3-hydroxy-2-methylbutyryl-CoA dehydrogenase

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In enzymology, a 3-hydroxy-2-methylbutyryl-CoA dehydrogenase (EC is an enzyme that catalyzes the chemical reaction

(2S,3S)-3-hydroxy-2-methylbutanoyl-CoA + NAD+ 2-methylacetoacetyl-CoA + NADH + H+

Thus, the two substrates of this enzyme are (2S,3S)-3-hydroxy-2-methylbutanoyl-CoA and NAD+, whereas its 3 products are 2-methylacetoacetyl-CoA, NADH, and H+.

This enzyme belongs to the family of oxidoreductases, specifically those acting on the CH-OH group of donor with NAD+ or NADP+ as acceptor. The systematic name of this enzyme class is (2S,3S)-3-hydroxy-2-methylbutanoyl-CoA:NAD+ oxidoreductase. Other names in common use include 2-methyl-3-hydroxybutyryl coenzyme A dehydrogenase, 2-methyl-3-hydroxybutyryl coenzyme A dehydrogenase, and 2-methyl-3-hydroxy-butyryl CoA dehydrogenase. This enzyme participates in valine, leucine and isoleucine degradation.

Structural studies

As of late 2007, only one structure has been solved for this class of enzymes, with the PDB accession code 2O23.


  • IUBMB entry for
  • BRENDA references for (Recommended.)
  • PubMed references for
  • PubMed Central references for
  • Google Scholar references for
  • Conrad RS, Massey LK, Sokatch JR (1974). "D- and L-isoleucine metabolism and regulation of their pathways in Pseudomonas putida". J. Bacteriol. 118: 103&ndash, 11. PMID 4150713.

External links

The CAS registry number for this enzyme class is 52227-66-4.

Gene Ontology (GO) codes