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Publication : G551D CF mice display an abnormal host response and have impaired clearance of Pseudomonas lung disease.

First Author  McMorran BJ Year  2001
Journal  Am J Physiol Lung Cell Mol Physiol Volume  281
Issue  3 Pages  L740-7
PubMed ID  11504703 Mgi Jnum  J:108633
Mgi Id  MGI:3624455 Doi  10.1152/ajplung.2001.281.3.L740
Citation  McMorran BJ, et al. (2001) G551D CF mice display an abnormal host response and have impaired clearance of Pseudomonas lung disease. Am J Physiol Lung Cell Mol Physiol 281(3):L740-7
abstractText  Several 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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