MeSH 搜索器

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 1.2.4.3.
推出的年份: 2004
副标题
树号: D08.811.682.657.350.760
MeSH 单一 ID: D042942
进入的组:
  • 2-Oxoisocaproate Dehydrogenase
  • 2 Oxoisocaproate Dehydrogenase
  • Dehydrogenase, 2-Oxoisocaproate
  • 2-Oxoisovalerate Dehydrogenase (Lipoamide)
  • Branched-Chain Keto Acid Dehydrogenase
  • Branched Chain Keto Acid Dehydrogenase
  • BCKA Decarboxylase
  • Decarboxylase, BCKA
  • Branched Chain Alpha-Keto Acid Decarboxylase
  • Branched Chain Alpha Keto Acid Decarboxylase
  • Branched Chain Ketoacid Dehydrogenase
  • Branched-Chain 2-Oxo Acid Dehydrogenase
  • Branched Chain 2 Oxo Acid Dehydrogenase
  • Branched-Chain Oxo-Acid Dehydrogenase
  • Branched Chain Oxo Acid Dehydrogenase
  • Dehydrogenase, Branched-Chain Oxo-Acid
  • Oxo-Acid Dehydrogenase, Branched-Chain
  • Alpha-Keto Acid Dehydrogenase
  • Acid Dehydrogenase, Alpha-Keto
  • Alpha Keto Acid Dehydrogenase
  • Dehydrogenase, Alpha-Keto Acid
早前的内容:
  • Amino Acids, Branched Chain (1979-1981)
  • Carboxy-Lyases (1974-1979)
  • Keto Acids (1973-1981)
  • Ketone Oxidoreductases (1982-2003)
  • Multienzyme Complexes (1982-2003)

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