Methylmalonyl-CoA Mutase

Synonyms

(R)-2-Methyl-3-oxopropanoyl-CoA CoA-carbonylmutase

Isomerase, Methylmalonyl-CoA

Methylmalonyl CoA Isomerase

Methylmalonyl CoA Mutase

Methylmalonyl-CoA Isomerase

Mutase, Methylmalonyl-CoA

An enzyme that catalyzes 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. EC 5.4.99.2.