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货号： bs-19200R-RBITC 基本售价： 2980.0 元 规格： 100ul
产品信息
- 产品编号
- bs-19200R-RBITC
- 英文名称
- Anti-phospho-Nephrin (Tyr1176 + Tyr1193)/RBITC
- 中文名称
- 罗丹明（RBITC）标记的磷酸化肾小球细胞粘附分子受体抗体
- 别 名
- Nephrin (phospho Y1176 + Y1193); p-Nephrin (phospho Y1176 + Y1193); 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.
- 规格价格
- 100ul/2980元购买 大包装/询价
- 说 明 书
- 100ul
- 产品类型
- 磷酸化抗体
- 研究领域
- 细胞生物 信号转导 细胞粘附分子 细胞骨架
- 抗体来源
- Rabbit
- 克隆类型
- Polyclonal
- 交叉反应
- Human, Mouse,
- 产品应用
- ICC=1:50-200 IF=1:50-200
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
- 分 子 量
- 138kDa
- 性 状
- Lyophilized or Liquid
- 浓 度
- 1mg/ml
- 免 疫 原
- KLH conjugated synthesised phosphopeptide derived from human Nephrin around the phosphorylation site of Tyr1176 + Tyr1193
- 亚 型
- IgG
- 纯化方法
- affinity purified by Protein A
- 储 存 液
- 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
- 保存条件
- 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.
- 产品介绍
- background:
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.
Database links:Entrez Gene: 4868Human
Entrez Gene: 54631Mouse
Omim: 602716Human
SwissProt: O60500Human
SwissProt: Q9QZS7Mouse
Unigene: 122186Human
Unigene: 437830Mouse
Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.