Anti-nphp3 antibody produced in rabbit prestige antibodies(r) powered by atlas antibodies, affinity isolated antibody,

Non disponible en dehors du Royaume-Uni et de l'Irlande

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.

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.

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: 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.

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)

Legal Information

Prestige Antibodies is a registered trademark of Sigma-Aldrich Co. LLC

Linkage

Corresponding Antigen APREST71412.

Physical form

Solution in phosphate-buffered saline, pH 7.2, containing 40% glycerol and 0.02% sodium azide