This is a real-world medical entrance exam question, please give the true answer based on the question and selection. Topic name: NEET 2019, Subject name: Biochemistry
[{'content': 'Glutamine is increased in CSF, blood & urine in which defect: \nSelect and explain. \nA. Arginosuccinate synthetase, B. OTC, C. CPS-I, D. Arginase', 'role': 'user'}, {'content': 'Answer: C \nExplanation: Defect in urea cycle Defect Enzyme involved Hyperammonemia type I Carbamoyl phosphate synthase I Hyperammonemia type II Ornithine transcarbamoylase Citrullinemia Arginosuccinate synthase Arginosuccinic aciduria Arginosuccinase Hyperarginemia Arginase In all enzyme deficiency ammonia will increase but cps 1 is rate limiting enzyme so it will increase ammonia most & and increase in ammonia lead to increase in glutamine. NOTE: The only urea cycle disorder that is inheritedX-linked recessively ishyperammonemia type II.', 'role': 'assistant'}]