NAGS Antibody (E-8): sc-515127

NAGS Antibody (E-8) is a mouse monoclonal IgG1 kappa light chain antibody that detects NAGS protein of human origin by western blotting (WB), immunoprecipitation (IP), immunofluorescence (IF), and enzyme-linked immunosorbent assay (ELISA). Anti-NAGS antibody (E-8) is available in both non-conjugated and various conjugated forms, including agarose, horseradish peroxidase (HRP), phycoerythrin (PE), fluorescein isothiocyanate (FITC), and multiple Alexa Fluor® conjugates. N-acetylglutamate synthase (NAGS) plays a crucial role in the urea cycle, which is essential for removing excess nitrogen from the body. This enzyme catalyzes the formation of N-acetylglutamate from glutamate and acetylcoenzyme A, serving as an obligatory activator of carbamyl phosphate synthetase I (CPSI), the first and rate-limiting enzyme in the urea cycle. A deficiency in NAGS can lead to severe hyperammonemia, a potentially life-threatening condition characterized by elevated ammonia levels in the blood. In humans, 21 mutations in the NAGS gene have been identified, with ten mutations linked to acute neonatal hyperammonemia and others associated with late-onset forms of the disorder. Treatment options, such as N-carbamylglutamate, have shown promise in alleviating hyperammonemia in patients with inherited and secondary NAGS deficiencies. The expression of NAGS is primarily localized in the liver, small intestine, and kidney, highlighting its importance in metabolic processes across these vital organs.

Alexa Fluor^® is a trademark of Molecular Probes Inc., OR., USA

LI-COR^® and Odyssey^® are registered trademarks of LI-COR Biosciences

NAGS Antibody (E-8) References:

1. Cloning and expression of the human N-acetylglutamate synthase gene.  |  Caldovic, L., et al. 2002. Biochem Biophys Res Commun. 299: 581-6. PMID: 12459178
2. Mutation analysis in patients with N-acetylglutamate synthase deficiency.  |  Häberle, J., et al. 2003. Hum Mutat. 21: 593-7. PMID: 12754705
3. Mammalian N-acetylglutamate synthase.  |  Morizono, H., et al. 2004. Mol Genet Metab. 81 Suppl 1: S4-11. PMID: 15050968
4. Biochemical properties of recombinant human and mouse N-acetylglutamate synthase.  |  Caldovic, L., et al. 2006. Mol Genet Metab. 87: 226-32. PMID: 16321554
5. Mutations and polymorphisms in the human N-acetylglutamate synthase (NAGS) gene.  |  Caldovic, L., et al. 2007. Hum Mutat. 28: 754-9. PMID: 17421020
6. A trial with N-carbamylglutamate may not detect all patients with NAGS deficiency and neonatal onset.  |  Nordenström, A., et al. 2007. J Inherit Metab Dis. 30: 400. PMID: 17510757
7. Contrasting features of urea cycle disorders in human patients and knockout mouse models.  |  Deignan, JL., et al. 2008. Mol Genet Metab. 93: 7-14. PMID: 17933574
8. N-carbamylglutamate markedly enhances ureagenesis in N-acetylglutamate deficiency and propionic acidemia as measured by isotopic incorporation and blood biomarkers.  |  Tuchman, M., et al. 2008. Pediatr Res. 64: 213-7. PMID: 18414145

Monoclonal antibodies for mammalian and non-mammalian protein targets represent essentially all targets covered by polyclonal antibodies. Over 8,300 of our antibodies are offered as ImmunoCruz^® conjugates which eliminates the need for a secondary antibody.

Santa Cruz Biotechnology antibodies have over 360,000 research citations. Anti-NAGS Antibody (E-8) has 0 citations in a variety of scientific publications.

Primary antibodies like Anti-NAGS Antibody (E-8) for mammalian target proteins are recommended for the detection of a range of mammalian species, primarily of mouse, rat and human species. Many mammalian antibodies are also ideal for veterinary research and reactive with ovine, porcine, caprine, feline, canine, bovine and equine protein targets. Santa Cruz Biotechnology primary monoclonal antibodies are suitable for laboratory research applications and are not intended for diagnostic or therapeutic use.

NAGS Antibody (E-8) Specifications

Alternate Product Name: NAGS

Product Clone Name: E-8

Classification: Primary Antibody

Clonality: Monoclonal Antibody

Antibody Isotype: IgG₁ kappa light chain

Amino Acids: 226-341

Epitope Target: NAGS

Epitope Species: human

Product Reactivity/Detection: NAGS

Species Reactivity/Detection: human

Additional Mammalian Reactivity: N/A

Additional Non-Mammalian Reactivity: N/A

Applications: WB, IP, IF, ELISA

Vialing Information: Each vial contains 200 µg IgG₁ kappa light chain in 1.0 ml of PBS with < 0.1% sodium azide and 0.1% gelatin

Research Citations: 0 citations in various scientific publications

Available Conjugates/Forms for NAGS Antibody (E-8): AC: Agarose, AF488: Alexa Fluor^® 488, AF546: Alexa Fluor^® 546, AF594: Alexa Fluor^® 594, AF647: Alexa Fluor^® 647, AF680: Alexa Fluor^® 680, AF790: Alexa Fluor^® 790, FITC: Fluoroscein, HRP: Horseradish Peroxidase, PE: Phycoerythrin