FITC标记的内皮糖蛋白抗体
产品名称： FITC标记的内皮糖蛋白抗体
英文名称： Anti-CD105/FITC
产品编号： HZ-4609R-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： IF=1:50-200
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Rabbit Anti-CD105/FITC Conjugated antibody
FITC标记的内皮糖蛋白抗体
| 英文名称 | Anti-CD105/FITC |
| 中文名称 | FITC标记的内皮糖蛋白抗体 |
| 别 名 | END; Endoglin; ENG; FLJ41744; HHT1; ORW; ORW1; Osler Rendu Weber syndrome 1; RP11 228B15.2; CD 105; CD105 antigen; EGLN_HUMAN; AI528660; AI662476; S-endoglin; SN6. |
| 规格价格 | 100ul/2980元 购买 大包装/询价 |
| 说 明 书 | 100ul |
| 研究领域 | 肿瘤 心血管 细胞生物 免疫学 信号转导 干细胞 细胞膜受体 细胞表面分子 血管内皮细胞 |
| 抗体来源 | Rabbit |
| 克隆类型 | Polyclonal |
| 交叉反应 | Human, Mouse, Rat, |
| 产品应用 | IF=1:50-200 not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user. |
| 分 子 量 | 70kDa |
| 细胞定位 | 细胞膜 |
| 性 状 | Lyophilized or Liquid |
| 浓 度 | 1mg/ml |
| 免 疫 原 | KLH conjugated synthetic peptide derived from human CD105/Endoglin |
| 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 homodimeric transmembrane protein which is a major glycoprotein of the vascular endothelium. This protein is a component of the transforming growth factor beta receptor complex and it binds to the beta1 and beta3 peptides with high affinity. Mutations in this gene cause hereditary hemorrhagic telangiectasia, also known as Osler-Rendu-Weber syndrome 1, an autosomal dominant multisystemic vascular dysplasia. This gene may also be involved in preeclampsia and several types of cancer. Alternatively spliced transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, May 2013] Function: Major glycoprotein of vascular endothelium. May play a critical role in the binding of endothelial cells to integrins and/or other RGD receptors. Subunit: Homodimer that forms an heteromeric complex with the signaling receptors for transforming growth factor-beta: TGFBR1 and/or TGFBR2. It is able to bind TGF-beta 1, and 3 efficiently and TGF-beta 2 less efficiently. Interacts with TCTEX1D4. Interacts with ARRB2. Subcellular Location: Membrane; Single-pass type I membrane protein. Tissue Specificity: Endoglin is restricted to endothelial cells in all tissues except bone marrow. DISEASE: Defects in ENG are the cause of hereditary hemorrhagic telangiectasia type 1 (HHT1) [MIM:187300]; also known as Osler-Rendu-Weber syndrome 1 (ORW1). HHT1 is an autosomal dominant multisystemic vascular dysplasia, characterized by recurrent epistaxis, muco-cutaneous telangiectases, gastro-intestinal hemorrhage, and pulmonary (PAVM), cerebral (CAVM) and hepatic arteriovenous malformations; all secondary manifestations of the underlying vascular dysplasia. Although the first symptom of HHT1 in children is generally nose bleed, there is an important clinical heterogeneity. Database links: Entrez Gene: 2022 Human Entrez Gene: 13805 Mouse Entrez Gene: 497010 Rat Omim: 131195 Human SwissProt: P17813 Human SwissProt: Q63961 Mouse Unigene: 76753 Human Unigene: 225297 Mouse Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications |
该基因编码同源二聚体跨膜蛋白，其是血管内皮的主要糖蛋白。这种蛋白是转化生长因子-β受体复合物的一个组成部分，它与具有高亲和力的β1和β3肽结合。该基因的突变导致遗传性出血性毛细血管扩张症，也称为Osler Rendu Weber综合征1，常染色体显性多系统血管发育不良。这种基因也可能参与先兆子痫和几种类型的癌症。另一种编码不同的异构体的剪接转录变体已被发现用于该基因。[ RefSeq，2013年5月提供]