Propionyl-CoA Carboxylase Alpha Subunit (PCCA)

The propionyl-CoA carboxylase alpha subunit is one of two essential subunits that together form the mitochondrial enzyme propionyl-CoA carboxylase (PCC). This enzyme catalyzes the ATP-dependent carboxylation of propionyl-CoA to methylmalonyl-CoA, a key step in the catabolism of certain amino acids, odd-chain fatty acids, and cholesterol. Mutations in PCCA cause propionic acidemia.

Other names

PCCA_HUMANPCCase alpha subunitPropionyl Coenzyme A carboxylase, alpha polypeptidePropionyl-CoA:carbon dioxide ligase alpha subunit

Inhibition of propionyl-CoA carboxylase would prevent the conversion of propionyl-CoA to methylmalonyl-CoA, impacting branched-chain amino acid and odd-chain fatty acid metabolism. There are no commonly known drugs that directly target this subunit but the enzyme is targetable.