Pyruvate carboxylase: Difference between revisions

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<StructureSection load='2qf7' size='340' side='right' caption='Pyruvate carboxylase complex with CoA, ATP-gamma-S,glycerol, Mg++ (green), Zn++ (grey) Cl- (green) (PDB code [[2qf7]])' scene=''>
<StructureSection load='2qf7' size='340' side='right' caption='Pyruvate carboxylase complex with CoA, ATP-gamma-S, glycerol, Mg++ (green), Zn++ (grey) Cl- (green) (PDB code [[2qf7]])' scene='93/939263/Cv/2'>


==Function==
==Function==
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==Disease==
==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<ref>PMID:30870574</ref> . Deregulation of PC expression is associated with type 2 diabetics and tumorgenesis in several cancers<ref>PMID:29362846</ref> .
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<ref>PMID:30870574</ref>. Deregulation of PC expression is associated with type 2 diabetics and tumorgenesis in several cancers<ref>PMID:29362846</ref> .


==Structural highlights==
==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.  The effector CoA is bound to the BC domain and the allosteric effector domain<ref>PMID:17717183</ref> .
The 3D structure of PC shows its <scene name='93/939263/Cv/3'>4 domains: BC, CT, allosteric effector and BCCP</scene>.  
 
The <scene name='93/939263/Cv/7'>ATP moiety active site is in the BC domain and contains 2 Mg++ ions</scene>. Water molecules shown as red spheres. <scene name='93/939263/Cv/8'>Close up view of 2 Mg++ coordination sites</scene>.   
 
The <scene name='93/939263/Cv/9'>effector CoA is bound to the BC domain and the allosteric effector domain</scene><ref>PMID:17717183</ref>.  


==3D structures of pyruvate carboxylase==
==3D structures of pyruvate carboxylase==