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

Preferred term

3-Methyl-2-Oxobutanoate Dehydrogenase (Lipoamide)  

Type

  • Topical Descriptor

Broader concept

Related concepts

Entry terms

  • 2 Oxoisocaproate Dehydrogenase
  • 2-Oxoisocaproate Dehydrogenase
  • 2-Oxoisovalerate Dehydrogenase (Lipoamide)
  • Acid Dehydrogenase, Alpha-Keto
  • Alpha Keto Acid Dehydrogenase
  • Alpha-Keto Acid Dehydrogenase
  • BCKA Decarboxylase
  • Branched Chain 2 Oxo Acid Dehydrogenase
  • Branched-Chain 2-Oxo Acid Dehydrogenase
  • Branched Chain Alpha Keto Acid Decarboxylase
  • Branched Chain Alpha-Keto Acid Decarboxylase
  • Branched Chain Keto Acid Dehydrogenase
  • Branched Chain Ketoacid Dehydrogenase
  • Branched-Chain Keto Acid Dehydrogenase
  • Branched Chain Oxo Acid Dehydrogenase
  • Branched-Chain Oxo-Acid Dehydrogenase
  • Decarboxylase, BCKA
  • Dehydrogenase, 2-Oxoisocaproate
  • Dehydrogenase, Alpha-Keto Acid
  • Dehydrogenase, Branched-Chain Oxo-Acid
  • Oxo-Acid Dehydrogenase, Branched-Chain

Scope note

  • 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.

History note

  • 2004; use 3-METHYL-2-OXBUTANOATE DEHYDROGENASE (LIPOAMIDE) (NM) 1973-2003

In other languages

URI

http://www.yso.fi/onto/mesh/D042942

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