This gene encodes the mitochondrial enzyme methylmalonyl Coenzyme A mutase.

In humans, the product of this gene is a vitamin B12-dependent enzyme which catalyzes the isomerization of methylmalonyl-CoA to succinyl-CoA, while in other species this enzyme may have different functions.

Mutations in this gene may lead to various types of methylmalonic aciduria. [provided by RefSeq, Jul 2008]