Hyperammonemia type I is due to deficiency of
Deficiency of any of the urea cycle enzymes result in hyperammonemia. Carbamoyl phosphate synthetase1 deficiency or hyperammonemia type 1 is comparatively rare and is characterized by severe hyperammonemia.Ref: DM Vasudevan - Textbook of Biochemistry, 7th edition, page no: 205
CPS-I
CPS-II
Arginase
Arginosuccinase
Metabolism of protein and amino acid