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

来源：

SCIENCE | 1999年 / 284卷 / 5419期

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

页码：1534 / 1537

页数：4

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