Definitions of 3-methyl-2-oxobutanoate dehydrogenase (lipoamide)

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Definition of '3-methyl-2-oxobutanoate dehydrogenase (lipoamide)'

U.S. National Library of Medicine

1. 3-methyl-2-oxobutanoate dehydrogenase (lipoamide)
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.

'3-methyl-2-oxobutanoate dehydrogenase (lipoamide)' - Nearby Entries
3-isopropylmalate dehydrogenase 3-mercaptopropionic acid 3-methoxy-4-hydroxyphenylethanol 3-methyl-2-oxobutanoate dehydrogenase (lipoamide) 3-o-methylglucose 3-oxoacyl-(acyl-carrier-protein) synthase 3-phosphoshikimate 1-carboxyvinyltransferase

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