Defective Angiogenesis in Mice Lacking Endoglin

作者：Dean Y. Li, Lise K. Sorensen, Benjamin S. Brooke, Lisa D. Urness, Elaine C. Davis, Douglas G. Taylor, Beth B. Boak, Daniel P. Wendel · 发表于：Science · 年份：1999 · DOI：10.1126/science.284.5419.1534 · 被引用次数：861 · 研究领域：Vascular Anomalies and Treatments、Pulmonary Hypertension Research and Treatments、Tracheal and airway disorders

Endoglin is a transforming growth factor-beta (TGF-beta) binding protein expressed on the surface of endothelial cells. Loss-of-function mutations in the human endoglin gene ENG cause hereditary hemorrhagic telangiectasia (HHT1), a disease characterized by vascular malformations. Here it is shown that by gestational day 11.5, mice lacking endoglin die from defective vascular development. However, in contrast to mice lacking TGF-beta, vasculogenesis was unaffected. Loss of endoglin caused poor vascular smooth muscle development and arrested endothelial remodeling. These results demonstrate that endoglin is essential for angiogenesis and suggest a pathogenic mechanism for HHT1.