Angiogenesis

, Volume 11, Issue 1, pp 79–89

Endoglin in angiogenesis and vascular diseases

  • Peter ten Dijke
  • Marie-José Goumans
  • Evangelia Pardali
Original paper

DOI: 10.1007/s10456-008-9101-9

Cite this article as:
ten Dijke, P., Goumans, MJ. & Pardali, E. Angiogenesis (2008) 11: 79. doi:10.1007/s10456-008-9101-9

Abstract

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.

Keywords

AngiogenesisBMPEndothelial cellsHereditary hemorrhagic telangiectasiaSignal transductionSmadTGF-β

Copyright information

© Springer Science+Business Media B.V. 2008

Authors and Affiliations

  • Peter ten Dijke
    • 1
  • Marie-José Goumans
    • 1
  • Evangelia Pardali
    • 1
  1. 1.Department of Molecular Cell BiologyLeiden University Medical CenterLeidenThe Netherlands