Abstract:Inborn defects in the carbamoylphosphate synthase 1 (CPS1) cause a reduction of N‐acetylglutamate (NAG), an essential cofactor for the function of the urea cycle, and consequently elevated blood levels of the neurotoxic ammonia. N‐carbamoylglutamate (carglumic acid, carbaglu®, NCG) is a structural analog of NAG that can substitute for NAG on CPS1, thereby reactivating the urea cycle and finally reducing blood ammonia levels. The renal clearance of NCG exceeds the glomerular filtration rate suggesting an active… Show more
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