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Desmosomes in the Heart: A Review of Clinical and Mechanistic Analyses

机译:心脏中的桥粒:临床和机理分析的回顾

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Desmosomes have long been appreciated as intercellular junctions that are vital for maintaining the structural integrity of stratified epithelia. More recent clinical investigations of patients with diseases such as arrhythmogenic cardiomyopathy have further highlighted the importance of desmosomes in cardiac tissue, where they help to maintain coordination of cardiac myocytes. Here, we review clinical and mechanistic studies that provide insight into the functions of desmosomal proteins in skin and heart during homeostasis and in disease. While intercellular junctions are organized differently in cardiac and epithelial tissues, studies conducted in epithelial systems may inform our understanding of cardiac desmosomes. We explore traditional and non-traditional roles of desmosomal proteins, ranging from adhesive capacities to nuclear functions. Finally, we discuss how these studies can guide future investigations focused on determining the molecular mechanisms by which desmosomal mutations promote the development of cardiac diseases.
机译:长期以来,桥粒一直被认为是细胞间连接,对于维持分层上皮的结构完整性至关重要。对患有诸如心律失常性心肌病等疾病的患者的最新临床研究进一步强调了心脏组织中桥粒的重要性,因为桥粒有助于维持心肌细胞的协调。在这里,我们回顾了临床和机制研究,这些研究提供了在稳态和疾病中皮肤和心脏中桥粒蛋白功能的见解。虽然在心脏和上皮组织中细胞间连接的组织方式不同,但在上皮系统中进行的研究可能有助于我们了解心脏桥粒。我们探讨了桥粒蛋白的传统和非传统作用,范围从粘附能力到核功能。最后,我们讨论了这些研究如何指导未来的研究,这些研究的重点是确定桥粒突变促进心脏病发展的分子机制。

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