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Publication : Regulating the cell shift of endothelial cell-like myofibroblasts in pulmonary fibrosis.

First Author  Wu X Year  2023
Journal  Eur Respir J Volume  61
Issue  6 PubMed ID  36758986
Mgi Jnum  J:353990 Mgi Id  MGI:7718742
Doi  10.1183/13993003.01799-2022 Citation  Wu X, et al. (2023) Regulating the cell shift of endothelial cell-like myofibroblasts in pulmonary fibrosis. Eur Respir J 61(6)
abstractText  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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