<unk>  (CPS I deficiency) is an autosomal recessive metabolic disorder that causes ammonia to accumulate in the blood due to a lack of the enzyme<unk><unk>  synthetase I. Ammonia, which is formed when proteins are broken down in the body, is toxic if the levels become too high. The nervous system is especially sensitive to the effects of excess ammonia.
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