propionyl-CoA carboxylase

(redirected from Propionyl CoA carboxylase)

pro·pi·o·nyl-CoA car·box·yl·ase

an enzyme that catalyzes the reaction of propionyl-CoA with CO2 and ATP to produce ADP, orthophosphate, and d-methylmalonyl-CoA; a biotin-dependent enzyme; an inherited deficiency of this enzyme will lead to propionic acidemia and developmental retardation.
References in periodicals archive ?
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.