Carbamoyl phosphate synthetase
FunctionCarbamoyl 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.[1] There are 3 forms of CPS:
DiseaseCPS I deficiency causes the accumulation of NH3 in the blood. Structural highlightsCPS 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 .[2] CPS I contains a methylglyoxal synthetase (MGS) domain which binds . Water molecules are shown as red spheres. . . . 3D structures of carbamoyl phosphate synthetaseCarbamoyl phosphate synthetase 3D structures
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ReferencesReferences
- ↑ 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