Description_x000D_ Biochem/physiol Actions_x000D_ PDHA1 pyruvate dehydrogenase (lipoamide) alpha 1 is a member of the pyruvate dehydrogenase (PDH) complex. PDHA1 is a nuclear-encoded mitochondrial multienzyme complex which catalyzes the overall conversion of pyruvate to acetyl-CoA and CO(2), and provides the primary link between glycolysis and the tricarboxylic acid (TCA) cycle . PDHA1 plays a critical role in the brain that usually obtains all of its energy from the aerobic oxidation of glucose. Mutations in the PDHA1 are associated with pyruvate dehydrogenase E1-alpha deficiency and X-linked Leigh syndrome._x000D_ Physical form_x000D_ Supplied in 50mM sodium phosphate, pH 7.0, 300mM NaCl, 150mM imidazole, 0.1mM PMSF, 0.25mM DTT, 25% glycerol._x000D_ Preparation Note_x000D_ after opening, aliquot into smaller quantities and store at -70 °C. Avoid repeating handling and multiple freeze/thaw cycles