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Carbamoyl-phosphate synthetase 1 (CPS1) is a protein encoded by the CPS1 gene on chromosome 2q35. CPS1 contains 1,464 amino acids with a molecular weight of around 160 kDa. Post-translational modifications of CPS1 may include phosphorylation, glycosylation, and disulfide bond formation, although specific details may vary depending on cellular context. CPS1 is primarily an intracellular protein localized within the mitochondria of hepatic cells.
The main function of CPS1 is to catalyze the first step of the urea cycle, which is responsible for the detoxification of ammonia in the liver. Specifically, CPS1 facilitates the conversion of ammonia and bicarbonate into carbamoyl phosphate, a crucial intermediate in the urea cycle. This process allows for the excretion of excess nitrogen in the form of urea, preventing the buildup of toxic ammonia in the bloodstream.
CPS1 is predominantly expressed in hepatocytes, the main functional cells of the liver. Its expression is tightly regulated at both the transcriptional and post-translational levels, primarily by dietary protein intake and hormonal signals such as glucagon and insulin. Dysregulation of CPS1 expression or activity can lead to hyperammonemia, a condition characterized by elevated levels of ammonia in the blood, which can result in neurological impairment and other complications.
Mutations in the CPS1 gene are associated with carbamoyl phosphate synthetase 1 deficiency, a rare genetic disorder characterized by impaired urea cycle function. This condition can lead to severe hyperammonemia and neurological symptoms, posing significant health risks if left untreated. One study suggests CPS1 is a robust acute liver injury (ALI) biomarker. Another study revealed CPS1’s prognostic potential in acute liver failure (ALF). Further research may elucidate the potential therapeutic implications of targeting CPS1 and its utility as a diagnostic marker in clinical settings.
NeoBiotechologies offers a variety of antibodies against CPS1 that have been validated for immunofluorescence and immunohistochemistry. Additionally, we hold exclusive rights to CPS1 antibodies available for licensing or collaboration [https://www.neobiotechnologies.com/shop/?s=CPS1].
Carbamoyl-phosphate synthase [ammonia], mitochondrial, Carbamoyl-phosphate synthetase I, Carbamoyl-phosphate synthetase 1 (CPS1); Carbamoylphosphate synthetase 1; CPSase 1; CPSASE1
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