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AF647標記的CD105抗體

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英文名稱 Anti-CD105/AF647
中文名稱 AF647標記的CD105抗體
別    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.  
規格價格 100ul      
說 明 書 100ul  
研究領域 腫瘤  心血管  細胞生物  免疫學  信號轉導  干細胞  生長因子和激素  細胞膜受體  細胞表面分子  內皮細胞  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應 Human, Rat,  (predicted: Mouse, Chicken, Dog, Pig, Cow, Horse, )
產品應用 IF=1:50-200 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 70kDa
性    狀 Lyophilized or Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human CD105
亞    型 IgG
純化方法 affinity purified by Protein A
儲 存 液 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
保存條件 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.
產品介紹 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.




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

細胞膜受體(Membrane Receptors)
CD105(Endoglin):CD105是一種存在于細胞表面的同源二聚體跨膜糖蛋白,是TGF-β受體復合物的組成部分,是TGF-β的附屬受體,能與多種TGF-β超家族成員結合尤其與TGF-β1、TGF-β3有很高的親和力,調節TGF-βs與其受體結合而參與信號傳導,是內皮細胞增殖相關膜抗原,在培養的高增殖活性內皮細胞和許多惡性腫瘤組織血管內皮細胞中高表達,參與血管生成,但其在血管生成調節中的作用機制尚未闡明。主要用于各種惡性腫瘤組織中的血管生成的研究,
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