3-Methyl-2-Oxobutanoate Dehydrogenase (Lipoamide)


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