3-Methyl-2-Oxobutanoate Dehydrogenase (Lipoamide)
Synonyms
2 Oxoisocaproate Dehydrogenase
2-Oxoisocaproate Dehydrogenase
2-Oxoisovalerate Dehydrogenase (Lipoamide)
2-oxoisocaproate dehydrogenase
Acid Dehydrogenase, Alpha-Keto
Alpha Keto Acid Dehydrogenase
Alpha-Keto Acid Dehydrogenase
BCKA Decarboxylase
Branched Chain 2 Oxo Acid Dehydrogenase
Branched Chain Alpha Keto Acid Decarboxylase
Branched Chain Alpha-Keto Acid Decarboxylase
Branched Chain Keto Acid Dehydrogenase
Branched Chain Ketoacid Dehydrogenase
Branched Chain Oxo Acid Dehydrogenase
Branched-Chain 2-Oxo Acid Dehydrogenase
Branched-Chain Keto Acid Dehydrogenase
Branched-Chain Oxo-Acid Dehydrogenase
Decarboxylase, BCKA
Dehydrogenase, 2-Oxoisocaproate
Dehydrogenase, Alpha-Keto Acid
Dehydrogenase, Branched-Chain Oxo-Acid
Oxo-Acid Dehydrogenase, Branched-Chain
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.
See Also