IUBMB Enzyme Nomenclature


Accepted name: ethylmalonyl-CoA decarboxylase

Reaction: (S)-ethylmalonyl-CoA = butanoyl-CoA + CO2

Systematic name: (S)-ethylmalonyl-CoA carboxy-lyase (butanoyl-CoA-forming)

Comments: The enzyme, which exists in all vertebrates, decarboxylates ethylmalonyl-CoA, a potentially toxic compound that is formed in low amounts by the activity of EC (acetyl-CoA carboxylase) and EC (propanoyl-CoA carboxylase). It prefers the S isomer, and can decarboxylate (R)-ethylmalonyl-CoA with lower efficiency. cf. EC, (S)-methylmalonyl-CoA decarboxylase (sodium-transporting).

Links to other databases: BRENDA, EXPASY, KEGG, Metacyc, CAS registry number:


1. Linster, C.L., Noel, G., Stroobant, V., Vertommen, D., Vincent, M.F., Bommer, G.T., Veiga-da-Cunha, M. and Van Schaftingen, E. Ethylmalonyl-CoA decarboxylase, a new enzyme involved in metabolite proofreading. J. Biol. Chem. 286 (2011) 42992-43003. [PMID: 22016388]

[EC created 2012]

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