Emerging Roles of Airway Epithelial Cells in Idiopathic Pulmonary Fibrosis
Member of the German Center for Lung Research (DZL), Institute of Lung Health and Immunity and Comprehensive Pneumology Center with the CPC-M BioArchive, Helmholtz Zentrum München GmbH, 81377 Munich, Germany
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Academic Editors: Malgorzata Wygrecka and Elie El Agha
Cells 2022, 11(6), 1050; https://doi.org/10.3390/cells11061050
Received: 1 February 2022
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Revised: 16 March 2022
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Accepted: 17 March 2022
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Published: 19 March 2022
(This article belongs to the Special Issue State of the Art in Idiopathic Pulmonary Fibrosis)
Idiopathic pulmonary fibrosis (IPF) is a fatal disease with incompletely understood aetiology 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 mechanistic 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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Keywords:
basal cells; bronchial epithelium; airway epithelium; lung fibrosis; MUC5B; single cell RNA sequencing; epithelial populations; IPF
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MDPI and ACS Style
Chakraborty, A.; Mastalerz, M.; Ansari, M.; Schiller, H.B.; Staab-Weijnitz, C.A. Emerging Roles of Airway Epithelial Cells in Idiopathic Pulmonary Fibrosis. Cells 2022, 11, 1050. https://doi.org/10.3390/cells11061050
AMA Style
Chakraborty A, Mastalerz M, Ansari M, Schiller HB, Staab-Weijnitz CA. Emerging Roles of Airway Epithelial Cells in Idiopathic Pulmonary Fibrosis. Cells. 2022; 11(6):1050. https://doi.org/10.3390/cells11061050
Chicago/Turabian StyleChakraborty, Ashesh, Michal Mastalerz, Meshal Ansari, Herbert B. Schiller, and Claudia A. Staab-Weijnitz. 2022. "Emerging Roles of Airway Epithelial Cells in Idiopathic Pulmonary Fibrosis" Cells 11, no. 6: 1050. https://doi.org/10.3390/cells11061050
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