Recombinant Pyruvate Dehydrogenase E1 α Monoclonal Antibody

The pyruvate dehydrogenase (PDH) complex is a nuclear-encoded mitochondrial multienzyme complex that 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. The PDH complex is composed of multiple copies of three enzymatic components: pyruvate dehydrogenase (E1), dihydrolipoamide acetyltransferase (E2) and lipoamide dehydrogenase (E3) . The E1 enzyme is a heterotetramer of two alpha and two beta subunits. This gene encodes the E1 alpha 1 subunit containing the E1 active site, and plays a key role in the function of the PDH complex. Mutations in this gene are associated with pyruvate dehydrogenase E1-alpha deficiency and X-linked Leigh syndrome. Alternatively spliced transcript variants encoding different isoforms have been found for this gene.

PDHA1; PDHA; PDHAD; PDHCE1A; PHE1A; E1 alpha polypeptide 1; mitochondrial; ODPA; PDH; PDHE1 A type I; PDHE1-A type I; PDHα; Pyruvate Dehydrogenase (lipoamide) alpha 1; Pyruvate dehydrogenase complex; Pyruvate Dehydrogenase E1 alpha; Pyruvate dehydrogenase E1 component subunit alpha; somatic form