CPS1 (Carbamoyl-phosphate synthase 1): CPS1 is a crucial enzyme in the urea cycle that plays an essential role in detoxifying ammonia in the body. It catalyzes the formation of carbamoyl phosphate from ammonia and bicarbonate, initiating the process of urea synthesis in the liver. The urea cycle is vital for removing excess nitrogen generated from protein and amino acid breakdown, preventing the toxic accumulation of ammonia in the bloodstream. CPS1 activity is tightly regulated to maintain nitrogen balance and prevent hyperammonemia, a condition associated with neurological damage and other serious complications.