3-Methyl-2-Oxobutanoate Dehydrogenase (Lipoamide)
MESHD042942
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.
Synonyms
- Branched-Chain Oxo-Acid Dehydrogenase
- Branched Chain Oxo Acid Dehydrogenase
- Dehydrogenase, Branched-Chain Oxo-Acid
- Oxo-Acid Dehydrogenase, Branched-Chain
- 2-Oxoisocaproate Dehydrogenase
- 2 Oxoisocaproate Dehydrogenase
- Dehydrogenase, 2-Oxoisocaproate
- 2-Oxoisovalerate Dehydrogenase (Lipoamide)
- Alpha-Keto Acid Dehydrogenase
- Acid Dehydrogenase, Alpha-Keto
- Alpha Keto Acid Dehydrogenase
- Dehydrogenase, Alpha-Keto Acid
- BCKA Decarboxylase
- Decarboxylase, BCKA
- Branched Chain Alpha-Keto Acid Decarboxylase
- Branched Chain Alpha Keto Acid Decarboxylase
- Branched Chain Ketoacid Dehydrogenase
- Branched-Chain 2-Oxo Acid Dehydrogenase
- Branched Chain 2 Oxo Acid Dehydrogenase
- Branched-Chain Keto Acid Dehydrogenase
- Branched Chain Keto Acid Dehydrogenase
Source last updated 2026-05-24T15:25:13.889Z