Homo sapiens nephrosis 1, congenital, Finnish type (nephrin) (NPHS1)
Synonyms:
CNF; nephrin; NPHN
Immunogen
A synthetic peptide corresponding to residues surrounding tyrosine 1217 of mouse Nephrin was used an immunogen. This antibody detects both Nephrin phosphorylated on tyrosine 1217.
Buffer
Store at -20C. Buffer: 50 mM Tris-Glycine (pH 7.4), 0.15 M NaCl, 40% Glycerol, 0.01% sodium azide and 0.05% BSA. Stable for 12 months from date of receipt.
Nephrin (NPHS1) is an immunoglobulin family adhesion protein expressed in visceral glomerular epithelial cells (podocytes), where it is targeted to the intercellular slit diaphragm of mature foot processes (1). It is an essential component of the kidney filtration barrier, and mutations in the nephrin gene are associated with congenital nephrotic syndrome (2). Nephrin binds via phosphorylated tyrosine residues in the intracellular domain to SH2 domain-containing signaling molecules such as Nck (3).
Related Pathway
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A. Western blot analysis on (A) HEK293T cells transfected with wild type CD 16/7 Nephrin (B) HEK293T cells transfected with active tyrosine kinase (vSrc) (C) HEK293T cells transfected with Nephrin mutated at Tyr1217 and vSrc using Phospho Nephrin (pY1176/1193) RabMAb .
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