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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 development and arrested endothelial remodeling. These results demonstrate that endoglin is essential for angiogenesis and suggest a pathogenic mechanism for HHT1., HHT is an autosomal dominant vascular dysplasia characterized by recurrent epistaxis, telangiectasia, gastrointestinal hemorrhage, and pulmonary, cerebral, and hepatic arteriovenous malformations (1). ENG, the gene responsible for HHT1, encodes an [...]