FITC标记的磷酸化肾小球细胞粘附分子受体抗体
产品名称： FITC标记的磷酸化肾小球细胞粘附分子受体抗体
英文名称： Anti-phospho-Nephrin (Tyr1176 + Tyr1193)/FITC
产品编号： HZ-19200R-FITC
产品价格： null
产品产地： 中国/上海
品牌商标： HZbscience
更新时间： 2023-08-17T10:24:20
使用范围： ICC=1:50-200 IF=1:50-20
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Rabbit Anti-phospho-Nephrin (Tyr1176 + Tyr1193)/FITC Conjugated antibody
FITC标记的磷酸化肾小球细胞粘附分子受体抗体
| 英文名称 | Anti-phospho-Nephrin (Tyr1176 + Tyr1193)/FITC |
| 中文名称 | FITC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: 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 |
该基因编码在肾小球滤过屏障中起作用的细胞粘附分子免疫球蛋白家族的成员。该基因主要表达于肾组织，该蛋白是在肾小球足细胞的裂隙膈膜发现的1型跨膜蛋白。狭缝膈膜被认为是一种超滤器，在尿液形成过程中排除白蛋白和其他血浆大分子。该基因的突变导致芬兰型先天性肾病1，其特点是严重的蛋白尿和裂隙膈肌和足突的丢失。