3-Methyl-2-Oxobutanoate Dehydrogenase (Lipoamide)

**Definition:** 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.

**Tree numbers:** - D08.811.682.657.350.760

**Synonyms:** - Branched-Chain Oxo-Acid Dehydrogenase - 2-Oxoisocaproate Dehydrogenase - 2-Oxoisovalerate Dehydrogenase (Lipoamide) - Alpha-Keto Acid Dehydrogenase - BCKA Decarboxylase - Branched Chain Alpha-Keto Acid Decarboxylase - Branched Chain Ketoacid Dehydrogenase - Branched-Chain 2-Oxo Acid Dehydrogenase - Branched-Chain Keto Acid Dehydrogenase