Technical Data
N2028-52A
Nephrin (NPHN, CNF, Nephrosis 1 Congenital Finnish Type, NPHS1, Renal Glomerulus-specific Cell Adhesion Receptor)
Description:
Nephrin is encoded by NPHS1 gene (chromosome 19q13.1), the core protein of the interpodocytes slit diaphragm of kidney glomerulus, these podocytes are highly differentiated with characteristic interdigitating foot processes covering the outer glomerular basement membrane, the space between these foot processes is spanned by a tight junction to provide the large surface area for filtration. The mutated NPHS1 gene in congenital nephrotic syndrome of Finnish type, a human disease that leads to massive proteinuria in utero and nephrosis at birth. Nephrin is a transmembrane protein of immunoglobulin super family with 8 Ig like domains, identified in human (1241aa), rat (1252aa), and mouse (1256aa). Nephrin is found to be specifically expressed in kidney-slit diaphragm, brain and pancreas, the protein plays a crucial role in development and function of kidney filtration barrier, this barrier is crucial for maintaining the water and electrolyte balance without losing circulating proteins into the urine. Nephrin interacts with podocin and with CD2AP C-terminal domain, It tends to be a signaling molecule that activates canonical protein kinase cascades, which is initiated by three closely related proteins called NEPH proteins (NEPH1, NEPH2, & NEPH3), The NEPH proteins share a common domain architecture consisting of 5 extracellular Ig domain followed by transmembrane domain, These proteins bind to C-terminal domain of podocin, which interacts with C-terminal of nephrin and greatly enhances nephrin-induced signaling. Filtrin a type I transmembrane protein belonging to immunoglobulin superfamily of cell adhesion molecules, due to its structural similarity and sequence homology with nephrin, another protein named Podocalyxin belongs to the same functionally important complex of proteins of glomerular filtration barrier and is associated with proteinuria.

Applications:
Suitable for use in ELISA and Western Blot. Other applications not tested.

Recommended Dilution:
ELISA: 1:10,000-1:50,000; Control peptide can be used to coat ELISA plates at 1ug/ml. See N2028-52B.
Western Blot: 1:1000-1:5000 using ECL technique.
Optimal dilutions to be determined by the researcher.

Storage and Stability:
May be stored at 4C for short-term only. For long-term storage, store at -20C. Aliquots are stable for at least 12 months at -20C. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap. Further dilutions can be made in assay buffer.
TypeIsotypeCloneGrade
PabSerum
SizeStorageShippingSourceHost
100ul-20CBlue IceRatRabbit
Concentration:
As reported
Immunogen:
20aa peptide from rat NPHN1. Location: ~C-terminus, Intracellular domain. Species Sequence Homology: Human, mouse - 81%.
Purity:
Serum
Form
Supplied as a lyophilized powder from PBS, 0.05% sodium azide.
Specificity:
Recognizes rat Nephrin.
Intended for research use only. Not for use in human, therapeutic, or diagnostic applications.
Yuan H et al (2002) Am.J. Physiol 282, F585-F591, Heli Putaala et al (2001) Human Mol. Gen.10, 1-8; Dorbit B.Donoviel et al (2001) Mol and Celluar Biol 21, 4829-4836, David B. Kershaw et al (1997) JBC 272, 15708-15714. Lorenz Sellin et al (2002) FASEB journal 10 Online (review)