Endoglin/CD105   Protein, Antibody, ELISA Kit, cDNA Clone

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10149-H02H-20
10149-H02H-50
20 µg 
50 µg 
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발현 숙주: Human Cells  
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10149-H08H-20
10149-H08H-50
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50 µg 
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발현 숙주: Human Cells  
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50407-M08H-50
50407-M08H-100
50407-M08H-20
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100 µg 
20 µg 
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Endoglin/CD105  관련 경로

Endoglin/CD105  요약 및 단백질 정보

Endoglin/CD105  배경

유전자 요약: ENG 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 TGFB1 and TGFB3 with high affinity. Mutations in ENG gene cause hereditary hemorrhagic telangiectasia, also known as Osler-Rendu-Weber syndrome 1, an autosomal dominant multisystemic vascular dysplasia. Alternatively spliced transcript variants encoding different isoforms have been found for ENG gene. [provided by RefSeq, Sep 2008]
General information above from NCBI
하부단위 구조: Homodimer that forms a 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. Interacts with GDF2.
세포하 위치: Membrane; Single-pass type I membrane protein.
조직 특이성: Endoglin is restricted to endothelial cells in all tissues except bone marrow.
질병과의 관련성: Hereditary hemorrhagic telangiectasia 1 (HHT1) [MIM:187300]: 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. Note=The disease is caused by mutations affecting the gene represented in this entry.

Endoglin, also known as CD105, is a type I  homodimeric transmembrane glycoprotein with a large, disulfide-linked, extracellular region and a short, constitutively phosphorylated cytoplasmic tail. Endoglin contains an RGD tripeptide which is a key recognition structure in cellular adhesion,,suggesting a critical role for endoglin in the binding of endothelial cells to integrins and/or other RGD receptors. Endoglin is highly expressed on vascular endothelial cells, chondrocytes, and syncytiotrophoblasts of term placenta. It is also found on activated monocytes, mesenchymal stem cells and leukemic cells of lymphoid and myeloid lineages. As an accessory receptor for the TGF-β superfamily ligands, endoglin binds TGF-β1 and TGF-β3 with high affinity not by itself but by associating with TGF-β type I I receptor (TβRII) and activates the downstream signal pathways. In addition, in human umbilical vein endothelial cells, ALK-1 is also a receptor kinase for endoglin threonine phosphorylation, and mutations in either of the two genes result in the autosomal-dominant vascular dysplasia, hereditary hemorrhagic telangiectasia (HHT). Endoglin has been regarded as a powerful biomarker of neovascularization, and is associated with several solid tumor types.

Endoglin/CD105  대체 이름

Endoglin/CD105  관련 연구

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