3-Methyl-2-Oxobutanoate Dehydrogenase (Lipoamide) |
| 2-Oxoisocaproate Dehydrogenase; 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 |
| 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. |
|
|
|