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Emerging roles of airway epithelial cells in idiopathic pulmonary fibrosis.
Cells 11:1050 (2022)
Idiopathic pulmonary fibrosis (IPF) is a fatal disease with incompletely understood aetiol-ogy and limited treatment options. Traditionally, IPF was believed to be mainly caused by repetitive injuries to the alveolar epithelium. Several recent lines of evidence, however, suggest that IPF equally involves an aberrant airway epithelial response, which contributes significantly to disease development and progression. In this review, based on recent clinical, high-resolution imaging, genetic, and single-cell RNA sequencing data, we summarize alterations in airway structure, function, and cell type composition in IPF. We furthermore give a comprehensive overview on the genetic and mecha-nistic evidence pointing towards an essential role of airway epithelial cells in IPF pathogenesis and describe potentially implicated aberrant epithelial signalling pathways and regulation mechanisms in this context. The collected evidence argues for the investigation of possible therapeutic avenues targeting these processes, which thus represent important future directions of research.
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Publication type
Article: Journal article
Document type
Review
Keywords
Airway Epithelium ; Basal Cells ; Bronchial Epithelium ; Epithelial Populations ; Ipf ; Lung Fibrosis ; Muc5b ; Single Cell Rna Sequencing
ISSN (print) / ISBN
2073-4409
e-ISSN
2073-4409
Journal
Cells
Quellenangaben
Volume: 11,
Issue: 6,
Article Number: 1050
Publisher
MDPI
Publishing Place
Basel
Non-patent literature
Publications
Institute(s)
Lung Health and Immunity (LHI)
Grants
Deutsche Forschungsgemeinschaft
Deutsche Forschungsgemeinschaft