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Dictionary » 3-Methyl-2-Oxobutanoate Dehydrogenase (Lipoamide)

3-Methyl-2-Oxobutanoate Dehydrogenase (Lipoamide)

Introduction: 3-Methyl-2-Oxobutanoate Dehydrogenase (Lipoamide)

Description of 3-Methyl-2-Oxobutanoate Dehydrogenase (Lipoamide)

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
Source: MeSH 2007

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