Alpha Keto Acid Dehydrogenase
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 22.214.171.124.