• B J McMorran, J S Palmer, D P Lunn, D Oceandy, E O Costelloe, G R Thomas, D A Hume, and B J Wainwright.
• Institute for Molecular Biosciences, University of Queensland, St. Lucia, Queensland 4072, Australia.
• Am. J. Physiol. Lung Cell Mol. Physiol. 2001 Sep 1; 281 (3): L740-7.

AbstractSeveral cystic fibrosis (CF) mouse models demonstrate an increased susceptibility to Pseudomonas aeruginosa lung infection, characterized by excessive inflammation and high rates of mortality. Here we developed a model of chronic P. aeruginosa lung disease in mice homozygous for the murine CF transmembrane conductance regulator G551D mutation that provides an excellent model for CF lung disease. After 3 days of infection with mucoid P. aeruginosa entrapped in agar beads, the G551D animals lost substantially more body weight than non-CF control animals and were less able to control the infection, harboring over 40-fold more bacteria in the lung. The airways of infected G551D animals contained altered concentrations of the inflammatory mediators tumor necrosis factor-alpha, KC/N51, and macrophage inflammatory protein-2 during the first 2 days of infection, suggesting that an ineffective inflammatory response is partly responsible for the clearance defect.

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