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Product Name phospho-Nephrin (Tyr1217) Chinese Name 磷酸化肾小球Cell adhesion molecule受体抗体 Alias Nephrin (phospho Y1217): p-Nephrin (phospho Y1217); CNF; Nephrin; Nephrosis 1 congenital Finnish type; Nephrosis 1, congenital, Finnish type (nephrin); NPHN; NPHN_HUMAN; NPHS 1; Nphs1; Renal glomerulus specific cell adhesion receptor; Renal glomerulus-specific cell adhesion receptor. Product Type Phosphorylated anti Research Area Cell biology Signal transduction Cell adhesion molecule Cytoskeleton Immunogen Species Rabbit Clonality Polyclonal React Species Human, (predicted: Mouse, Rat, ) Applications WB=1:500-2000 ELISA=1:5000-10000
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.Theoretical molecular weight 136kDa Cellular localization The cell membrane Form Liquid Concentration 1mg/ml immunogen KLH conjugated synthesised phosphopeptide derived from human Nephrin around the phosphorylation site of Tyr1217: GI(p-Y)DQ Lsotype IgG Purification affinity purified by Protein A Buffer Solution 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol. Storage Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. Attention This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. PubMed PubMed Product Detail 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]
Function:
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.
Subcellular Location:
Cell membrane. Predominantly located at podocyte slit diaphragm between podocyte foot processes. Also associated with podocyte apical plasma membrane.
Tissue Specificity:
Specifically expressed in podocytes of kidney glomeruli.
Post-translational modifications:
Phosphorylated on tyrosine residues.
DISEASE:
Defects in NPHS1 are the cause of nephrotic syndrome type 1 (NPHS1) [MIM:256300]; also known as Finnish congenital nephrosis (CNF). A renal disease characterized clinically by proteinuria, hypoalbuminemia, hyperlipidemia, and edema. Kidney biopsies show non-specific histologic changes such as focal segmental glomerulosclerosis and diffuse mesangial proliferation. Some affected individuals have an inherited steroid-resistant form and progress to end-stage renal failure.
Similarity:
Belongs to the immunoglobulin superfamily.
Contains 1 fibronectin type-III domain.
Contains 8 Ig-like C2-type (immunoglobulin-like) domains.
SWISS:
O60500
Gene ID:
4868
Database links:Entrez Gene: 4868 Human
Entrez Gene: 54631 Mouse
Omim: 602716 Human
SwissProt: O60500 Human
SwissProt: Q9QZS7 Mouse
Unigene: 122186 Human
Unigene: 437830 Mouse
Product Picture Tissue/cell: human kidney tissue; 4% Paraformaldehyde-fixed and paraffin-embedded;
Antigen retrieval: citrate buffer ( 0.01M, pH 6.0 ), Boiling bathing for 15min; Block endogenous peroxidase by 3% Hydrogen peroxide for 30min; Blocking buffer (normal goat serum,C-0005) at 37℃ for 20 min;
Incubation: Anti-phospho-Nephrin (Tyr1217) Polyclonal Antibody, Unconjugated(SL19199R) 1:200, overnight at 4°C, followed by conjugation to the secondary antibody(SP-0023) and DAB(C-0010) staining
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