Mechanism action of Carglumic acid
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 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.
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