Medical Definition of Methylmalonyl-CoA mutase
1.
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)
Lexicographical Neighbors of Methylmalonyl-CoA Mutase
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