Hyperammonemia type I is due to deficiency of -
Ans. is 'a' i.e., CPS-I o Deficiency of any of the urea cycle enzyme would result in hyperammonemiaQ.o When the block is in one of the earlier steps, the condition is more severe, since ammonia itself accumulates,o Deficiency of later enzymes result in accumulation of other intermediates which are less toxic and hence symptoms are less.Disorders caused by genetic defects of urea cycle enzymesDisordersHyperammonemia type-IHyperammonemia type-IICitrullinemiaArgininosuccinic aciduriaArgininemiaDefective enzymeCarbamoyl phosphate synthase-IOrnithine transcarbamoylaseArginosuccinate synthaseArgininosuccinate lyaseArginaseProducts accumulatedAmmoniaAmmoniaCitrullineArgininosuccinateArginine
CPS-I
CPS-n
Arginase
Arginosuccinase
Urea Cycle and Disorders