Original paper


, Volume 11, Issue 1, pp 79-89

First online:

Endoglin in angiogenesis and vascular diseases

  • Peter ten DijkeAffiliated withDepartment of Molecular Cell Biology, Leiden University Medical Center Email author 
  • , Marie-José GoumansAffiliated withDepartment of Molecular Cell Biology, Leiden University Medical Center
  • , Evangelia PardaliAffiliated withDepartment of Molecular Cell Biology, Leiden University Medical Center

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Endoglin is a transmembrane auxillary receptor for transforming growth factor-β (TGF-β) that is predominantly expressed on proliferating endothelial cells. Endoglin deficient mice die during midgestation due to cardiovascular defects. Mutations in endoglin and activin receptor-like kinase 1 (ALK1), an endothelial specific TGF-β type I receptor, have been linked to hereditary hemorrhagic telangiectasia (HHT), an autosomal dominant vascular dysplasia characterized by telangiectases and arteriovenous malformations. Endoglin heterozygote mice develop HHT-like vascular abnormalities, have impaired tumor and post-ischemic angiogenesis and demonstrate an endothelial nitric oxide synthase-dependent deterioration in the regulation of vascular tone. In pre-eclampsia, placenta-derived endoglin has been shown to be strongly upregulated and high levels of soluble endoglin are released into the circulation. Soluble endoglin was found to cooperate with a soluble form of vascular endothelial growth factor receptor 1 in the pathogenesis of pre-eclampsia by inducing endothelial cell dysfunction. Endoglin is highly expressed in tumor-associated endothelium, and endoglin antibodies have been successfully used to target activated endothelial cells and elicit anti-angiogenic effects in tumor mouse models. These exciting advances provide opportunities for the development of new therapies for diseases with vascular abnormalities.


Angiogenesis BMP Endothelial cells Hereditary hemorrhagic telangiectasia Signal transduction Smad TGF-β