Most common genentic enzyme defect in urea cycle is
OTC combines carbamyl phosphate with ornithine to make citrulline which is subsequently processed tourea (see: cycle diagram). Along with CPSI and NAGS deficiency, OTCdeficiency is the most severe of theurea cycle disorders. Patients with complete OTC deficiency rapidly develop hyperammonemia in the newborn period Ref-Harpers illustrated biochemistry 30/e p583
Arginase
Arginosuccunate lyase
Ornithine transcarbomylase
Carbomoyl phosphate synthase I
Metabolism of nucleic acids