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Type: Journal article
Title: Airway disease phenotypes in animal models of cystic fibrosis
Author: McCarron, A.
Donnelley, M.
Parsons, D.
Citation: Respiratory Research, 2018; 19(1):54-1-54-12
Publisher: BioMed Central
Issue Date: 2018
ISSN: 1465-9921
Statement of
Alexandra McCarron, Martin Donnelley and David Parsons
Abstract: In humans, cystic fibrosis (CF) lung disease is characterised by chronic infection, inflammation, airway remodelling, and mucus obstruction. A lack of pulmonary manifestations in CF mouse models has hindered investigations of airway disease pathogenesis, as well as the development and testing of potential therapeutics. However, recently generated CF animal models including rat, ferret and pig models demonstrate a range of well characterised lung disease phenotypes with varying degrees of severity. This review discusses the airway phenotypes of currently available CF animal models and presents potential applications of each model in airway-related CF research.
Keywords: Cystic fibrosis; animal models; lung disease; pathology
Rights: © The Author(s). 2018 Open Access This article is distributed under the terms of the Creative Commons Attribution 4.0 International License (, which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. The Creative Commons Public Domain Dedication waiver ( applies to the data made available in this article, unless otherwise stated.
DOI: 10.1186/s12931-018-0750-y
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