A ketone oxidoreductase that catalyzes the overall conversion of alpha-keto acids to ACYL-CoA and CO2. The enzyme requires THIAMINE DIPHOSPHATE as a cofactor. Defects in genes that code for subunits of the enzyme are a cause of MAPLE SYRUP URINE DISEASE. The enzyme was formerly classified as EC 1.2.4.3.
Also Known As:
Alpha-Keto Acid Dehydrogenase; Branched Chain Ketoacid Dehydrogenase; 2-Oxoisocaproate Dehydrogenase; BCKA Decarboxylase; Branched Chain Alpha-Keto Acid Decarboxylase; Branched-Chain 2-Oxo Acid Dehydrogenase; Branched-Chain Keto Acid Dehydrogenase; Branched-Chain Oxo-Acid Dehydrogenase; 2 Oxoisocaproate Dehydrogenase; Acid Dehydrogenase, Alpha-Keto; Alpha Keto Acid Dehydrogenase; Branched Chain 2 Oxo Acid Dehydrogenase; Branched Chain Alpha Keto Acid Decarboxylase; Branched Chain Keto Acid Dehydrogenase; Branched Chain Oxo Acid Dehydrogenase; Decarboxylase, BCKA; Dehydrogenase, 2-Oxoisocaproate; Dehydrogenase, Alpha-Keto Acid; Dehydrogenase, Branched-Chain Oxo-Acid; Oxo-Acid Dehydrogenase, Branched-Chain; 2-Oxoisovalerate Dehydrogenase (Lipoamide)