Carbamoyl phosphate synthetase 1 (CPS1)

Carbamoyl phosphate synthetase 1 (*CPS1*) is a mitochondrial enzyme encoded by the *CPS1* gene located on chromosome 2q34. CPS1 catalyzes the first, rate-limiting step of the urea cycle by combining ammonia and bicarbonate to form carbamoyl phosphate, which is essential for detoxification of ammonia in the liver. Its mRNA is mainly expressed in hepatocytes. Mutations or repression of *CPS1* lead to hyperammonemia, resulting in severe neurological damage or death. It is of interest both as a biomarker of liver function and as a potential RNA-level target for intervention in certain liver cancers and metabolic disorders[1][3][4][6][7][9].

Other names

CPSICPS 1Carbamoyl-phosphate synthase 1Carbamoyl phosphate synthase 1

For ammonia-scavenging: Compounds induce CPS1 expression to enhance ammonia elimination\nFor experimental silencing: RNAi/antisense to reduce CPS1 mRNA for research in cancer cell lines