methylmalonyl-CoA

(redirected from Methylmalonyl CoA)

meth·yl·mal·o·nyl-CoA

(meth'il-mal'o-nil),
An intermediate in the degradation of several metabolites (for example, valine, methionine, odd-chain fatty acids, theronine); elevated in cases of pernicious anemia.
References in periodicals archive ?
MMA due to methylmalonyl CoA mutate deficiency - normal FA, tHcy, B12 and methionine; Cb1-RD defects - high tHcy, low plasma methionine, normal serum B12 and FA;
LDHA, lactate dehydrogenase A; LDHB, lactate dehydrogenase B; PDHA1, pyruvate dehydrogenase (lipoamide) alpha 1; DLAT, dihydrolipoamide S-cetyltransferase; DLD, dihydrolipoamide dehydrogenase; GK, glycerol kinase; GPD1, glycerol-3-phosphate dehydrogenase 1; GPD2, glycerol -3-phosphate dehydrogenase 2; ACSS3, acyl CoA synthetase short chain family member 3; PCCA, propionyl CoA carboxylase alpha; PCCB, propionyl CoA carboxylase beta; MCEE, methylmalonyl CoA epimerase; MUT, methylmalonyl CoA mutase.
Methionine synthase (in the cytosol) requires methylcobalamin as cofactor, and methylmalonyl CoA mutase (in mitochondria) requires 5' deoxyadenosylcobalamin as coenzyme.
Heterogeneous alleles and expression of methylmalonyl CoA mutase in mut methylmalonic acidemia.