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首页> 外文期刊>International Journal of Molecular Sciences >TGF-β Signaling-Related Genes and Thoracic Aortic Aneurysms and Dissections
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TGF-β Signaling-Related Genes and Thoracic Aortic Aneurysms and Dissections

机译：TGF-β信号传导相关基因与胸主动脉瘤和解剖

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Transforming growth factor-β (TGF)-β signaling plays a crucial role in the development and maintenance of various organs, including the vasculature. Accordingly, the mutations in TGF-β signaling pathway-related genes cause heritable disorders of the connective tissue, such as Marfan syndrome (MFS), Loeys-Dietz syndrome (LDS), and Shprintzen-Goldberg syndrome (SGS), and these syndromes may affect skeletal, ocular, pulmonary, and cardiovascular systems. Aortic root aneurysms are common problems that can result in aortic dissection or rupture, which is the leading cause of sudden death in the natural history of MFS and LDS, and recent improvements in surgical treatment have improved life expectancy. However, there is currently no genotype-specific medical treatment. Accumulating evidence suggest that not only structural weakness of connective tissue but also increased TGF-β signaling contributes to the complicated pathogenesis of aortic aneurysm formation, but a comprehensive understanding of governing molecular mechanisms remains lacking. Inhibition of angiotensin II receptor signaling and endothelial dysfunction have gained attention as a possible MFS treatment strategy, but interactions with TGF-β signaling remain elusive. Heterozygous loss-of-function mutations in TGF-β receptors 1 and 2 ( TGFBR1 and TGFBR2 ) cause LDS, but TGF-β signaling is activated in the aorta (referred to as the TGF-β paradox) by mechanisms yet to be elucidated. In this review, we present and discuss the current understanding of molecular mechanisms responsible for aortopathies of MFS and related disorders.
机译：转化生长因子-β（TGF）-β信号在各种器官（包括脉管系统）的发育和维持中起着至关重要的作用。因此，TGF-β信号通路相关基因的突变会引起结缔组织的遗传性疾病，例如马凡氏综合症（MFS），洛伊斯-狄兹氏综合症（LDS）和Shprintzen-Goldberg氏综合症（SGS），这些综合症可能影响骨骼，眼，肺和心血管系统。主动脉根部动脉瘤是常见问题，可能导致主动脉夹层破裂或破裂，这是MFS和LDS自然病史中猝死的主要原因，并且最近在外科治疗方面的改进提高了预期寿命。但是，目前尚无基因型特异性药物治疗。越来越多的证据表明，不仅结缔组织的结构薄弱，而且TGF-β信号转导的增加都导致主动脉瘤形成的复杂发病机理，但仍缺乏对控制分子机制的全面了解。作为一种可能的MFS治疗策略，抑制血管紧张素II受体信号传导和内皮功能障碍已引起关注，但与TGF-β信号传导的相互作用仍然难以捉摸。 TGF-β受体1和2（TGFBR1和TGFBR2）的杂合功能丧失突变引起LDS，但主动脉中的TGF-β信号传导被激活（称为TGF-β悖论），其机制尚待阐明。在这篇综述中，我们介绍并讨论了导致MFS和相关疾病主动脉病变的分子机制的最新认识。

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