推荐产品
生物来源
rabbit
偶联物
unconjugated
抗体形式
IgG fraction of antiserum
抗体产品类型
primary antibodies
克隆
polyclonal
表单
buffered aqueous solution
分子量
58 kDa
种属反应性
rat, mouse, rabbit, pig, human, dog, bovine, horse
浓度
0.5 mg - 1 mg/mL
技术
western blot: suitable
NCBI登记号
UniProt登记号
运输
wet ice
储存温度
−20°C
靶向翻译后修饰
unmodified
基因信息
human ... NAGS(162417)
免疫原
Synthetic peptide directed towards the C terminal region of human NAGS
应用
Anti-NAGS antibody produced in rabbit is suitable for western blotting at a concentration of 1.25μg/ml.
生化/生理作用
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.
序列
Synthetic peptide located within the following region: YLDKFVVSSSRQGQGSGQMLWECLRRDLQTLFWRSRVTNPINPWYFKHSD
外形
Purified antibody supplied in 1x PBS buffer with 0.09% (w/v) sodium azide and 2% sucrose.
免责声明
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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储存分类代码
12 - Non Combustible Liquids
WGK
WGK 3
闪点(°F)
Not applicable
闪点(°C)
Not applicable
法规信息
新产品
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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