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 22.214.171.124, acetyl-CoA carboxylase and EC 126.96.36.199, propanoyl-CoA carboxylase. It prefers the S isomer, and can decarboxylate (R)-methylmalonyl-CoA with lower efficiency. cf. EC 188.8.131.52, (S)-methylmalonyl-CoA decarboxylase (sodium-transporting).
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. [DOI] [PMID: 22016388]