The protein encoded by this gene is a subunit of the propionyl-CoA carboxylase (PCC) enzyme, which is involved in the catabolism of propionyl-CoA. PCC is a mitochondrial enzyme that probably acts as a dodecamer of six alpha subunits and six beta subunits. This gene encodes the beta subunit of PCC. Defects in this gene are a cause of propionic acidemia type II (PA-2). Multiple transcript variants encoding different isoforms have been found for this gene.
PCCB; propionyl CoA carboxylase, beta polypeptide; propionyl Coenzyme A carboxylase, beta polypeptide; propionyl-CoA carboxylase beta chain, mitochondrial; mitochondrial; PCCase subunit beta; PCCB_HUMAN; pccBC Complementation group; Propanoyl CoA:carbon dioxide ligase subunit beta; Propanoyl-CoA:carbon dioxide ligase subunit beta; Propionyl CoA carboxylase beta chain, mitochondrial; Propionyl-CoA carboxylase , beta subunit; Propionyl-CoA carboxylase beta chain; R74805; DKFZp451E113; zgc:85813