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.