Anti-Nephrin/NPHS1 Antibody Picoband® Fluoro594 Conjugated

NPHS1, also called Nephrin, is mapped to 19q13.12. This gene encodes a member of the immunoglobulin family of cell adhesion molecules that functions in the glomerular filtration barrier in the kidney. It gene is primarily expressed in renal tissues, and the protein is a type-1 transmembrane protein found at the slit diaphragm of glomerular podocytes. The slit diaphragm is thought to function as an ultrafilter to exclude albumin and other plasma macromolecules in the formation of urine. A defect in the gene for nephrin, NPHS1, is associated with congenital nephrotic syndrome of the Finnish type and causes massive amounts of protein to be leaked into the urine, orproteinuria. NPHS1 is also required for cardiovascular development.

E.coli-derived human Nephrin recombinant protein (Position: V1084-V1241) . Human Nephrin shares 79.9% and 75.3% amino acid (aa) sequence identity with mouse and rat Nephrin, respectively.

Seems to play a role in the development or function of the kidney glomerular filtration barrier. Regulates glomerular vascular permeability. May anchor the podocyte slit diaphragm to the actin cytoskeleton. Plays a role in skeletal muscle formation through regulation of myoblast fusion (By similarity) . .

1. Wagner, N; Morrison, H; Pagnotta, S; Michiels, JF; Schwab, Y; Tryggvason, K; Schedl, A; Wagner, KD (2011-03-29) . The podocyte protein nephrin is required for cardiac vessel formation.. Human Molecular Genetics 20 (11) : 2182–94. 2. Wartiovaara, J., Ofverstedt, L.-G., Khoshnoodi, J., Zhang, J., Makela, E., Sandin, S., Ruotsalainen, V., Cheng, R. H., Jalanko, H., Skoglund, U., Tryggvason, K. Nephrin strands contribute to a porous slit diaphragm scaffold as revealed by electron tomography. J. Clin. Invest. 114: 1475-1483, 2004.

Cell membrane ; Single-pass type I membrane protein . Predominantly located at podocyte slit diaphragm between podocyte foot processes. Also associated with podocyte apical plasma membrane. .