IUBMB Enzyme Nomenclature


Reaction: a long-chain (S)-3-hydroxyacyl-CoA + NAD+ = a long-chain 3-oxoacyl-CoA + NADH + H+

Glossary: a long-chain acyl-CoA = an acyl-CoA thioester where the acyl chain contains 13 to 22 carbon atoms.

Other name(s): β-hydroxyacyl-CoA dehydrogenase; long-chain 3-hydroxyacyl coenzyme A dehydrogenase; 3-hydroxyacyl-CoA dehydrogenase; LCHAD

Systematic name: long-chain-(S)-3-hydroxyacyl-CoA:NAD+ oxidoreductase

Comments: This enzyme was purified from the mitochondrial inner membrane. The enzyme has a preference for long-chain substrates, and activity with a C16 substrate was 6- to 15-fold higher than with a C4 substrate. (cf. EC 3-hydroxyacyl-CoA dehydrogenase).

Links to other databases: BRENDA, EXPASY, KEGG, Metacyc, PDB, CAS registry number: 84177-52-6


1. El-Fakhri, M. and Middleton, B. The existence of an inner-membrane-bound, long acyl-chain-specific 3-hydroxyacyl-CoA dehydrogenase in mammalian mitochondria. Biochim. Biophys. Acta 713 (1982) 270-279. [PMID: 7150615]

[EC created 1986]

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