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N-acetylglutamate (NAG)
Activates carbamoyl phosphate synthetase-1 in urea cycle.
Carbamoyl phosphate synthetase-1 (CPS-1)
Enzyme that catalyzes carbamoyl phosphate formation.
Ornithine
Amino acid that combines with carbamoyl phosphate.
Citrulline
Product of ornithine and carbamoyl phosphate reaction.
Argininosuccinate synthetase
Enzyme that converts citrulline and aspartate to argininosuccinate.
Arginase
Enzyme that hydrolyzes arginine to urea and ornithine.
Fumarate
Intermediate produced during argininosuccinate breakdown.
Urea
Final product of the urea cycle, excreted in urine.