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Rabbit Anti-phospho-Nephrin (Tyr1176 + Tyr1193)/BF350 Conjugated antibody (bs-19200R-BF350)
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说 明 书: 100ul  
100ul/2980.00元
大包装/询价
产品编号 bs-19200R-BF350
英文名称1 Rabbit Anti-phospho-Nephrin (Tyr1176 + Tyr1193)/BF350 Conjugated antibody
中文名称 BF350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,  (predicted: 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: 4868 Human

Entrez Gene: 54631 Mouse

Omim: 602716 Human

SwissProt: O60500 Human

SwissProt: Q9QZS7 Mouse

Unigene: 122186 Human

Unigene: 437830 Mouse



Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
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