EC - Ethylmalonyl-CoA decarboxylase

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IntEnz Enzyme Nomenclature


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



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)-methylmalonyl-CoA with lower efficiency. cf. EC (methylmalonyl-CoA decarboxylase).

Links to other databases

Enzymes and pathways: NC-IUBMB , BRENDA , ExplorEnz , ENZYME@ExPASy , KEGG , MetaCyc , UniPathway
Structural data: CSA , EC2PDB


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

[EC created 2012]