Airway disease phenotypes in animal models of cystic fibrosis

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 testi...

Full description

Bibliographic Details
Main Authors: Alexandra McCarron, Martin Donnelley, David Parsons
Format: Article
Language:English
Published: BMC 2018-04-01
Series:Respiratory Research
Subjects:
Online Access:http://link.springer.com/article/10.1186/s12931-018-0750-y
Description
Summary: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.
ISSN:1465-993X