Lung disease in the cystic fibrosis mouse exposed to bacterial pathogens

Nat Genet. 1995 Apr;9(4):351-7. doi: 10.1038/ng0495-351.


Lung disease is the major cause of death in cystic fibrosis (CF), but there is no evidence for overt lung involvement at birth. We show here that the same is true for the gene targeted cftrm1HGU mutant mouse. Furthermore, this CF mouse model demonstrates an impaired capacity to clear Staphylococcus aureus and Burkholderia (Pseudomonas) cepacia, two opportunistic lung pathogens closely associated with lung disease in CF subjects. The cftrm1HGU homozygotes display mucus retention and frank lung disease in response to repeated microbial exposure. Thus, lung disease in the cftrm1HGU mouse develops in response to bacterial infection, establishing a model to dissect the pathogenesis of CF pulmonary disease and providing a clinically relevant end point to assess the efficacy of pharmacologic or genetic interventions.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Animals
  • Bacterial Infections / etiology*
  • Bacterial Infections / pathology
  • Burkholderia cepacia
  • Cystic Fibrosis / complications*
  • Cystic Fibrosis / genetics*
  • Cystic Fibrosis / pathology
  • Disease Models, Animal
  • Homozygote
  • Humans
  • Lung / microbiology
  • Lung / pathology
  • Lung Diseases / etiology*
  • Lung Diseases / pathology
  • Mice
  • Mice, Mutant Strains
  • Mice, Transgenic
  • Opportunistic Infections / etiology*
  • Opportunistic Infections / pathology
  • Pseudomonas Infections / etiology
  • Pseudomonas Infections / pathology
  • Staphylococcal Infections / etiology
  • Staphylococcal Infections / pathology