methylmalonyl-CoA mutasemedical dictionary

<enzyme>

<cell biology> An enzyme that catalyses the conversion of methylmalonyl-CoA to succinyl-CoA by transfer of the carbonyl group. It requires a cobamide coenzyme. A block in this enzymatic conversion leads to the metabolic disease, methylmalonic aciduria.

Chemical name: (R)-2-Methyl-3-oxopropanoyl-CoA CoA-carbonylmutase

Registry number: EC 5.4.99.2

(12 Dec 1998)