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|本期目录/Table of Contents|

马凡氏综合征血管病变的基础研究进展

《心脏杂志》[ISSN:1009-7236/CN:61-1268/R]

期数:
2012年第6期
页码:
760-762
栏目:
综述
出版日期:
2012-12-25

文章信息/Info

Title:
Progress in basic research on vascular pathological changes of Marfan syndrome
作者:
公 兵 综述; 杨秀滨 审校
(北京协和医学院、中国医学科学院、阜外心血管病医院心脏外科，北京 100037)
Author(s):
GONG Bing;  YANG Xiu-bin
(Department of Surgery, Peking Union Medical College and Chinese Academy of Medical Sciences, Cardiovascular Institute and Fuwai Hospital, Beijing 100037, China)
关键词:
马凡氏综合征; 转化生长因子β; 非经典TGF-β信号通路; miR-29b
Keywords:
MFS;  TGF-β;  noncanonical TGF-β signaling;  miR-29b
分类号:
R54
DOI:
-
文献标识码:
A
摘要:
马凡氏综合征（marfan syndrome，MFS）是一种常染色体显性遗传性结缔组织病，主要累及心血管系统、眼及骨骼系统。心血管的病变主要为主动脉瘤与主动脉夹层，而其破裂是引起患者死亡的主要原因。新近研究发现，引起MFS血管病变发生发展的原因与转化生长因子β（TGF-β）的过度激活及其非经典信号转导通路有着很大的关系，同时研究结果显示，miR-29b在早期MFS主动脉病变的进展中起着关键作用。
Abstract:
Marfan syndrome (MFS) is an autosomal dominant connective tissue disorder with multisystemic manifestations, which typically involve the cardiovascular, ocular and skeletal systems. Aortic aneurysm rupture and aortic dissection are the main causes of mortality in patients with MFS. Recent studies reveal that upregulated transforming growth factor β (TGF-β) and its noncanonical signaling are related to the development of vascular diseases and that miR-29b plays a key role in early development of aortic diseases in MFS.

参考文献/References

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[7]Holm TM,Habashi JP,Doyle JJ,et al.Noncanonical tgfbeta signaling contributes to aortic aneurysm progression in marfan syndrome mice[J].Science,2011,332(6027):358-361.
[8]Habashi JP,Doyle JJ,Holm TM,et al.Angiotensin ii type 2 receptor signaling attenuates aortic aneurysm in mice through erk antagonism[J].Science,2011, 332(6027):361-365.
[9]Lee MK, Pardoux C,Hall MC,et al.Tgf-beta activates erk map kinase signalling through direct phosphorylation of shca[J].EMBO J,2007,26(17):3957-3967.
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备注/Memo

备注/Memo:
收稿日期：2012-03-22.通讯作者：杨秀滨，教授，主要从事心血管外科相关疾病研究 Email:xiubinyang@yahoo.com 作者简介：公兵，硕士生 Email:gongbing217124@126.com
更新日期/Last Update: 2012-12-30