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Product Name Nephrin Chinese Name 肾小球Cell adhesion molecule受体抗体 Alias 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. literatures Specific References (4) | SL4866R has been referenced in 4 publications.[IF=6.107] Nan Zang. et al. cGAS-STING Activation by Cytosolic Mitochondrial DNA Contributes to Podocyte Injury in Diabetic Kidney Disease. ISCIENCE. 2022 Sep;:105145 WB ; Mouse.[IF=3.647] Lin D et al. Aldehyde dehydrogenase 2 regulates autophagy via the Akt-mTOR pathway to mitigate renal ischemia-reperfusion injury in hypothermic machine perfusion. Life Sci . 2020 Jul 15;253:117705. IHC,WB ; mouse.[IF=3.414] Cao S et al. Effect of shenyan xiaobai granule on nephrin and podocin of adriamycin-induced renal injury: A randomised controlled trial. J Ethnopharmacol. 2019 Nov 15;244:112104. IHC-P ; Rat.[IF=3.375] Sheung-Fat Koet al. Protective effect of combined therapy with hyperbaric oxygen and autologous adipose-derived mesenchymal stem cells on renal function in rodent after acute ischemia-reperfusion injury. Am J Transl Res . 2020 Jul 15;12(7):3272-3287. IHC, IF ; rat.Research Area Signal transduction Cell adhesion molecule Cytoskeleton Extracellular matrix Immunogen Species Rabbit Clonality Polyclonal React Species Mouse, (predicted: Human, Rat, Dog, Pig, Cow, ) Applications WB=1:500-2000 ELISA=1:5000-10000 IHC-P=1:100-500 IHC-F=1:100-500 ICC=1:100-500 IF=1:100-500 (Paraffin sections need antigen repair)
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.Theoretical molecular weight 138kDa Cellular localization cytoplasmic The cell membrane Form Liquid Concentration 1mg/ml immunogen KLH conjugated synthetic peptide derived from human Nephrin: 401-500/1241 <Extracellular> 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 Primary steroid resistant nephrotic syndrome (SRNS) is characterized by childhood onset of proteinuria and progression to end stage renal disease. Kidney podocytes and their slit diaphragms form the final barrier to urinary protein loss. Congenital nephrotic syndrome (CNS) is caused by mutations in NPHS1 (nephrin) or NPHS2. Nephrin, a recently identified protein is a member of a group of podocyte proteins that constitute major component of the slit diaphragm especially in the foot process. Nephrin, a cell adhesion molecule, may play a crucial role in maintaining the glomerular filtration barrier. Recent studies have suggested that mutations in the gene for Nephrin reportedly lead to congenital nephrosis. Three novel podocyte proteins, Podocin, Nephrin and alpha Actinin 4 have been identified in congenital and experimental models of proteinuria. The role of Nephrin in anti apoptotic activity in podocyte slit diaphragm is believed to be associated with vascular endothelial derived growth factors VEGF signaling.
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.
Subunit:
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.
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 at Tyr-1193 by FYN, leading to the recruitment and activation of phospholipase C-gamma-1/PLCG1.
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
Unigene: 48745 Rat
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