Concept information
Término preferido
3-Methyl-2-Oxobutanoate Dehydrogenase (Lipoamide)
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Tipo
-
mesh:Descriptor
Definición
- 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.
Concepto genérico
Etiquetas alternativas
- 2-Oxoisocaproate Dehydrogenase
- 2-oxoisocaproate dehydrogenase
- 2-Oxoisovalerate Dehydrogenase (Lipoamide)
- 9082-72-8 (CRN)
- Alpha-Keto Acid Dehydrogenase
- BCKA Decarboxylase
- Branched Chain Alpha-Keto Acid Decarboxylase
- Branched Chain Ketoacid Dehydrogenase
- Branched-Chain 2-Oxo Acid Dehydrogenase
- Branched-Chain Keto Acid Dehydrogenase
- Branched-Chain Oxo-Acid Dehydrogenase
En otras lenguas
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francés
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2-oxoisocaproate dehydrogenase
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2-Oxoisovalérate déshydrogénase (lipoamide)
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3-Méthyl-2-oxobutanoate déshydrogénase (lipoamide)
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9082-72-8 (CRN)
URI
http://data.loterre.fr/ark:/67375/JVR-ZXCPSDCS-3
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