic respiration; mitochondrial electron transport, succinate to ubiquinone; nervous system development; succinate metabolic process; tricarboxylic acid cycle
Disease: Cardiomyopathy, Dilated, 1gg; Leigh Syndrome; Mitochondrial Complex Ii Deficiency; Paragangliomas 5
Reference #:  P31040 (UniProtKB)
Alt. Names/Synonyms: DHSA; Flavoprotein subunit of complex II; Fp; FpSDH; SDH1; SDH2; SDHA; SDHF; Succinate dehydrogenase [ubiquinone] flavoprotein subunit, mitochondrial; succinate dehydrogenase complex flavoprotein subunit; succinate dehydrogenase complex, subunit A, flavoprotein (Fp)
Gene Symbols: SDHA
Molecular weight: 72,692 Da
Basal Isoelectric point: 7.06  Predict pI for various phosphorylation states
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