Mouse models of cystic fibrosis: phenotypic analysis and research applications.

@article{Wilke2011MouseMO,
  title={Mouse models of cystic fibrosis: phenotypic analysis and research applications.},
  author={Martina Wilke and Ruvalic M Buijs-Offerman and Jamil Aarbiou and William H Colledge and David N Sheppard and Lhousseine Touqui and Alice G M Bot and Huub J. J. Jorna and Hugo R. de Jonge and Bob J Scholte},
  journal={Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society},
  year={2011},
  volume={10 Suppl 2},
  pages={S152-71}
}
Genetically modified mice have been studied for more than fifteen years as models of cystic fibrosis (CF). The large amount of experimental data generated illuminates the complex multi-organ pathology of CF and raises new questions relevant to human disease. CF mice have also been used to test experimental therapies prior to clinical trials. This review recapitulates the major phenotypic traits of CF mice and highlights important new findings including aberrant alveolar macrophages, bone and… CONTINUE READING

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References

Publications referenced by this paper.
Showing 1-10 of 203 references

A F508 mutation in mouse cystic fibrosis transmembrane conductance regulator results in a temperature-sensitive processing defect in vivo

  • PJ French, JH van Doorninck, RH Peters
  • J Clin Invest 1996;98:1304–12
  • 1996
Highly Influential
14 Excerpts

Identification of IFRD1 as a S168

  • Y Gu, IT Harley, LB Henderson
  • Journal of Cystic Fibrosis Volume 10 Suppl
  • 2011
2 Excerpts

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