Role of the Epicardium in the Development of the Atrioventricular Valves and Its Relevance to the Pathogenesis of Myxomatous Valve Disease.

Role of the Epicardium in the Development of the Atrioventricular Valves and Its Relevance to the Pathogenesis of Myxomatous Valve Disease.
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DOI:
10.3390/jcdd8050054
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发表时间:
2021-05-12
影响因子:
2.4
通讯作者:
Wessels A
Wessels A
中科院分区:
医学3区
文献类型:
--
作者:
Wolters R;Deepe R;Drummond J;Harvey AB;Hiriart E;Lockhart MM;van den Hoff MJB;Norris RA;Wessels A

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本文旨在纪念Adriana“Adri”Gittenberger-de Groot博士,并感谢她在发育心血管生物学领域的工作以及她留下的遗产。在她令人印象深刻的职业生涯中,Gittenberger-de Groot博士研究了心脏发育的许多方面,包括心脏瓣膜形成和疾病以及心外膜在心脏形成中的作用。在这篇文章中,我们回顾了一些工作的作用,心外膜衍生细胞(EPDCs)的房室瓣的发展和他们的潜在参与粘液瘤性瓣膜病(MVD)的发病机制。我们提供了一个概述的发展中的房室交界的关键事件,讨论心外膜在这些事件中的作用,并说明如何干扰的分子机制,参与心外膜依赖的房室交界的形成导致一些异常。这些异常包括AV瓣膜的缺陷,类似于在患有MVD的人类中观察到的那些。这些研究证明了心外膜对于AV瓣膜的正确形成和成熟的重要性,并表明在确定MVD的遗传起源和发病机制时,应考虑心外膜相关发育缺陷的可能性。
This paper is dedicated to the memory of Dr. Adriana “Adri” Gittenberger-de Groot and in appreciation of her work in the field of developmental cardiovascular biology and the legacy that she has left behind. During her impressive career, Dr. Gittenberger-de Groot studied many aspects of heart development, including aspects of cardiac valve formation and disease and the role of the epicardium in the formation of the heart. In this contribution, we review some of the work on the role of epicardially-derived cells (EPDCs) in the development of the atrioventricular valves and their potential involvement in the pathogenesis of myxomatous valve disease (MVD). We provide an overview of critical events in the development of the atrioventricular junction, discuss the role of the epicardium in these events, and illustrate how interfering with molecular mechanisms that are involved in the epicardial-dependent formation of the atrioventricular junction leads to a number of abnormalities. These abnormalities include defects of the AV valves that resemble those observed in humans that suffer from MVD. The studies demonstrate the importance of the epicardium for the proper formation and maturation of the AV valves and show that the possibility of epicardial-associated developmental defects should be taken into consideration when determining the genetic origin and pathogenesis of MVD.
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