| 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. NEPH1 (Human 605aa, 67kD protein, Mouse 789aa, the sequence is 42.96%conserved between human and mouse). NEPH1 was identified in mice by a retrovirus mediated mutagenesis screening, contains 5 Ig like domains and is structurally related to nephrin. The protein has a putative signal sequence at N-terminus and a transmembrane region. Inactivation of NEPH1 develops a nephrotic syndrome that resembles NPHS mutations, suggesting that all three proteins are essential for the integrity of glomerular podocytes. NEPH1 is expressed in kidney, brain, and smooth muscle of liver, lung and heart. Source: 16aa peptide from rat NEPH1 Location: ~C-terminus, Cytoplasmic domain Species Sequence Homology: Human, mouse-100% Applications: Suitable for use in ELISA as a control peptide for N2028-62. Other applications not tested. Recommended Dilution: ELISA: Coat ELISA plates at 1ug/ml 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 6 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. |
