Propionyl-CoA carboxylase subunits
propionyl-coa carboxylase subunits
Propionyl-CoA carboxylase (PCC) is the enzyme which catalyzes the carboxylation of propionyl-CoA to methylmalonyl-CoA and is encoded by the genes PCCA and PCCB to form a hetero-dodecamer. Within the holoenzyme, PCCA catalyzes the ATP-dependent carboxylation of the biotin carried by the biotin carboxyl carrier (BCC) domain, while PCCB then transfers the carboxyl group from carboxylated biotin to propionyl-CoA. Dysfunction of PCC leads to the inherited metabolic disorder propionic acidemia.