HPA009150
Anti-NPHP3 antibody produced in rabbit
Prestige Antibodies® Powered by Atlas Antibodies, affinity isolated antibody, buffered aqueous glycerol solution
别名:
Anti-CFAP31, Anti-FLJ30691, Anti-FLJ36696, Anti-KIAA2000, Anti-MKS7, Anti-NPH3, Anti-SLSN3
生物来源
rabbit
质量水平
偶联物
unconjugated
抗体形式
affinity isolated antibody
抗体产品类型
primary antibodies
克隆
polyclonal
产品线
Prestige Antibodies® Powered by Atlas Antibodies
表单
buffered aqueous glycerol solution
种属反应性
human
技术
immunohistochemistry: 1:50- 1:200
免疫原序列
PEFAHSSIDVEGPFANVNRDDWDIAVASLLQVTPLFSHSLWSNTVRCYLIYTDETQPEMDLFLKDYSPKLKRMCETMGYFFHAVYFPIDVENQYLTVRKWEIEKS
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 ... NPHP3(27031)
相关类别
一般描述
NPHP3 (adolescent nephronophthisis 3) gene is localized to 3q21-22, and belongs to a group of proteins linked with nephronophthisis (NPHP). NPHP is a group of cystic kidney disorder, inherited in an autosomal recessive manner. NPHP3 protein is composed of 1330 amino acids, and in mice, it is expressed in retina, respiratory epithelium, neural tissues, biliary tract, kidney tubules, and liver.
免疫原
Nephrocystin-3 recombinant protein epitope signature tag (PrEST)
应用
Anti-NPHP3 antibody produced in rabbit, a Prestige Antibody, is developed and validated by the Human Protein Atlas (HPA) project . Each antibody is tested by immunohistochemistry against hundreds of normal and disease tissues. These images can be viewed on the Human Protein Atlas (HPA) site by clicking on the Image Gallery link. The antibodies are also tested using immunofluorescence and western blotting. To view these protocols and other useful information about Prestige Antibodies and the HPA, visit sigma.com/prestige.
生化/生理作用
Null mutations in NPHP3 (adolescent nephronophthisis 3) are involved in the pathogenesis of renal-hepatic-pancreatic dysplasia (RHPD). It is an autosomal recessive disorder. In mice, homozygous missense mutation in this protein might be linked with polycystic kidney disease (pcy) phenotype. Mutations in this gene are linked with isolated NPHP cases and tapeto-retinal degeneration. It functions as an interacting partner of nephrocystin.
特点和优势
Prestige Antibodies® are highly characterized and extensively validated antibodies with the added benefit of all available characterization data for each target being accessible via the Human Protein Atlas portal linked just below the product name at the top of this page. The uniqueness and low cross-reactivity of the Prestige Antibodies® to other proteins are due to a thorough selection of antigen regions, affinity purification, and stringent selection. Prestige antigen controls are available for every corresponding Prestige Antibody and can be found in the linkage section.
Every Prestige Antibody is tested in the following ways:
Every Prestige Antibody is tested in the following ways:
- IHC tissue array of 44 normal human tissues and 20 of the most common cancer type tissues.
- Protein array of 364 human recombinant protein fragments.
外形
Solution in phosphate-buffered saline, pH 7.2, containing 40% glycerol and 0.02% sodium azide
其他说明
Corresponding Antigen APREST71412
法律信息
Prestige Antibodies is a registered trademark of Merck KGaA, Darmstadt, Germany
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
10 - Combustible liquids
WGK
WGK 1
闪点(°F)
Not applicable
闪点(°C)
Not applicable
个人防护装备
Eyeshields, Gloves, multi-purpose combination respirator cartridge (US)
法规信息
新产品
Gaëlle Blandin et al.
Skeletal muscle, 3(1), 3-3 (2013-02-19)
The complexity of the skeletal muscle and the identification of numerous human disease-causing mutations in its constitutive proteins make it an interesting tissue for proteomic studies aimed at understanding functional relationships of interacting proteins in both health and diseases. We
Heike Olbrich et al.
Nature genetics, 34(4), 455-459 (2003-07-23)
Nephronophthisis (NPHP), a group of autosomal recessive cystic kidney disorders, is the most common genetic cause of progressive renal failure in children and young adults. NPHP may be associated with Leber congenital amaurosis, tapeto-retinal degeneration, cerebellar ataxia, cone-shaped epiphyses, congenital
Lawrence Copelovitch et al.
American journal of medical genetics. Part A, 161A(7), 1743-1749 (2013-05-21)
We report on five consecutive sibs three with fatal renal-hepatic-pancreatic dysplastic (RHPD) syndrome and two pregnancies ending in early abortion. Three of the fetuses reached term and two survived for 15 and 58 days. They had diffusely cystic kidneys with
Torunn Fiskerstrand et al.
The Journal of molecular diagnostics : JMD, 12(1), 125-131 (2009-12-17)
We have investigated a family where two siblings had a developmental disorder associated with polycystic dysplastic kidney disease that was incompatible with postnatal survival. Additional features observed were ductal plate malformation in the liver, dysplasia of the pancreas, and (in
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