CPS1 (Carbamoyl-phosphate synthase 1): CPS1 is a key enzyme in the urea cycle that plays a vital role in detoxifying ammonia in the body. It catalyzes the formation of carbamoyl phosphate from ammonia and bicarbonate, starting the process of urea synthesis in the liver. The urea cycle is essential for removing excess nitrogen produced from protein and amino acid breakdown, preventing toxic ammonia buildup in the bloodstream. CPS1 activity is carefully regulated to maintain nitrogen balance and avoid hyperammonemia, a condition linked to neurological damage and other serious complications.