Entrez Summary: 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.[provided by RefSeq, May 2010].
UniProt Summary: N/A
Pfam DomainsGO Terms
Pfam Domains
Carboxyl trans
GO Terms
propionyl-CoA carboxylase activity
biotin metabolic process
short-chain fatty acid catabolic process
short-chain fatty acid metabolic process
water-soluble vitamin metabolic process
fatty acid catabolic process
monocarboxylic acid catabolic process
vitamin metabolic process
cellular lipid catabolic process
coenzyme metabolic process
carboxylic acid catabolic process
organic acid catabolic process
lipid catabolic process
fatty acid metabolic process
sulfur compound metabolic process
mitochondrial matrix
small molecule catabolic process
cofactor metabolic process
drug metabolic process
monocarboxylic acid metabolic process
cellular amide metabolic process
carboxylic acid metabolic process
cellular lipid metabolic process
oxoacid metabolic process
organic acid metabolic process
lipid metabolic process
mitochondrion
ATP binding
small molecule metabolic process
organic substance catabolic process
cellular catabolic process
CRISPR Data
Compound HitMost Correlated Genes in ChemogenomicsTissues where Essential in the Avana Dataset (DepMap 20Q1)