- ¥1380 - 2200
- LMAI Bio
- LM-4866R
- 进口/国产
- 2025年12月31日
- WB=1:500-2000 ELISA=1:500-1000 IHC-P=1:400-800 IHC-F=1:400-800 ICC=1:100-500 IF=1:100-500 （石蜡切片需做抗原修复）
- Rabbit
- Human, Mouse, Rat, Dog, Pig, Cow,
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- 详细信息
- 文献和实验
- 技术资料
- 供应商：
上海联迈生物工程有限公司
- 库存：
大量
- 目录编号：
LM-4866R
- 克隆性：
多克隆
- 抗原来源：
Rabbit
- 保质期：
1年
- 抗体英文名：
Nephrin
- 抗体名：
肾小球细胞粘附分子受体抗体
- 宿主：
Rabbit
- 适应物种：
Human, Mouse, Rat, Dog, Pig, Cow,
- 免疫原：
KLH conjugated synthetic peptide derived from human Nephrin:401-500/1241 <Extracellular>
- 亚型：
IgG
- 形态：
Lyophilized or Liquid
- 应用范围：
WB=1:500-2000 ELISA=1:500-1000 IHC-P=1:400-800 IHC-F=1:400-800 ICC=1:100-500 IF=1:100-500 （石蜡切片需做抗原修复）
- 浓度：
1mg/ml
- 保存条件：
Store at -20 °C
- 规格：
100ul 200ul
| 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. |
| 规格价格 | 100ul/1380元 购买 200ul/2200元 购买 大包装/询价 |
| 说 明 书 | 100ul 200ul |
| 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 | Human, Mouse, Rat, Dog, Pig, Cow, |
| 产品应用 | WB=1:500-2000 ELISA=1:500-1000 IHC-P=1:400-800 IHC-F=1:400-800 ICC=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. |
| 分 子 量 | 138kDa |
| 细胞定位 | 细胞浆 细胞膜 |
| 性 状 | Lyophilized or 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 |
| 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. |
| PubMed | PubMed |
| 产品介绍 | background: 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 Entrez Gene: 64563 Rat Omim: 602716 Human SwissProt: O60500 Human SwissProt: Q9QZS7 Mouse SwissProt: Q9R044 Rat Unigene: 122186 Human Unigene: 437830 Mouse Unigene: 48745 Rat
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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文献和实验细胞膜抗原Thy-1和上皮细胞的Heymann抗原等也可引起肾小球原位免疫复合物形成。典型的代表为实验性大鼠的Heymann肾炎。用肾小管刷状缘抗原免疫大鼠后，大鼠体内产生抗肾小管刷状缘抗体，并引起肾小球肾炎。目前已知这种刷状缘抗原即Heymann抗原，是一种分子量为330kD的糖蛋白（gp330），主要位于近曲小管刷状缘和肾小球。肾小球的gp330由脏层上皮细胞合成，合成后集中在上皮细胞足突底部表面与毛细血管基底膜相邻处。抗体与足突底部的gp330抗原结合，在毛细血管表面形成多数小丘状免疫复合物，免疫
。 CR3是由分子量165kD的α链（CD11b）和95kD的β链(CD18)非共价结合的糖蛋白,识别此分子的单克隆抗体有Mac-1和Mo-1等。CR3与CR4（CD11C/CD18）有共同的β链，因此其功能也多有相似之处，白细胞粘附缺陷病（leucocyteadhesion deficiency）病人缺乏这种共同的β链。病人的中性粒细胞虽正常，但不能停留在感染的部位，因此病人易反复遭受感染。这表明CR3和CR4均与吞噬功能密切相关。 四、CR4（gp150/95,CD11c/CD
纤维化的进程。为此，准确地了解、把握LN的活动状态和肾组织已存在的纤维化状况甚为重要。肾脏病理检查对指导LN的治疗具有重要意义。肾脏病理上LN活动性指标（AI）有：（1）肾小球细胞增殖性改变；（2）纤维素性坏死和细胞核溶解；（3）细胞性新月体；（4）白金耳现象和玻璃样血栓；（5）肾小球中性粒细胞浸润；（6）肾间质单核细胞浸润。慢性化指标（CI）包括：（1）肾小球硬化；（2）纤维性新月体；（3）肾小管萎缩；（4）肾间质纤维化。LN的治疗原则为依据LN肾脏病理类型（WHO分类标准）选择不同
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