Carbamoyl phosphate synthetase

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Template:STRUCTURE 1a9x Carbamoyl phosphate synthetase (CPS) catalyzes the production of carbamoyl phosphate in 3 steps. (1) Carbonyl phosphate is produced from bicarbonate and ATP. (2) Carbamic acid and inorganic phosphate are produced from carbonyl phosphate and NH3. (3) Carbamoyl phosphate and ADP are produced from carbamic acid and ATP. CPS is part of the pyrimidine and arginine biosynthesis as well as the urea cycle in vertebrates. Ornithine is an allosteric effector of CPS. There are 3 forms of CPS. CPS I participates in the urea cycle; CPS II participates in pyrimidine metabolism; CPS III is found in fish. CPS I and II are composed of 2 subunits. CPS I uses glutamine for NH3 source and contains a methylglyoxal synthetase (MGS) domain which binds ornithine. CPS I deficiency causes the accumulation of NH3 in the blood.

3D structures of carbamoyl phosphate synthetase

2yvq – CPS I MGS domain - human
1jdb - EcCPS + phosphate + glutamine + ADP – Escherichia coli
1a9x, 1cs0 - EcCPS + phosphate + ornithine + ADP
1c30, 1m6v - EcCPS (mutant) + phosphate + ornithine + ADP
1bxr - EcCPS + ornithine + AMPPNP
1ce8 - EcCPS + phosphate + ornithine + inosinic acid + ADP
1c3o - EcCPS (mutant) + phosphate + ornithine + glutamine + ADP
1kee - EcCPS + phosphate + ornithine + acivicin + ADP
1t36 - EcCPS (mutant) + phosphate + ornithine + UMP + ADP

Proteopedia Page Contributors and Editors (what is this?)

Alexander Berchansky, Michal Harel

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