CPS1

CPS1 (Carbamoyl-phosphate synthase 1): CPS1, a key enzyme in the urea cycle, plays a crucial role in the detoxification of ammonia in the body. This enzyme catalyzes the formation of carbamoyl phosphate from ammonia and bicarbonate, initiating the synthesis of urea in the liver. The urea cycle is essential for removing excess nitrogen generated from the breakdown of proteins and amino acids, preventing the accumulation of toxic ammonia in the bloodstream. CPS1 activity is tightly regulated to maintain nitrogen balance and prevent hyperammonemia, a condition associated with neurological impairment and other serious complications. Dysregulation of CPS1 function can lead to urea cycle disorders, characterized by impaired ammonia detoxification and metabolic abnormalities. Understanding the intricate regulation of CPS1 and its role in nitrogen metabolism is crucial for developing targeted therapies for urea cycle disorders and related metabolic conditions.