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|Tested species reactivity||Human, Mouse, Rat|
|Host / Isotype||Rabbit / IgG|
|Immunogen||A synthetic peptide corresponding to residues in human Nephrocystin-1|
|Purification||Antigen affinity chromatography|
|Storage buffer||PBS, pH 7.2|
|Contains||0.05% sodium azide|
|Storage Conditions||Store at 4°C short term. For long term storage, store at -20°C, avoiding freeze/thaw cycles.|
|Tested Applications||Dilution *|
|Western Blot (WB)||1:500-1:1000|
* Suggested working dilutions are given as a guide only. It is recommended that the user titrate the product for use in their own experiment using appropriate negative and positive controls.
This antibody detects endogenous protein at a molecular weight of 83 kDa.
Purity is >95% by SDS-PAGE.
Juvenile nephronophthisis type 1 is caused by mutations of NPHP1, the gene encoding for nephrocystin. Nephrocystin interacts with p130Cas (BCAR1), proline-rich tyrosine kinase-2 (PTK2B or Pyk2), and tensin in embryonic kidney and testis, indicating that these proteins participate in a common signaling pathway. Nephrocystin and p130Cas interact in mammalian cells and both proteins prominently localize at or near sites of cell-cell contact in polarized Madin-Darby canine kidney epithelial cells. Expression of nephrocystin results in phosphorylation of Pyk2 on Tyrosine 402 as well as activation of downstream mitogen-activated protein kinases, such as ERK1 and ERK2. Nephrocystin contains a src-homology 3 (SH3) domain, which is highly conserved throughout evolution.
For Research Use Only. Not for use in diagnostic procedures. Not for resale without express authorization.
Juvenile nephronophthisis 1 protein; Nephrocystin 1; Nephrocystin-1; Nephrocystin1; nephronophthisis 1 (juvenile); NPH1; NPHP1
JBTS4; NPH1; NPHP1; SLSN1