Carglumic acid is primarily used in the treatment of hyperammonemia, a condition characterized by elevated levels of ammonia in the blood. It is particularly effective in managing hyperammonemia caused by N-acetylglutamate synthase (NAGS) deficiency, a rare genetic disorder. By acting as a substitute for N-acetylglutamate, it helps activate the urea cycle, enabling the body to convert toxic ammonia into urea, which is then excreted through urine. This helps prevent the neurological damage and life-threatening complications associated with high ammonia levels. Additionally, it may be used in conjunction with other therapies for other urea cycle disorders to stabilize ammonia levels and improve patient outcomes.