| 别名 | Endoglin (CD antigen CD105) |
| 基因名称 | Endogenous |
| 蛋白名称 | ENG END |
| 推荐应用 | |
| 反应物种 | CD105(Endoglin) |
| 存储缓冲液 | IHC 1:100-200;WB 1:1000-5000;IF 1:200-1000;ELISA 1:5000-20000;IP 1:50-200 |
| Human Swissprot No. | 2022 |
| Mouse Gene ID | P17813 |
| 特异性 | Monoclonal |
| 稀释度 | Unmodified |
| 参考分子量 | 70kD |
| 预测分子量 | WB,IHC,IF,IP,ELISA |
| 运输及保存条件 | Protein A |
| 宿主 | 95kD |
| 同种型 | Rabbit |
| 背景介绍 | 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], |
| 细胞定位 | Cytoplasm |
| 功能 | Disease:Defects in ENG are the cause of hereditary hemorrhagic telangiectasia type 1 (HHT1) [MIM:187300, 108010]; 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.,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: TGF-beta receptors I and/or II. It is able to bind TGF-beta 1, and 3 efficiently and TGF-beta 2 less efficiently. Interacts with TCTEX1D4.,tissue specificity:Endoglin is restricted to endothelial cells in all tissues except bone marrow., |
| 纯化 | PBS, 50% glycerol, 0.05% Proclin 300, 0.05%BSA |
| Clonality | -15°C to -25°C/1 year(Do not lower than -25°C) |
| 修饰类型 | IgG,Kappa |
| 细胞定位 | Cytoplasm |
| 组织表达 | Detected on umbilical veil endothelial cells (PubMed:10625079). Detected in placenta (at protein level) (PubMed:1692830). Detected on endothelial cells (PubMed:1692830). |
周一至周日 8:00-18:00
