Potential Contribution of Alveolar Epithelial Type I Cells to Pulmonary Fibrosis
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Pulmonary fibrosis (PF) is characterized by inflammation and fibrosis of the interstitium and destruction of alveolar histoarchitecture ultimately leading to a fatal impairment of lung function. Different concepts describe either a dominant role of inflammatory pathways or a disturbed remodeling of resident cells of the lung parenchyma during fibrogenesis. Further, a combination of both the mechanisms has been postulated. The present review emphasizes the particular involvement of alveolar epithelial type I cells in all these processes, their contribution to innate immune/inflammatory functions and maintenance of proper alveolar barrier functions. Amongst the different inflammatory and repair events the purinergic receptor P2X7, an ATP-gated cationic channel that regulates not only apoptosis, necrosis, autophagy, and NLPR3 inflammosome activation, but also the turnover of diverse tight junction (TJ) and water channel proteins, seems to be essential for the stability of alveolar barrier integrity and for the interaction with protective factors during lung injury.
Ma W, Tang S, Yao P, Zhou T, Niu Q, Liu P Signal Transduct Target Ther. 2025; 10(1):75.
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Zhao Y, Zhou Y, Zhang W, Liu M, Duan J, Zhang X Nat Commun. 2025; 16(1):1303.
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Sims A, Schafer A, Okuda K, Leist S, Kocher J, Cockrell A mSphere. 2025; 10(2):e0095124.
PMID: 39882872 PMC: 11853001. DOI: 10.1128/msphere.00951-24.
Matrix-free human lung organoids derived from induced pluripotent stem cells to model lung injury.
Budeus B, Kroepel C, Stasch L, Klein D Stem Cell Res Ther. 2024; 15(1):468.
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Forde L, Gogoi D, Baird R, McCarthy C, Keane M, Reeves E J Innate Immun. 2024; 17(1):44-55.
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