肾小球细胞粘附分子受体抗体Anti-NephrinCNF; NPHN; Nephrosis 1 congenital Finnish type; NPHS 1; NPHS1; Renal glomerulus specific c
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性能参数：

中文名称：肾小球细胞粘附分子受体抗体
英文名称：Anti-Nephrin
别名：CNF; NPHN; Nephrosis 1 congenital Finnish type; NPHS 1; NPHS1; Renal glomerulus specific c
包装号：G320
产地：中国大陆/进口产品
商标/品牌：Santa、Abcam、MBL、BD、Cell Signaling Technology
供应商：田源试剂 TR、新试剂 NR
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产品详细描述

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.Subcellular Location : Cell membrane; Single-pass type I membrane protein （Potential）. Note=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. Expressed in kidney glomeruli. In the embryo,expressed in the mesonephric kidney at E11 with strong expression in cranial tubules with podocyte-like structures. Expression is observed in the podocytes of the developing kidney from E13. High expression is also detected in the developing cerebellum, hindbrain, spinal cord, retina ａnd hypothalamus. Expressed in skeletal muscle during myoblast fusion such as in the adult following acute injury ａnd in the embryo but not detected in uninjured adult skeletal muscle. Isoform 1 ａnd isoform 2 are expressed in the newborn brain ａnd developing cerebellum. Isoform 1 is the predominant isoform in adult kidney.Similarity : Belongs to the immunoglobulin superfamily. Contains 1 fibronectin type-III domain. Contains 8 Ig-like C2-type （immunoglobulin-like） domains.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 （By similarity）. Interacts with NPHS2.

肾小球细胞粘附分子受体抗体的质量标准：

■其它名称：	CNF; NPHN; Nephrosis 1 congenital Finnish type; NPHS 1; NPHS1; Renal glomerulus specific c
■交叉反应：	hu, mo, rat
■产品类型：	一抗
■应用领域:	/信号转导/细胞骨架
■蛋白分子：	predicted molecular weight: 138kDa
■浓度值：	1mg/1ml
■物理性状：	Lyophilized or Liquid
■免疫原：	KLH conjugated synthetic peptide derived from human Nephrin N-terminus
■亚型：	polyclonal
■纯化方法：	affinity purified by Protein A
■保存液：	0.01M PBS, pH 7.4 with 10 mg/ml BSA ａnd 0.1% Sodium azide
■应用技术：	//WB=1:100-500//ELISA=1:500-1000//IP=1:20-100//IHC-P=1:100-500//IHC-F=1:100-500//Flow-Cyt=1:100-500//IF=1:100-500（石蜡切片需做抗原修复）/not yet tested in other applications./optimal dilutions/concentrations should be determined by the end user.
■保存环境：	Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month ａnd for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C.

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肾小球细胞粘附分子受体抗体Anti-Nephrin的相关产品目录：







1mg/1ml 肾小球细胞粘附分子受体抗体 Anti-Nephrin CNF; NPHN; Nephrosis 1 congenital Finnish type; NPHS 1; NPHS1; Renal glomerulus specific c的来源为Rabbit，其相关产品：免疫组化常用试剂免疫印迹常用试剂各种标记二抗

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