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10:46
Isolation and Culture of Pulmonary Endothelial Cells from Neonatal Mice
Published on: December 14, 2010
缺陷血管新生在缺乏内分泌素的小鼠
D Y Li1, L K Sorensen, B S Brooke
1Program in Human Molecular Biology and Genetics, Department of Human Genetics, Howard Hughes Medical Institute, University of Utah, Salt Lake City, UT 84112-5330, USA. dean.li@hci.utah.edu
Science (New York, N.Y.)
|May 29, 1999
概括
内分泌素对血管发育至关重要. 缺乏内分泌蛋白的小鼠表现出缺陷的血管光滑肌肉和重塑,导致胚胎致死性,并表明遗传性出血端膜病变 (HHT1) 的原因.
科学领域:
- 血管生物学 血管生物学
- 发展生物学 发展生物学
- 遗传学 是一个遗传学.
背景情况:
- 内分泌蛋白 (ENG) 是TGF-β在内皮细胞上的结合蛋白.
- 在 ENG 中的突变会导致遗传性出血端膜病 (HHT1),一种血管疾病.
研究的目的:
- 通过使用小鼠模型,研究内皮质在血管发育中的作用.
- 阐明HHT1.1背后的致病机制.
主要方法:
- 产生和分析内素缺乏的小鼠.
- 在妊娠日检查血管发育11.5.5.
主要成果:
- 由于血管发育缺陷,内分泌素缺乏的小鼠在11.5个妊娠日死亡.
- 血管生成没有受到影响,但血管光滑肌肉发育和内皮重塑受损.
- 与TGF-β淘汰赛小鼠不同,内分泌素缺乏并没有影响血管生成.
结论:
- 内分泌素对于适当的血管生成和血管改造至关重要.
- 内分泌蛋白功能缺陷导致了HHT1.1中所见的血管形.
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