Journal
RESPIRATORY RESEARCH
Volume 19, Issue -, Pages -Publisher
BMC
DOI: 10.1186/s12931-018-0750-y
Keywords
Cystic fibrosis; Animal models; Lung disease; Pathology
Categories
Funding
- NHMRC [APP1098127]
- Women's and Children's Hospital Foundation
- MS McLeod PhD Scholarship
- Cure4CF Foundation
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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.
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