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 to form (OAA). PC is a biotin-containing enzyme. PC is a tetrameric protein containing biotin carboxylase (BC), carboxyltransferase (CT), allosteric effector (acetyl-CoA) and biotin carboxyl carrier protein (BCCP) 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] .
See also Major metabolic pathways converging on the citric acid cycle.
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]. Deregulation of PC expression is associated with type 2 diabetics and tumorgenesis in several cancers[3] .
Structural highlights
The 3D structure of PC shows its .
The . Water molecules shown as red spheres. .
The [4].
3D structures of pyruvate carboxylase
Pyruvate carboxylase 3D structures
