Mutations in the CPS1 gene cause<unk> .<unk>  belongs to a class of genetic diseases called urea cycle disorders. The urea cycle is a sequence of reactions that occurs in liver cells. This cycle processes excess nitrogen, generated when protein is used by the body, to make a compound called urea that is excreted by the kidneys. In<unk> , the enzyme that regulates the urea cycle is damaged or missing. The urea cycle cannot proceed normally, and nitrogen accumulates in the bloodstream in the form of ammonia. Ammonia is especially damaging to the nervous system, and excess ammonia causes neurological problems and other signs and symptoms of<unk> .
Carbamoyl phosphate synthetase I deficiency
Malonyl-CoA decarboxylase deficiency
Holocarboxylase synthetase deficiency
Aminoacylase 1 deficiency
Pyruvate kinase deficiency
2,4 Dienoyl-CoA reductase deficiency
Guanidinoacetate methyltransferase deficiency
Arginine:glycine amidinotransferase deficiency