Carbamoyl phosphate synthetase
From Proteopedia
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| - | < | + | <StructureSection load='1a9x' size='350' side='right' scene='49/493669/Cv/4' caption='E. coli carbamoyl phosphate synthetase large chain (cyan) and small chain (salmon) complex with ADP, phosphate and ornithine, [[1a9x]]'> |
| - | + | == Function == | |
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| - | + | '''Carbamoyl phosphate synthetase''' (CPS) catalyzes the production of carbamoyl phosphate from ATP, Mg+2, bicarbonate and glutamine. CPS is part of the pyrimidine and arginine biosynthesis as well as the urea cycle in vertebrates. Ornithine is an allosteric effector of CPS.<ref>PMID:5329589</ref> There are 3 forms of CPS:<br /> | |
| - | + | * '''CPS I''' participates in the urea cycle. CPS I uses glutamine for NH3 source.<br /> | |
| - | '''Carbamoyl phosphate synthetase''' (CPS) catalyzes the production of carbamoyl phosphate | + | * '''CPS II''' participates in pyrimidine metabolism<br /> |
| + | * '''CPS III''' is found in fish. | ||
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| + | == Disease == | ||
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| + | CPS I deficiency causes the accumulation of NH3 in the blood. | ||
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| + | == Structural highlights == | ||
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| + | CPS I and II are composed of 2 subunits. The large subunit contain active sites which bind nucleotides and other effectors. The small subunit catalyzes the hydrolysis of glutamine to glutamate and NH3. The <scene name='49/493669/Cv/3'>small subunit active site contains an active Cys residue</scene>.<ref>PMID:9636022</ref> CPS I contains a methylglyoxal synthetase (MGS) domain which binds <scene name='49/493669/Cv/6'>L-ornithine</scene>. Water molecules are shown as red spheres. | ||
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| + | <scene name='49/493669/Cv/11'>ADP binding site</scene>. | ||
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| + | <scene name='49/493669/Cv/12'>Mn coordination site</scene>. | ||
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| + | <scene name='49/493669/Cv/13'>K coordination site</scene>. | ||
==3D structures of carbamoyl phosphate synthetase== | ==3D structures of carbamoyl phosphate synthetase== | ||
| + | [[Carbamoyl phosphate synthetase 3D structures]] | ||
| + | </StructureSection> | ||
| - | + | == References == | |
| - | + | <references/> | |
| - | [[ | + | [[Category:Topic Page]] |
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Current revision
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References
- ↑ Kalman SM, Duffield PH, Brzozowski T. Purification and properties of a bacterial carbamyl phosphate synthetase. J Biol Chem. 1966 Apr 25;241(8):1871-7. PMID:5329589
- ↑ Thoden JB, Miran SG, Phillips JC, Howard AJ, Raushel FM, Holden HM. Carbamoyl phosphate synthetase: caught in the act of glutamine hydrolysis. Biochemistry. 1998 Jun 23;37(25):8825-31. PMID:9636022 doi:http://dx.doi.org/10.1021/bi9807761
