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, it belongs to the functionally important complex of proteins of glomerular filtration barrier. Filtrin (Human 219aa, 107kD, chrm 19q13.12, gene NLG1) belongs to the functionally important glomerular filtration barrier proteins, Filtrin is a nehrin-like protein expressed in glomeruli, It has structural similarity and partial consistent expression with nephrin, thus shows 30% and 44% homology with nephrin and NEPH1 respectively. Source: 20aa peptide from human Filtrin. Location: ~N-terminus. Species Sequence Homology: Mouse-90%. No significant sequence homology is seen with other related family members. Applications: ELISA: Can be used to coat ELISA plates at 1ug/ml and detected with antibodies (See F4511-45A). Storage and Stability: Lyophilized powder may be stored at -20C. Stable for 12 months at -20C. Reconstitute with sterile buffer or ddH2O. Aliquot to avoid repeated freezing and thawing. Store at -20C. Reconstituted product is stable for 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.
Reinheit:
Purified
Formulierung:
Supplied as a lyophilized powder from PBS, 0.05% sodium azide.
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