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2 Oxoisocaproate 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 1.2.4.3.