In all of the following enzyme deficiencies, hyperammonemia is a common feature, EXCEPT:
Excess ornithine from urea cycle is taken by enzyme OAT (Ornithine amino transferase) OAT deficiency leads to gyrate atrophy. In this deficiency, plasma, urine, spinal fluid and aqueous humour concentration of ornithine levels are 10-20 times higher than normal Hyperammonemia is absent because urea cycle is not disrupted in this deficiency Hyperammonemia occurs in enzyme deficiency of Urea cycle
Argininosuccinate synthetase
Carbamoyl Phosphate synthetase (CPS-I)
Ornithine transcarbamoylase (OTC)
Ornithine amino transferase
Urea cycle