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心外膜在房室瓣发育中的作用及其与黏液瘤样瓣膜病发病机制的相关性。

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

作者信息

Wolters Renélyn, Deepe Ray, Drummond Jenna, Harvey Andrew B, Hiriart Emilye, Lockhart Marie M, van den Hoff Maurice J B, Norris Russell A, Wessels Andy

机构信息

Department of Regenerative Medicine and Cell Biology, Medical University of South Carolina, 173 Ashley Avenue, Charleston, SC 29425, USA.

Department of Medical Biology, Amsterdam UMC, Academic Medical Center, Meibergdreef 15, 1105 AZ Amsterdam, The Netherlands.

出版信息

J Cardiovasc Dev Dis. 2021 May 12;8(5):54. doi: 10.3390/jcdd8050054.

DOI:10.3390/jcdd8050054
PMID:34066253
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC8152025/
Abstract

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.

摘要

本文谨献给阿德里亚娜·“阿德里”·吉滕贝格尔 - 德格鲁特博士，以缅怀她在发育心血管生物学领域的工作以及她留下的遗产。在她令人瞩目的职业生涯中，吉滕贝格尔 - 德格鲁特博士研究了心脏发育的许多方面，包括心脏瓣膜形成与疾病的各个方面以及心外膜在心脏形成中的作用。在本论文中，我们回顾了一些关于心外膜来源细胞（EPDCs）在房室瓣发育中的作用及其可能参与黏液瘤样瓣膜病（MVD）发病机制的研究工作。我们概述了房室交界处发育中的关键事件，讨论了心外膜在这些事件中的作用，并阐述了干扰与心外膜依赖性房室交界处形成相关的分子机制如何导致一系列异常。这些异常包括类似于患有MVD的人类所观察到的房室瓣缺陷。这些研究证明了心外膜对于房室瓣正常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。

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