首页> 美国卫生研究院文献>Journal of the American Heart Association: Cardiovascular and Cerebrovascular Disease >Aortopathy in a Mouse Model of Marfan Syndrome Is Not Mediated by Altered Transforming Growth Factor β Signaling
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Aortopathy in a Mouse Model of Marfan Syndrome Is Not Mediated by Altered Transforming Growth Factor β Signaling

机译:改变转化生长因子β信号不介导Marfan综合征的小鼠模型中的主动脉病变。

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摘要

BackgroundMarfan syndrome (MFS) is caused by mutations in the gene encoding fibrillin‐1 (FBN1); however, the mechanisms through which fibrillin‐1 deficiency causes MFS‐associated aortopathy are uncertain. Recently, attention was focused on the hypothesis that MFS‐associated aortopathy is caused by increased transforming growth factor‐β (TGF‐β) signaling in aortic medial smooth muscle cells (SMC). However, there are many reasons to doubt that TGF‐β signaling drives MFS‐associated aortopathy. We used a mouse model to test whether SMC TGF‐β signaling is perturbed by a fibrillin‐1 variant that causes MFS and whether blockade of SMC TGF‐β signaling prevents MFS‐associated aortopathy.
机译:背景马凡氏综合症(MFS)是由原纤维蛋白1(FBN1)编码基因的突变引起的。然而,纤维蛋白-1缺乏引起MFS相关主动脉病变的机制尚不确定。最近,注意力集中在与MFS相关的主动脉病是由主动脉内侧平滑肌细胞(SMC)中转化生长因子-β(TGF-β)信号增加引起的这一假设上。但是,有许多理由怀疑TGF-β信号驱动MFS相关的主动脉病变。我们使用小鼠模型测试了SMCTGF-β信号传导是否受到引起MFS的原纤维蛋白-1变体的干扰,以及对SMCTGF-β信号传导的阻断是否阻止了MFS相关的主动脉病。

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