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

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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.

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