Western Blot (WB)
(Western blot analysis of Nephrin expression in rat kidney extract (lane 1), mouse kidney extract (lane 2) and 293T whole cell lysates (lane 3). Nephrin at 134KD was detected using rabbit anti- Nephrin Antigen Affinity purified polyclonal antibody at 0.5ug/mL. The blot was developed using chemiluminescence (ECL) method. )
Rabbit Nephrin Polyclonal Antibody | anti-NPHS1 antibody
Anti-Nephrin Antibody
Each vial contains 5mg BSA, 0.9mg NaCl, 0.2mg Na2HPO4, 0.05mg NaN3.
Other applications have not been tested.
Western Blot (WB)
(Western blot analysis of Nephrin expression in rat kidney extract (lane 1), mouse kidney extract (lane 2) and 293T whole cell lysates (lane 3). Nephrin at 134KD was detected using rabbit anti- Nephrin Antigen Affinity purified polyclonal antibody at 0.5ug/mL. The blot was developed using chemiluminescence (ECL) method. )
Western Blot (WB)
(Western blot analysis of Nephrin expression in rat kidney extract (lane 1), mouse kidney extract (lane 2) and 293T whole cell lysates (lane 3). Nephrin at 134KD was detected using rabbit anti- Nephrin Antigen Affinity purified polyclonal antibody at 0.5ug/mL. The blot was developed using chemiluminescence (ECL) method. )
Background: 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.
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.
NCBI and Uniprot Product Information
NCBI Description
This gene encodes a member of the immunoglobulin family of cell adhesion molecules that functions in the glomerular filtration barrier in the kidney. The 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. Mutations in this gene result in Finnish-type congenital nephrosis 1, characterized by severe proteinuria and loss of the slit diaphragm and foot processes.[provided by RefSeq, Oct 2009]
Uniprot Description
NPHS1: 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. Interacts with CD2AP (via C-terminal domain). Interacts with MAGI1 (via PDZ 2 and 3 domains) forming a tripartite complex with IGSF5/JAM4. Interacts with DDN; the interaction is direct. Self-associates (via the Ig-like domains). Also interacts (via the Ig-like domains) with KIRREL/NEPH1 and KIRREL2; the interaction with KIRREL is dependent on KIRREL glycosylation. Forms a complex with ACTN4, CASK, IQGAP1, MAGI2, SPTAN1 and SPTBN1. Interacts with NPHS2. Specifically expressed in podocytes of kidney glomeruli. Belongs to the immunoglobulin superfamily. 2 isoforms of the human protein are produced by alternative splicing.
Protein type: Cell adhesion; Immunoglobulin superfamily; Membrane protein, integral
Chromosomal Location of Human Ortholog: 19q13.12
Cellular Component: integral to plasma membrane; plasma membrane
Molecular Function: myosin binding; protein binding
Biological Process: excretion; glomerular basement membrane development
Disease: Nephrotic Syndrome, Type 1
Research Articles on NPHS1
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Product Notes
The NPHS1 nphs1 (Catalog #AAA178658) is an Antibody produced from Rabbit and is intended for research purposes only. The product is available for immediate purchase. The Anti-Nephrin Antibody reacts with Human, Mouse, Rat and may cross-react with other species as described in the data sheet. AAA Biotech's Nephrin can be used in a range of immunoassay formats including, but not limited to, Western Blot (WB). Western Blot: 0.1-0.5ug/ml. Researchers should empirically determine the suitability of the NPHS1 nphs1 for an application not listed in the data sheet. Researchers commonly develop new applications and it is an integral, important part of the investigative research process. It is sometimes possible for the material contained within the vial of "Nephrin, Polyclonal Antibody" to become dispersed throughout the inside of the vial, particularly around the seal of said vial, during shipment and storage. We always suggest centrifuging these vials to consolidate all of the liquid away from the lid and to the bottom of the vial prior to opening. Please be advised that certain products may require dry ice for shipping and that, if this is the case, an additional dry ice fee may also be required.Precautions
All products in the AAA Biotech catalog are strictly for research-use only, and are absolutely not suitable for use in any sort of medical, therapeutic, prophylactic, in-vivo, or diagnostic capacity. By purchasing a product from AAA Biotech, you are explicitly certifying that said products will be properly tested and used in line with industry standard. AAA Biotech and its authorized distribution partners reserve the right to refuse to fulfill any order if we have any indication that a purchaser may be intending to use a product outside of our accepted criteria.Disclaimer
Though we do strive to guarantee the information represented in this datasheet, AAA Biotech cannot be held responsible for any oversights or imprecisions. AAA Biotech reserves the right to adjust any aspect of this datasheet at any time and without notice. It is the responsibility of the customer to inform AAA Biotech of any product performance issues observed or experienced within 30 days of receipt of said product. To see additional details on this or any of our other policies, please see our Terms & Conditions page.Item has been added to Shopping Cart
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