Used in the treatment of hyperammonemia, particularly in patients with N-acetylglutamate synthase (NAGS) deficiency. It acts as a substitute for N-acetylglutamate, which is a required activator of carbamoyl phosphate synthetase 1 (CPS1), a key enzyme in the urea cycle. By activating CPS1, it helps lower elevated blood ammonia levels. Also used off-label in other urea cycle disorders and acute hyperammonemic crises. Administered orally, it supports long-term management and acute intervention in both pediatric and adult patients.