肌成纤维细胞
肺纤维化
纤维化
特发性肺纤维化
肺
病理
人口
医学
癌症研究
生物
内科学
环境卫生
作者
Xinhui Wu,Daoqin Zhang,Xuguang Qiao,Li Zhang,Xinjiang Cai,Jaden Ji,A. Jocelyn,Yan Zhao,John A. Belperio,Kristina I. Boström,Yucheng Yao
出处
期刊:The European respiratory journal
[European Respiratory Society]
日期:2023-02-09
卷期号:61 (6): 2201799-2201799
被引量:7
标识
DOI:10.1183/13993003.01799-2022
摘要
Pulmonary fibrosis is a common and severe fibrotic lung disease with high morbidity and mortality. Recent studies have reported a large number of unwanted myofibroblasts appearing in pulmonary fibrosis, and shown that the sustained activation of myofibroblasts is essential for unremitting interstitial fibrogenesis. However, the origin of these myofibroblasts remains poorly understood. Here, we create new mouse models of pulmonary fibrosis and identify a previously unknown population of endothelial cell (EC)-like myofibroblasts in normal lung tissue. We show that these EC-like myofibroblasts significantly contribute myofibroblasts to pulmonary fibrosis, which is confirmed by single-cell RNA sequencing of human pulmonary fibrosis. Using the transcriptional profiles, we identified a small molecule that redirects the differentiation of EC-like myofibroblasts and reduces pulmonary fibrosis in our mouse models. Our study reveals the mechanistic underpinnings of the differentiation of EC-like myofibroblasts in pulmonary fibrosis and may provide new strategies for therapeutic interventions.
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