The protein encoded by this gene is the alpha subunit of the heterodimeric mitochondrial enzyme Propionyl-CoA carboxylase. PCCA encodes the biotin-binding region of this enzyme. Mutations in either PCCA or PCCB (encoding the beta subunit) lead to an enzyme deficiency resulting in propionic acidemia. Multiple transcript variants encoding different isoforms have been found for this gene.
PCCA; propionyl CoA carboxylase, alpha polypeptide; propionyl Coenzyme A carboxylase, alpha polypeptide; propionyl-CoA carboxylase alpha chain, mitochondrial; PCCase subunit alpha; Propanoyl CoA:carbon dioxide ligase subunit alpha; Propionyl CoA carboxylase alpha chain; Propionyl CoA carboxylase alpha chain mitochondrial; Propionyl Coenzyme A Carboxylase Alpha Polypeptide; PCCase alpha subunit; pccA complementation group; propanoyl-CoA:carbon dioxide ligase alpha subunit; wu:fb92g02; zgc:100925; fb92g02; propionyl-Coenzyme A carboxylase, alpha polypeptide