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Targeting Alveolar Repair in Idiopathic Pulmonary Fibrosis

Authors :
Lynne Murray
Darcy E. Wagner
Victoria A. Ptasinski
John Stegmayr
Maria G. Belvisi
Source :
American Journal of Respiratory Cell and Molecular Biology
Publication Year :
2021
Publisher :
American Thoracic Society, 2021.

Abstract

Idiopathic pulmonary fibrosis is a fatal interstitial lung disease with limited therapeutic options. Current evidence suggests that IPF may be initiated by repeated epithelial injury in the distal lung followed by abnormal wound healing responses which occur due to intrinsic and extrinsic factors. Mechanisms contributing to chronic damage of the alveolar epithelium in IPF include dysregulated cellular processes such as apoptosis, senescence, abnormal activation of developmental pathways, aging, as well as genetic mutations. Therefore, targeting the regenerative capacity of the lung epithelium is an attractive approach in the development of novel therapies for IPF. Endogenous lung regeneration is a complex process involving coordinated cross-talk between multiple cell types and re-establishment of a normal extracellular matrix environment. This review will describe the current knowledge of reparative epithelial progenitor cells in the alveolar region of the lung and discuss potential novel therapeutic approaches for IPF focusing on endogenous alveolar repair. This article is open access and distributed under the terms of the Creative Commons Attribution Non-Commercial No Derivatives License 4.0 (http://creativecommons.org/licenses/by-nc-nd/4.0/).

Details

ISSN :
15354989 and 10441549
Volume :
65
Database :
OpenAIRE
Journal :
American Journal of Respiratory Cell and Molecular Biology
Accession number :
edsair.doi.dedup.....bdd3a2f8de9c3c0351ba2e9b0c45cef8
Full Text :
https://doi.org/10.1165/rcmb.2020-0476tr