Propionyl-CoA carboxylase alpha chain, mitochondrial (PCCA)

Propionyl-CoA carboxylase alpha chain, mitochondrial (PCCA), is the alpha subunit of a biotin-dependent mitochondrial enzyme critical for the carboxylation of propionyl-CoA to (S)-methylmalonyl-CoA, an essential step in the catabolism of odd-chain fatty acids and branched-chain amino acids. The enzyme is a heterododecamer, composed of six alpha (PCCA) and six beta (PCCB) subunits, and requires biotin as a cofactor. Mutations in PCCA cause propionic acidemia, an autosomal recessive inborn error of metabolism characterized by life-threatening episodes of metabolic acidosis and long-term neurological and systemic complications. While not currently a target for small molecule drugs, PCCA is fundamental to cellular intermediary metabolism and serves as a diagnostic marker in metabolic disease.

Other names

Propionyl-CoA carboxylase subunit alphaPCCase subunit alphaPropanoyl-CoA:carbon dioxide ligase subunit alphaPropionyl-CoA carboxylase alpha subunitPCCase alpha subunitpccA complementation groupPropionyl coenzyme A carboxylase, alpha polypeptide

No targeted pharmacological mechanism of action as a drug target. Disease management focuses on dietary restriction or removing propionyl-CoA substrate source.