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Key Documents

AV51183

Sigma-Aldrich

Anti-NAGS antibody produced in rabbit

IgG fraction of antiserum

Synonym(s):

Anti-AGAS, Anti-ARGA, Anti-MGC133025, Anti-N-Acetylglutamate synthase

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About This Item

UNSPSC Code:
12352203
NACRES:
NA.41

biological source

rabbit

conjugate

unconjugated

antibody form

IgG fraction of antiserum

antibody product type

primary antibodies

clone

polyclonal

form

buffered aqueous solution

mol wt

58 kDa

species reactivity

rat, mouse, rabbit, pig, human, dog, bovine, horse

concentration

0.5 mg - 1 mg/mL

technique(s)

western blot: suitable

NCBI accession no.

NP_694551

UniProt accession no.

Q8N159

shipped in

wet ice

storage temp.

−20°C

target post-translational modification

unmodified

Gene Information

human ... NAGS(162417)

Immunogen

Synthetic peptide directed towards the C terminal region of human NAGS

Application

Anti-NAGS antibody produced in rabbit is suitable for western blotting at a concentration of 1.25μg/ml.

Biochem/physiol Actions

N-acetylglutamate synthase (NAGS) is a mitochondrial enzyme that catalyzes the reaction between glutamate and acetyl coenzyme-A to form N-acetylglutamate. NAG regulates ureagenesis since it is the cofactor of the enzyme (carbamyl phosphate synthetase I) that catalyzes the first step of the urea cycle. Deficiency of NAGS results in hyperammonemia.

Sequence

Synthetic peptide located within the following region: YLDKFVVSSSRQGQGSGQMLWECLRRDLQTLFWRSRVTNPINPWYFKHSD

Physical form

Purified antibody supplied in 1x PBS buffer with 0.09% (w/v) sodium azide and 2% sucrose.

Disclaimer

Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.

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Storage Class

12 - Non Combustible Liquids

wgk_germany

WGK 3

flash_point_f

Not applicable

flash_point_c

Not applicable

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Certificates of Analysis (COA)

Lot/Batch Number
QC16619

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A Nordenström et al.
Journal of inherited metabolic disease, 30(3), 400-400 (2007-05-19)
N-acetylglutamate synthase (NAGS) deficiency is a rare urea cycle disorder. An effective treatment, N-carbamoyl-L-glutamic acid (NCGA), is now available, increasing the importance of identifying and treating these patients early. We describe a case with genetically verified NAGS deficiency and neonatal
Peter Gessler et al.
European journal of pediatrics, 169(2), 197-199 (2009-06-18)
N-Acetylglutamate synthase (NAGS) deficiency is a rare urea cycle disorder, which may present in the neonatal period with severe hyperammonemia and marked neurological impairment. We report on a Turkish family with a patient who died due to hyperammonemia in the

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