Carbamoyl-phosphate synthase CPS1 Light
Ref. 3D-CRB1000409
| 25nMol | 202.00 € |
Product Information
- H-AVNTLNEALEFAK-OH
Carbamoyl phosphate synthase I is the rate-limiting enzyme that catalyses the first committed step of the hepatic urea cycle by synthesising carbamoyl phosphate from ammonia, bicarbonate, and 2 molecules of ATP.The mitochondrial isozyme is designated CPS I and the cytoplasmic enzyme CPS II. CPS II is part of a multifunctional enzyme, called the CAD trifunctional protein of pyrimidine biosynthesis. CPS1 deficiency is a recessively inherited disorder which affects the urea cycle and is due to CPS1 gene mutations. CPS1 causes life-threatening hyperammonemia. Interestingly, carbamoyl phosphate synthase is also implicated in vascular function, as it influences nitric oxide concentrations and nitric-oxide-mediated vasodilation.
Chemical properties
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