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Rabbit Anti-CD105/FITC Conjugated antibody
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
Omim: 131195 Human
SwissProt: P17813 Human
Unigene: 76753 Human
Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
细胞膜受体（Membrane Receptors)
CD105（Endoglin）：CD105是一种存在于细胞表面的同源二聚体跨膜糖蛋白，是TGF-β受体复合物的组成部分，是TGF-β的附属受体，能与多种TGF-β超家族成员结合尤其与TGF-β1、TGF-β3有很高的亲和力，调节TGF-βs与其受体结合而参与信号传导，是内皮细胞增殖相关膜抗原，在培养的高增殖活性内皮细胞和许多恶性肿瘤组织血管内皮细胞中高表达，参与血管生成，但其在血管生成调节中的作用机制尚未阐明。主要用于各种恶性肿瘤组织中的血管生成的研究,
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