An intermediate in the degradation of several metabolites ( e.g., valine, methionine, odd-chain fatty acid s, theronine); elevated in cases of pernicious anemia.
- methylmalonyl-CoA epimerase an enzyme that catalyzes the interconversion of d-methylmalonyl-CoA and l-methylmalonyl-CoA.
- methylmalonyl-CoA mutase an enzyme that catalyzes a reversible interconversion of l-methylmalonyl-CoA and succinyl-CoA; a cobalamin-dependent enzyme; deficiency of this enzyme will result in methylmalonic acidemia.
Medical dictionary. 2011.