<prompt> Glutamine is increased in CSF, blood urine in which defect:Arginosuccinate synthetaseOTCCPS-IArginase  <response>: 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. 