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Emerging roles of airway epithelial cells in idiopathic pulmonary fibrosis.

Cells 11:1050 (2022)
Verlagsversion DOI PMC
Open Access Gold
Creative Commons Lizenzvertrag
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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Publikationstyp Artikel: Journalartikel
Dokumenttyp Review
Schlagwörter Airway Epithelium ; Basal Cells ; Bronchial Epithelium ; Epithelial Populations ; Ipf ; Lung Fibrosis ; Muc5b ; Single Cell Rna Sequencing
Sprache englisch
Veröffentlichungsjahr 2022
HGF-Berichtsjahr 2022
ISSN (print) / ISBN 2073-4409
e-ISSN 2073-4409
Zeitschrift Cells
Quellenangaben Band: 11, Heft: 6, Seiten: , Artikelnummer: 1050 Supplement: ,
Verlag MDPI
Verlagsort Basel
Institut(e) Institute of Lung Health and Immunity (LHI)
German Center for Lung Research (DZL)
POF Topic(s) 80000 - German Center for Lung Research
30202 - Environmental Health
Forschungsfeld(er) Lung Research
PSP-Element(e) G-501800-817
G-501600-001
G-501800-810
Förderungen
Deutsche Forschungsgemeinschaft
Scopus ID 85126578732
PubMed ID 35326501
Erfassungsdatum 2022-07-20