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).
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Protein Aliases: PCCase subunit beta; PCCB; Propanoyl-CoA:carbon dioxide ligase subunit beta; propionyl CoA carboxylase, beta polypeptide; propionyl Coenzyme A carboxylase, beta polypeptide; Propionyl-CoA carboxylase beta chain, mitochondrial
Gene Aliases: 1300012P06Rik; AI314687; PCCB; R74805
Molecular Function: ligase