Cayman CPS1 (N-Term) Rabbit Monoclonal Antibody (RM395)

Carbamoyl phosphate synthetase I (CPS1) is a ligase involved in the urea cycle. It is composed of an N-terminal domain containing an ancestral inactive glutaminase, a bicarbonate phosphorylation site, and a C-terminal domain containing a carbamate phosphorylation site and the binding site for the CPS1 allosteric activator N-acetyl-L-glutamate (NAG). It is primarily expressed in the liver and intestinal epithelial cells and is localized to the mitochondrial matrix.^, CPS1, with NAG as a cofactor, catalyzes the formation of carbamoyl phosphate from ammonia, bicarbonate, and ATP as the first, rate-limiting step in the urea cycle. Mutations in CPS1 result in CPS1 deficiency, a disorder characterized by low levels of active CPS1, arginine, and citrulline and high levels of ammonia and glutamine, leading to neurological deficits and, potentially, death.^, Protein levels of CPS1 are increased in patient-derived rectal cancer tumors and associated with shorter disease-specific survival and metastasis-free survival. Cayman’s CPS1 (N-Term) Rabbit Monoclonal Antibody (RM395) can be used for immunohistochemistry (IHC) and Western blot (WB) applications.

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