Function
Pyruvate carboxylase (PC) encoded by the gene PC is an enzyme (EC 6.4.1.1) of the ligase class that catalyzes (depending on the species) the physiologically irreversible carboxylation of pyruvate to form oxaloacetate (OAA). PC is a biotin-containing enzyme. PC is a tetrameric protein containing biotin carboxylase, carboxyltransferase, allosteric effector (acetyl-CoA) and biotin carboxyl carrier domains. PC is regulated by acetyl-CoA and Asp. The biotin moiety transfers the carboxyl group from the biotin carboxylase active site to the carboxyltransferase active site[1] .
Disease
Mutations in the PC gene cause 3 types of clinical spectra. Type A and B are neonatal forms causing early death; type C causes mild intellectual delay[2] .
Structural highlights
3D structures of pyruvate carboxylase
Pyruvate carboxylase 3D structures
