Nephrin (NPHS1) (1243-1256) Guinea Pig Polyclonal Antibody

CAT#: BP5030

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Nephrin (NPHS1) (1243-1256) guinea pig polyclonal antibody, Serum



USD 539.00


Availability*
In Stock

Size
    • 100 ul


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Specifications

Product Data
Applications IF, IHC, WB
Recommended Dilution Western blot: 1/500.
Immunohistochemistry/Immunofluorescence Microscopy on frozen tissue: 1/50.
Immunohistochemistry on Paraffin Sections: 1/50 (microwave treatment recommended).
Incubation Time: 1 h at RT or overnight at 2-8°C.
Reactivities Human, Mouse
Host Guinea Pig
Clonality Polyclonal
Immunogen Synthetic peptide (intracellular domain, aa1243-1256)
EPGSLPFELRGHLV
Specificity The antibody reacts specifically with Nephrin.
Immunolocalization: Antibody BP5030 stains positively podocytes of the kidney, radial glial cells of the brain, Sertoli cells of testis, and beta islet cells of the pancreas.
Tested Reactivities on Cultured Cell Lines: PCL (Podocyte Cell Line), M-1 (Cortical Collecting Duct Cells).
Formulation State: Serum
State: Whole Antiserum
Preservative: 0.09% Sodium Azide
Conjugation Unconjugated
Storage Store undiluted at 2-8°C for one month or (in aliquots) at -20°C for longer.
Avoid repeated freezing and thawing.
Stability Shelf life: one year from despatch.

Background Nephrin was first described as a kidney podocyte marker protein (MW 135,000 calculated from aa sequence data; apparent Mr 185,000 after SDS-PAGE). Nephrin is a transmembrane cell adhesion molecule located at the slit diaphragm.
Primary steroid resistant nephrotic syndrome (SRNS) is characterized by childhood onset of proteinuria and progression to end stage renal disease. Kidney podocytes and their slit diaphragms form the final barrier to urinary protein loss. Congenital nephrotic syndrome (CNS) is caused by mutations in NPHS1 (nephrin) or NPHS2. Nephrin, a recently identified protein is a member of a group of podocyte proteins that constitute major component of the slit diaphragm especially in the foot process. Nephrin, a cell adhesion molecule, may play a crucial role in maintaining the glomerular filtration barrier. Recent studies have suggested that mutations in the gene for Nephrin reportedly lead to congenital nephrosis. Three novel podocyte proteins, Podocin, Nephrin and alpha Actinin 4 have been identified in congenital and experimental models of proteinuria. The role of Nephrin in anti apoptotic activity in podocyte slit diaphragm is believed to be associated with vascular endothelial derived growth factors VEGF signaling.
Synonyms NPHS1, NPHN
Reference Data
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