• 靶点：
• CD105(Endoglin)

• 基因名称：
• ENG END

• 蛋白名称：
• Endoglin (CD antigen CD105)

• Human Gene Id：
• 2022

• Human Swiss Prot No：
• P17813

• 特异性：
• endogenous

• 组成：
• PBS, 50% glycerol, 0.05% Proclin 300, 0.05%BSA

• 来源：
• Monoclonal, rabbit, IgG, Kappa

• 稀释：
• IHC 1:100-200,WB 1:1000-5000,IF 1:200-1000,ELISA 1:5000-20000,IP 1:50-200

• 纯化工艺：
• Protein A

• 储存：
• -15°C to -25°C/1 year(Do not lower than -25°C)

• 分子量：
• 70kD

• 实测条带：
• 95kD

• 背景：
• 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],

• 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

• 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).

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