Tags: Enzyme.
Propionyl-CoA carboxylase catalyses the carboxylation reaction of propionyl CoA in the mitochondrial matrix. The enzyme is biotin-dependent. The product of the reaction is (S)-methylmalonyl CoA. Propionyl CoA is the end product of metabolism of odd-chain fatty acids and is also a metabolite of most methyl-branched fatty acids. It is also the main metabolite of valine and together with acetyl-CoA is a metabolite of isoleucine as well as a methionine metabolite.