TGF-β and BMPR2 Signaling in PAH: Two Black Sheep in One Family.

TGF-β and BMPR2 Signaling in PAH: Two Black Sheep in One Family.
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DOI:
10.3390/ijms19092585
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发表时间:
2018-08-31
影响因子:
5.6
通讯作者:
Goumans MJ
Goumans MJ
中科院分区:
生物学2区
文献类型:
--
作者:
Rol N;Kurakula KB;Happé C;Bogaard HJ;Goumans MJ

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关于转化生长因子β(TGF-β)和骨形态发生蛋白(BMP)信号转导参与肺动脉高压(PAH)的知识不断增加。人们对参与该途径的单个组分的功能有了越来越多的了解,但目前缺乏对这些组分在PAH中如何相互作用的明确综合。大多数关注点都集中在BMPR 2下游的信号传导上,但必须包括TGF-β信号传导在PAH中的作用。本综述概述了在PAH中观察到的通过TGF-β家族受体干扰信号传导与血管重塑和心脏效应的最新技术水平。针对这两种途径的近期(前)临床研究将以PAH以外的心血管研究领域的扩展观点进行讨论,表明新的未来前景。
Knowledge pertaining to the involvement of transforming growth factor β (TGF-β) and bone morphogenetic protein (BMP) signaling in pulmonary arterial hypertension (PAH) is continuously increasing. There is a growing understanding of the function of individual components involved in the pathway, but a clear synthesis of how these interact in PAH is currently lacking. Most of the focus has been on signaling downstream of BMPR2, but it is imperative to include the role of TGF-β signaling in PAH. This review gives a state of the art overview of disturbed signaling through the receptors of the TGF-β family with respect to vascular remodeling and cardiac effects as observed in PAH. Recent (pre)-clinical studies in which these two pathways were targeted will be discussed with an extended view on cardiovascular research fields outside of PAH, indicating novel future perspectives.
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