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3-Methyl-2-Oxobutanoate Dehydrogenase (Lipoamide)

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Definition: 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.     
See Also Maple Syrup Urine Disease 		Other names Oxo-Acid Dehydrogenase, Branched-Chain; Dehydrogenase, Branched-Chain Oxo-Acid; Dehydrogenase, Alpha-Keto Acid; Dehydrogenase, 2-Oxoisocaproate; Decarboxylase, BCKA; Branched Chain Oxo Acid Dehydrogenase; Branched Chain Keto Acid Dehydrogenase; Branched Chain Alpha Keto Acid Decarboxylase; Branched Chain 2 Oxo Acid Dehydrogenase; Alpha Keto Acid Dehydrogenase; Acid Dehydrogenase, Alpha-Keto; 2 Oxoisocaproate Dehydrogenase; Branched-Chain Oxo-Acid Dehydrogenase; Branched-Chain Keto Acid Dehydrogenase; Branched-Chain 2-Oxo Acid Dehydrogenase; Branched Chain Ketoacid Dehydrogenase; Branched Chain Alpha-Keto Acid Decarboxylase; BCKA Decarboxylase; Alpha-Keto Acid Dehydrogenase; 2-Oxoisocaproate Dehydrogenase

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Sources: NLM Medical Subject Headings, NIH UMLS, Drugs@FDA, FDA AERS original data copyright United States Government. No endorsement implied. Last modified 6/6/2012
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