Nature Communications (Oct 2017)

Cystic fibrosis swine fail to secrete airway surface liquid in response to inhalation of pathogens

  • Xiaojie Luan,
  • George Belev,
  • Julian S. Tam,
  • Santosh Jagadeeshan,
  • Noman Hassan,
  • Paula Gioino,
  • Nikolay Grishchenko,
  • Yanyun Huang,
  • James L. Carmalt,
  • Tanya Duke,
  • Teela Jones,
  • Bev Monson,
  • Monique Burmester,
  • Tomer Simovich,
  • Orhan Yilmaz,
  • Veronica A. Campanucci,
  • Terry E. Machen,
  • L. Dean Chapman,
  • Juan P. Ianowski

DOI
https://doi.org/10.1038/s41467-017-00835-7
Journal volume & issue
Vol. 8, no. 1
pp. 1 – 9

Abstract

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Cystic fibrosis is caused by mutations in the CFTR chloride channel, leading to reduced airway surface liquid secretion. Here the authors show that exposure to bacteria triggers secretion in wild-type but not in pig models of cystic fibrosis, suggesting an impaired response to pathogens contributes to infection.