内皮糖蛋白抗体
规格：1mg/1ml
英文名： CD105
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.
分子量： 70kDa
储存液：0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glyce
克隆类型：Polyclonal
亚型：IgG
纯化方法：affinity purified by Protein A
**原：KLH conjugated synthetic peptide derived from human CD105
交叉反应：Human, Mouse, Rat,
细胞定位：细胞膜
内皮糖蛋白抗体产品介绍：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: 内皮糖蛋白抗体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, 内皮糖蛋白抗体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. Gene ID: 2022 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.
内皮糖蛋白抗体产品应用：WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 Flow-Cyt=1:20-500 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.
研究领域：肿瘤 心血管 细胞生物 **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
储存条件： Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
来源： Rabbit
外观： Lyophilized or Liquid