Pathogenic Mechanisms Underlying Idiopathic Pulmonary Fibrosis
| Autor: | Stefan W. Ryter, Benjamin J. Moss, Ivan O. Rosas |
|---|---|
| Rok vydání: | 2022 |
| Předmět: |
Cell type
business.industry Mesenchymal stem cell Endothelial Cells Context (language use) respiratory system medicine.disease Idiopathic Pulmonary Fibrosis Pathology and Forensic Medicine Pathogenesis Idiopathic pulmonary fibrosis Cancer research Humans Medicine Epigenetics Signal transduction business Lung Myofibroblast Signal Transduction |
| Zdroj: | Annual Review of Pathology: Mechanisms of Disease. 17:515-546 |
| ISSN: | 1553-4014 1553-4006 |
| DOI: | 10.1146/annurev-pathol-042320-030240 |
| Popis: | The pathogenesis of idiopathic pulmonary fibrosis (IPF) involves a complex interplay of cell types and signaling pathways. Recurrent alveolar epithelial cell (AEC) injury may occur in the context of predisposing factors (e.g., genetic, environmental, epigenetic, immunologic, and gerontologic), leading to metabolic dysfunction, senescence, aberrant epithelial cell activation, and dysregulated epithelial repair. The dysregulated epithelial cell interacts with mesenchymal, immune, and endothelial cells via multiple signaling mechanisms to trigger fibroblast and myofibroblast activation. Recent single-cell RNA sequencing studies of IPF lungs support the epithelial injury model. These studies have uncovered a novel type of AEC with characteristics of an aberrant basal cell, which may disrupt normal epithelial repair and propagate a profibrotic phenotype. Here, we review the pathogenesis of IPF in the context of novel bioinformatics tools as strategies to discover pathways of disease, cell-specific mechanisms, and cell-cell interactions that propagate the profibrotic niche. Expected final online publication date for the Annual Review of Pathology: Mechanisms of Disease, Volume 17 is January 2022. Please see http://www.annualreviews.org/page/journal/pubdates for revised estimates. |
| Databáze: | OpenAIRE |
| Externí odkaz: |
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