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
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)
留言 (0)