系统性硬皮病中的内皮细胞向间充质细胞转化。
Endothelial-to-mesenchymal transition in systemic sclerosis.
机构信息
Clinical Pathology Unit, Department of Biotechnological and Applied Clinical Sciences, University of L'Aquila, L'Aquila, Italy.
Division of Rheumatology, Department of Biotechnological and Applied Clinical Sciences, University of L'Aquila, L'Aquila, Italy.
出版信息
Clin Exp Immunol. 2021 Jul;205(1):12-27. doi: 10.1111/cei.13599. Epub 2021 Apr 18.
Abstract
Systemic sclerosis (SSc) is an autoimmune disease characterized by significant vascular alterations and multi-organ fibrosis. Microvascular alterations are the first event of SSc and injured endothelial cells (ECs) may transdifferentiate towards myofibroblasts, the cells responsible for fibrosis and collagen deposition. This process is identified as endothelial-to-mesenchymal transition (EndMT), and understanding of its development is pivotal to identify early pathogenetic events and new therapeutic targets for SSc. In this review, we have highlighted the molecular mechanisms of EndMT and summarize the evidence of the role played by EndMT during the development of progressive fibrosis in SSc, also exploring the possible therapeutic role of its inhibition.
摘要
系统性硬化症（SSc）是一种自身免疫性疾病，其特征为显著的血管改变和多器官纤维化。微血管改变是 SSc 的首发事件，受损的内皮细胞（ECs）可能向肌成纤维细胞转分化，肌成纤维细胞是导致纤维化和胶原沉积的细胞。这个过程被确定为内皮细胞-间充质转化（EndMT），了解其发生机制对于确定 SSc 的早期发病机制事件和新的治疗靶点至关重要。在这篇综述中，我们强调了 EndMT 的分子机制，并总结了 EndMT 在 SSc 进行性纤维化发展过程中所起作用的证据，同时还探讨了其抑制作用的可能治疗作用。
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