CPS1 and hyperammonemia due to N-acetylglutamate synthase deficiency: For UCDs other than NAGSD, ammonia/nitrogen scavenger drugs (sodium benzoate, sodium phenylbutyrate, and glycerol phenylbutyrate) are used to control ammonia levels.5, 6N‐carbamylglutamate is a CPS1 activator that is used to replace NAGS in patients with NAGSD (and some with CPS1D).