First Author | Li DY | Year | 1999 |
Journal | Science | Volume | 284 |
Issue | 5419 | Pages | 1534-7 |
PubMed ID | 10348742 | Mgi Jnum | J:55403 |
Mgi Id | MGI:1337912 | Doi | 10.1126/science.284.5419.1534 |
Citation | Li DY, et al. (1999) Defective angiogenesis in mice lacking endoglin. Science 284(5419):1534-7 |
abstractText | 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. |