Journal
AMERICAN JOURNAL OF RESPIRATORY CELL AND MOLECULAR BIOLOGY
Volume 65, Issue 4, Pages 347-365Publisher
AMER THORACIC SOC
DOI: 10.1165/rcmb.2020-0476TR
Keywords
epithelium; lung; regeneration; stem cells
Funding
- AstraZeneca
- Knut and Alice Wallenberg Foundation [PA2016-1522]
- Medical Faculty at Lund University, Region Skane
- Swedish Research Council [2018-02352]
- Swedish Research Council [2018-02352] Funding Source: Swedish Research Council
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Idiopathic pulmonary fibrosis (IPF) is a fatal interstitial lung disease initiated by repeated epithelial injuries, leading to abnormal wound healing responses. Targeting the regenerative capacity of lung epithelium is crucial for developing novel therapies for IPF, which involves coordinated cross-talk among multiple cell types and reestablishment of a normal extracellular matrix environment.
Idiopathic pulmonary fibrosis (IPF) is a fatal interstitial lung disease with limited therapeutic options. Current evidence suggests that IPF may be initiated by repeated epithelial injuries in the distal lung, which are followed by abnormal wound healing responses that occur because of 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 the developmental pathways, aging, and 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 among multiple cell types and reestablishment 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.
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