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Regenerative Metaplastic Clones in COPD Lung Drive Inflammation and Fibrosis
Journal article   Open access   Peer reviewed

Regenerative Metaplastic Clones in COPD Lung Drive Inflammation and Fibrosis

Wei Rao, Shan Wang, Marcin Duleba, Suchan Niroula, Kristina Goller, Jingzhong Xie, Rajasekaran Mahalingam, Rahul Neupane, Audrey-Ann Liew, Matthew Vincent, …
Cell, Vol.181(4), pp.848-864.e18
05/14/2020
DOI: 10.1016/j.cell.2020.03.047
PMCID: PMC7294989
PMID: 32298651
url
https://doi.org/10.1016/j.cell.2020.03.047View
Published (Version of record) Open Access

Abstract

Chronic obstructive pulmonary disease (COPD) is a progressive condition of chronic bronchitis, small airway obstruction, and emphysema that represents a leading cause of death worldwide. While inflammation, fibrosis, mucus hypersecretion, and metaplastic epithelial lesions are hallmarks of this disease, their origins and dependent relationships remain unclear. Here we apply single-cell cloning technologies to lung tissue of patients with and without COPD. Unlike control lungs, which were dominated by normal distal airway progenitor cells, COPD lungs were inundated by three variant progenitors epigenetically committed to distinct metaplastic lesions. When transplanted to immunodeficient mice, these variant clones induced pathology akin to the mucous and squamous metaplasia, neutrophilic inflammation, and fibrosis seen in COPD. Remarkably, similar variants pre-exist as minor constituents of control and fetal lung and conceivably act in normal processes of immune surveillance. However, these same variants likely catalyze the pathologic and progressive features of COPD when expanded to high numbers. [Display omitted] •COPD lung is dominated by three pathogenic stem cell variants•These variants are epigenetically committed to metaplastic lesions found in COPD•Variants drive key COPD features of inflammation, fibrosis, and airway obstruction•Similar variants in fetal and normal lung likely serve sentinel functions The hallmark features of COPD (inflammation, fibrosis, and mucus hypersecretion) are driven by distinct pathogenic progenitors which pre-exist as minor populations in healthy lungs but dominate in the disease state relative to normal lung stem cells.
chronic lung disease COPD fibrosis inflammation lung metaplasia myofibroblasts neutrophils p63 single cell cloning stem cells

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