| 单词 | 3-Methyl-2-Oxobutanoate Dehydrogenase (Lipoamide) |
| 释义 | 3-Methyl-2-Oxobutanoate Dehydrogenase (Lipoamide)《英文msh词典》3-Methyl-2-Oxobutanoate Dehydrogenase (Lipoamide) [主题词] 3-Methyl-2-Oxobutanoate Dehydrogenase (Lipoamide) [同义词] 2-Oxoisovalerate Dehydrogenase (Lipoamide) [入口词] 2-Oxoisocaproate Dehydrogenase [入口词] Alpha-Keto Acid Dehydrogenase [入口词] BCKA Decarboxylase [入口词] Branched Chain Alpha-Keto Acid Decarboxylase [入口词] Branched Chain Ketoacid Dehydrogenase [入口词] Branched-Chain 2-Oxo Acid Dehydrogenase [入口词] Branched-Chain Keto Acid Dehydrogenase [入口词] Branched-Chain Oxo-Acid Dehydrogenase [入口词] 2 Oxoisocaproate Dehydrogenase [入口词] Acid Dehydrogenase,Alpha-Keto [入口词] Alpha Keto Acid Dehydrogenase [入口词] Branched Chain 2 Oxo Acid Dehydrogenase [入口词] Branched Chain Alpha Keto Acid Decarboxylase [入口词] Branched Chain Keto Acid Dehydrogenase [入口词] Branched Chain Oxo Acid Dehydrogenase [入口词] Decarboxylase,BCKA [入口词] Dehydrogenase,2-Oxoisocaproate [入口词] Dehydrogenase,Alpha-Keto Acid [入口词] Dehydrogenase,Branched-Chain Oxo-Acid [入口词] Oxo-Acid Dehydrogenase,Branched-Chain [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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