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3-Methyl-2-Oxobutanoate Dehydrogenase (Lipoamide)

"3-Methyl-2-Oxobutanoate Dehydrogenase (Lipoamide)" is a descriptor in the National Library of Medicine's controlled vocabulary thesaurus, MeSH (Medical Subject Headings). Descriptors are arranged in a hierarchical structure, which enables searching at various levels of specificity.

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.


This graph shows the total number of publications written about "3-Methyl-2-Oxobutanoate Dehydrogenase (Lipoamide)" by people in Harvard Catalyst Profiles by year, and whether "3-Methyl-2-Oxobutanoate Dehydrogenase (Lipoamide)" was a major or minor topic of these publication.
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Funded by the NIH National Center for Advancing Translational Sciences through its Clinical and Translational Science Awards Program, grant number UL1TR002541.