产品编号 | bs-4866R |
英文名称 | Nephrin |
中文名称 | 肾小球细胞粘附分子受体抗体 |
别 名 | 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. |
Specific References (4) | bs-4866R 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.
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研究领域 | 信号转导 细胞粘附分子 细胞骨架 细胞外基质 |
抗体来源 | Rabbit |
克隆类型 | Polyclonal |
交叉反应 | Mouse (predicted: Human,Rat,Dog,Pig,Cow) |
产品应用 | WB=1:500-2000, IHC-P=1:100-500, IHC-F=1:100-500, ICC=1:100-500, IF=1:100-500, ELISA=1:5000-10000
not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user. |
理论分子量 | 138kDa |
细胞定位 | 细胞浆 细胞膜 |
性 状 | Liquid |
浓 度 | 1mg/ml |
免 疫 原 | KLH conjugated synthetic peptide derived from human Nephrin: 401-500/1241 <Extracellular> |
亚 型 | IgG |
纯化方法 | affinity purified by Protein A |
缓 冲 液 | 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol. |
保存条件 | Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. |
注意事项 | This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. |
PubMed | PubMed |
产品介绍 |
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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1、抗体溶解方法 | |
2、抗体修复方式 | |
3、常用试剂的配制 | |
4、免疫组化操作步骤 | |
5、免疫组化问题解答 | |
6、Western Blotting 操作步骤 | |
7、Western Blotting 问题解答 | |
8、关于肽链的设计 | |
9、多肽的溶解与保存 | |
10、酶标抗体效价测定程序 | |