| 单词 | Dehydrogenase,Branched-Chain Oxo-Acid |
| 释义 | Dehydrogenase,Branched-Chain Oxo-Acid《英文msh词典》Dehydrogenase,Branched-Chain Oxo-Acid [入口词] Dehydrogenase,Branched-Chain Oxo-Acid [主题词] 3-Methyl-2-Oxobutanoate Dehydrogenase (Lipoamide) [英文释义] 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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