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Concept information

... > Enzymes and Coenzymes > Enzymes > Oxidoreductases > Oxidoreductases Acting on Aldehyde or Oxo Group Donors > Ketone Oxidoreductases > 3-Methyl-2-Oxobutanoate Dehydrogenase (Lipoamide)

Terme préférentiel

3-Methyl-2-Oxobutanoate Dehydrogenase (Lipoamide)  

Type

  • mesh:Descriptor

Définition

  • 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.

Concept générique

Concepts associés

Synonyme(s)

  • 2-Oxoisocaproate Dehydrogenase
  • 2-oxoisocaproate dehydrogenase
  • 2-Oxoisovalerate Dehydrogenase (Lipoamide)
  • 9082-72-8 (CRN)
  • Alpha-Keto Acid Dehydrogenase
  • BCKA Decarboxylase
  • Branched-Chain 2-Oxo Acid Dehydrogenase
  • Branched Chain Alpha-Keto Acid Decarboxylase
  • Branched Chain Ketoacid Dehydrogenase
  • Branched-Chain Keto Acid Dehydrogenase
  • Branched-Chain Oxo-Acid Dehydrogenase

Qualificatif(s) autorisé(s)

Traductions

URI

http://data.loterre.fr/ark:/67375/JVR-ZXCPSDCS-3

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RDF/XML TURTLE JSON-LD Date de création 09/07/2003, dernière modification le 09/07/2013