Defective angiogenesis in mice lacking endoglin

被引:681
作者
Li, DY [1 ]
Sorensen, LK
Brooke, BS
Urness, LD
Davis, EC
Taylor, DG
Boak, BB
Wendel, DP
机构
[1] Univ Utah, Program Human Mol Biol & Genet, Salt Lake City, UT 84112 USA
[2] Univ Utah, Dept Human Genet, Salt Lake City, UT 84112 USA
[3] Univ Utah, Dept Oncol Sci, Salt Lake City, UT 84112 USA
[4] Univ Utah, Dept Med, Salt Lake City, UT 84112 USA
[5] Univ Utah, Howard Hughes Med Inst, Salt Lake City, UT 84112 USA
[6] Univ Texas, SW Med Ctr, Dept Cell Biol & Physiol, Dallas, TX 75235 USA
关键词
D O I
10.1126/science.284.5419.1534
中图分类号
O [数理科学和化学]; P [天文学、地球科学]; Q [生物科学]; N [自然科学总论];
学科分类号
07 ; 0710 ; 09 ;
摘要
Endoglin is a transforming growth factor-beta (TCF-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.
引用
收藏
页码:1534 / 1537
页数:4
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