PDF(Pubmed)
Abstract：
Idiopathic pulmonary fibrosis is a lethal, progressive, and irreversible condition that has become a significant focus of medical research due to its increasing incidence. This rising trend presents substantial challenges for patients, healthcare providers, and researchers. Despite the escalating burden of pulmonary fibrosis, the available therapeutic options remain limited. Currently, the United States Food and Drug Administration has approved two drugs for the treatment of pulmonary fibrosis-nintedanib and pirfenidone. However, their therapeutic effectiveness is limited, and they cannot reverse the fibrosis process. Additionally, these drugs are associated with significant side effects. Myofibroblasts play a central role in the pathophysiology of pulmonary fibrosis, significantly contributing to its progression. Consequently, strategies aimed at inhibiting myofibroblast differentiation or promoting their dedifferentiation hold promise as effective treatments. This review examines the regulation of myofibroblast dedifferentiation, exploring various signaling pathways, regulatory targets, and potential pharmaceutical interventions that could provide new directions for therapeutic development.
摘要：
特发性肺纤维化是一种致命的,进步,和不可逆转的情况，由于其发病率的增加，已成为医学研究的重要焦点。这种上升趋势给患者带来了巨大的挑战，医疗保健提供者，和研究人员。尽管肺纤维化的负担不断增加，可用的治疗选择仍然有限。目前,美国食品和药物管理局已批准两种治疗肺纤维化的药物-尼达尼布和吡非尼酮。然而,它们的治疗效果有限，它们不能逆转纤维化过程。此外,这些药物有明显的副作用。肌成纤维细胞在肺纤维化的病理生理学中起着核心作用，对它的进步有很大的贡献。因此,旨在抑制肌成纤维细胞分化或促进其去分化的策略有望成为有效的治疗方法。这篇综述探讨了肌成纤维细胞去分化的调节，探索各种信号通路，监管目标,以及潜在的药物干预措施，可以为治疗发展提供新的方向。
