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Lung disease in the cystic fibrosis mouse exposed to bacterial pathogens

Nature Genetics volume 9, pages 351–357 (1995)Cite this article

Abstract

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.

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Authors and Affiliations

  1. MRC Human Genetics Unit, Western General Hospital, Crewe Road, Edinburgh, EH4 2XU, UK

    Donald J. Davidson, Julia R. Dorin, Gerard McLachlan & David J. Porteous

  2. Biomedical Research Facility, Western General Hospital, Crewe Road, Edinburgh, EH4 2XU, UK

    Vincent Ranaldi

  3. Department of Pathology, University of Edinburgh Medical School, Teviot Place, Edinburgh, UK

    David Lamb

  4. Department of Medical Microbiology, University of Edinburgh Medical School, Teviot Place, Edinburgh, UK

    Catherine Doherty & John Govan

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  1. Donald J. Davidson
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  2. Julia R. Dorin
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  7. John Govan
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  8. David J. Porteous
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Davidson, D., Dorin, J., McLachlan, G. et al. Lung disease in the cystic fibrosis mouse exposed to bacterial pathogens. Nat Genet 9, 351–357 (1995). https://doi.org/10.1038/ng0495-351

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