HPA009150
Anti-NPHP3 antibody produced in rabbit
Prestige Antibodies® Powered by Atlas Antibodies, affinity isolated antibody, buffered aqueous glycerol solution
Synonym(s):
Anti-CFAP31, Anti-FLJ30691, Anti-FLJ36696, Anti-KIAA2000, Anti-MKS7, Anti-NPH3, Anti-SLSN3
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100 μL
$598.00
$598.00
Usually ships in 1 week. (Orders outside of US and Europe, please allow an additional 1-2 weeks for delivery)
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100 μL
$598.00
About This Item
$598.00
Usually ships in 1 week. (Orders outside of US and Europe, please allow an additional 1-2 weeks for delivery)
biological source
rabbit
conjugate
unconjugated
antibody form
affinity isolated antibody
antibody product type
primary antibodies
clone
polyclonal
product line
Prestige Antibodies® Powered by Atlas Antibodies
form
buffered aqueous glycerol solution
species reactivity
human
technique(s)
immunohistochemistry: 1:50- 1:200
immunogen sequence
PEFAHSSIDVEGPFANVNRDDWDIAVASLLQVTPLFSHSLWSNTVRCYLIYTDETQPEMDLFLKDYSPKLKRMCETMGYFFHAVYFPIDVENQYLTVRKWEIEKS
UniProt accession no.
shipped in
wet ice
storage temp.
−20°C
target post-translational modification
unmodified
Gene Information
human ... NPHP3(27031)
General description
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.
Immunogen
Nephrocystin-3 recombinant protein epitope signature tag (PrEST)
Application
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.
Biochem/physiol Actions
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.
Features and Benefits
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.
Linkage
Corresponding Antigen APREST71412
Physical form
Solution in phosphate-buffered saline, pH 7.2, containing 40% glycerol and 0.02% sodium azide
Legal Information
Prestige Antibodies is a registered trademark of Merck KGaA, Darmstadt, Germany
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
10 - Combustible liquids
wgk_germany
WGK 1
flash_point_f
Not applicable
flash_point_c
Not applicable
ppe
Eyeshields, Gloves, multi-purpose combination respirator cartridge (US)
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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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