Background：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.
