AV51183
Anti-NAGS antibody produced in rabbit
IgG fraction of antiserum
别名:
Anti-AGAS, Anti-ARGA, Anti-MGC133025, Anti-N-Acetylglutamate synthase
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关于此项目
NACRES:
NA.41
UNSPSC Code:
12352203
Conjugate:
unconjugated
Clone:
polyclonal
Application:
WB
Citations:
2
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.
UniProt accession no.
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.
Physical form
Purified antibody supplied in 1x PBS buffer with 0.09% (w/v) sodium azide and 2% sucrose.
Other Notes
Synthetic peptide located within the following region: YLDKFVVSSSRQGQGSGQMLWECLRRDLQTLFWRSRVTNPINPWYFKHSD
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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存储类别
12 - Non Combustible Liquids
wgk
WGK 3
flash_point_f
Not applicable
flash_point_c
Not applicable
法规信息
新产品
此项目有
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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