Anti-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.Synonyms: Carbamoyl Phosphate Synthase, mitochondrial, CPSase I. Immunogen: Peptide from the N-terminal region of CPS1. Clone Designation: RM395. Formulation: (Request formulation change), 100 µl of protein A-affinity purified monoclonal antibody. Storage Buffer: PBS, with 50% glycerol, 1% BSA, and 0.09% sodium azide. Host: Rabbit. Isotype: IgG. Applications: IHC, WB. Cross Reactivity: (+) CPS1.