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

B J McMorran, J S Palmer, D P Lunn, D Oceandy, E O Costelloe, G R Thomas, D A Hume, B J Wainwright

Research output: Contribution to journalArticlepeer-review


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.
Original languageEnglish
Pages (from-to)L740-7
JournalAmerican Journal of Physiology - Lung Cellular and Molecular Physiology
Issue number3
Publication statusPublished - Sep 2001


  • Alleles
  • Animals
  • Body Weight
  • Bronchoalveolar Lavage Fluid
  • Chronic Disease
  • Colony Count, Microbial
  • Cystic Fibrosis
  • Cystic Fibrosis Transmembrane Conductance Regulator
  • Cytokines
  • Homozygote
  • Inflammation Mediators
  • Lung
  • Lung Diseases
  • Mice
  • Mice, Mutant Strains
  • Mutation
  • Pneumonia, Bacterial
  • Pseudomonas Infections
  • Pseudomonas aeruginosa
  • Reference Values


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