CARGLUMIC ACID IS A SYNTHETIC STRUCTURAL ANALOGUE OF N-ACETYLGLUTAMATE (NAG), WHICH IS AN ESSENTIAL ALLOSTERIC ACTIVATOR OF THE LIVER ENZYME CARBAMOYL PHOSPHATE SYNTHETASE 1 (CPS1). CPS1 IS FOUND IN THE MITOCHONDRIA AND IS THE FIRST ENZYME OF THE UREA CYCLE, WHICH CONVERTS AMMONIA INTO UREA. CARGLUMIC ACID ACTS AS A REPLACEMENT FOR NAG IN NAGS DEFICIENCY PATIENTS BY ACTIVATING CPS1 BUT IT DOES NOT HELP TO REGULATE THE UREA CYCLE.