Pyruvate carboxylase

spinqualitylabelsall models Pyruvate carboxylase complex with CoA, ATP-gamma-S, glycerol, Mg++ (green), Zn++ (grey) Cl- (green) (PDB code 2qf7) Show:Asymmetric Unit Biological Assembly Drag the structure with the mouse to rotate   Export Animated Image   Print3D 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 (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 . 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. Deregulation of PC expression is associated with type 2 diabetics and tumorgenesis in several cancers . Structural highlights The 3D structure of PC shows its 4 domains: BC, CT, allosteric effector and BCCP. The ATP moiety active site is in the BC domain and contains 2 Mg++ ions. Water molecules shown as red spheres. Close up view of 2 Mg++ coordination sites. The effector CoA is bound to the BC domain and the allosteric effector domain. 3D structures of pyruvate carboxylase Pyruvate carboxylase 3D structures

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