Recapitulating idiopathic pulmonary fibrosis related alveolar epithelial dysfunction in a human iPSC-derived air-liquid interface model.

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ID: 104292
2020
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Ranked #14 of 39 articles by views in faseb journal : official publication of the federation of american societies for experimental biology

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Abstract
Idiopathic pulmonary fibrosis (IPF) is a fatal disease of unknown cause that is characterized by progressive fibrotic lung remodeling. An abnormal emergence of airway epithelial-like cells within the alveolar compartments of the lung, herein termed bronchiolization, is often observed in IPF. However, the origin of this dysfunctional distal lung epithelium remains unknown due to a lack of suitable human model systems. In this study, we established a human induced pluripotent stem cell (iPSC)-derived air-liquid interface (ALI) model of alveolar epithelial type II (ATII)-like cell differentiation that allows us to investigate alveolar epithelial progenitor cell differentiation in vitro. We treated this system with an IPF-relevant cocktail (IPF-RC) to mimic the pro-fibrotic cytokine milieu present in IPF lungs. Stimulation with IPF-RC during differentiation increases secretion of IPF biomarkers and RNA sequencing (RNA-seq) of these cultures reveals significant overlap with human IPF patient data. IPF-RC treatment further impairs ATII differentiation by driving a shift toward an airway epithelial-like expression signature, providing evidence that a pro-fibrotic cytokine environment can influence the proximo-distal differentiation pattern of human lung epithelial cells. In conclusion, we show for the first time, the establishment of a human model system that recapitulates aspects of IPF-associated bronchiolization of the lung epithelium in vitro.
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schruf2020recapitulatingfaseb Use this key to autocite in the manuscript while using SciMatic Manuscript Manager or Thesis Manager
Authors Eva Schruf, Victoria Schroeder, Huy Q Le, Tanja Schönberger, Dagmar Raedel, Emily L Stewart, Katrin Fundel-Clemens, Teresa Bluhmki, Sabine Weigle, Michael Schuler, Matthew J Thomas, Ralf Heilker, Megan J Webster, Martin Dass, Manfred Frick, Birgit Stierstorfer, Karsten Quast, James P Garnett
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Journal faseb journal : official publication of the federation of american societies for experimental biology
Year 2020
DOI
10.1096/fj.201902926R
URL
Keywords
induced pluripotent stem cells ipf alveolar epithelium bronchiolization pro-fibrotic milieu

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