Murine Models of Acute and Chronic Lung Infection with Cystic Fibrosis Pathogens

Animal models of acute and chronic infection, along with mice genetically modified for the Cftr gene, are a key asset in cystic fibrosis (CF) research. Despite some limitations, these models provide valuable resources to mimic the initial and progressive bronchopulmonary infection typical of CF patients. The following review summarizes the strengths and weaknesses of different types of animal models with a major emphasis placed on the significant species differences between mice and humans. Murine models of acute and chronic lung infection with Pseudomonas aeruginosa, Burkholderia cenocepacia, Staphylococcus aureus, and Haemophilus influenzae have been used to study the molecular mechanisms underlying the pathogen virulence and host defense. In addition, they have provided insights in the potential of vaccination to restrict infectious exacerbations, the activity of antibiotics, and the effectiveness of anti-inflammatory therapy in reducing lung damage. Indeed, animal models of infection should allow the validation of future therapeutic interventions for lung infections in patients with CF.