Anti-NAGS Antibody

Raised in rabbit against human N-Acetylglutamate Synthase (NAGS), this polyclonal IgG antibody from Antibodies.com enables immunodetection of the endogenous mitochondrial matrix enzyme by Western blot. NAGS catalyzes the formation of N-acetylglutamate from glutamate and acetyl-CoA; this metabolite is the essential allosteric activator of carbamoyl phosphate synthetase I, the first committed step of the hepatic urea cycle. Recessive loss-of-function variants in NAGS cause N-acetylglutamate synthase deficiency, a urea cycle disorder presenting with neonatal or late-onset hyperammonemia that is pharmacologically responsive to N-carbamylglutamate (carglumic acid).

Supplied as 100 µl of unconjugated antiserum in a single vial, the reagent is intended for research use only, supporting in vitro characterization of patient-derived samples, hepatocyte and hepatic cell line models, and recombinant expression systems probing urea cycle flux, mitochondrial nitrogen handling, and genotype-phenotype correlations in hyperammonemia. Host: Rabbit. Clonality: Polyclonal. Isotype: IgG. Reactivity: Human. Conjugate: Unconjugated. Application: WB.