Defective angiogenesis in mice lacking endoglin

DY Li, LK Sorensen, BS Brooke, LD Urness, EC Davis… - Science, 1999 - science.org
DY Li, LK Sorensen, BS Brooke, LD Urness, EC Davis, DG Taylor, BB Boak, DP Wendel
Science, 1999science.org
Endoglin is a transforming growth factor–β (TGF-β) 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-β,
vasculogenesis was unaffected. Loss of endoglin caused poor vascular smooth muscle …
Endoglin is a transforming growth factor–β (TGF-β) binding protein expressed on the surface of endothelial cells. Loss-of-function mutations in the human endoglin gene ENGcause 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-β, 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.
AAAS