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

Xiaojie Luan1, George Belev2, Julian S Tam3

  • 1Department of Physiology, University of Saskatchewan, Health Science Building, Room 2D01, 107 Wiggins Road, Saskatoon, SK, Canada, S7N 5E5.

Nature Communications
|October 7, 2017
PubMed

Insights

Cystic fibrosis (CF) airways fail to secrete liquid when exposed to bacteria. This impaired response in CFTR-mutated pigs suggests a mechanism for chronic lung infections in cystic fibrosis.

Area of Science:

  • Pulmonary Medicine
  • Microbiology
  • Genetics

Background:

  • Cystic fibrosis (CF) results from mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene, leading to chronic lung disease.
  • Impaired airway surface liquid (ASL) secretion and bacterial clearance are key pathogenic events in CF lung disease.
  • It remains unclear if inhaled bacteria trigger ASL secretion and if this response is abnormal in CF.

Purpose of the Study:

  • To investigate whether inhaled bacteria stimulate ASL secretion.
  • To determine if this response is impaired in cystic fibrosis models.

Main Methods:

  • Utilized a novel synchrotron-based in vivo imaging technique.
  • Exposed wild-type and CFTR-deficient (CFTR-/-) pigs to cystic fibrosis-relevant bacteria.

Main Results:

  • Wild-type pigs demonstrated both basal and Toll-like receptor-mediated ASL secretion in response to inhaled bacteria.
  • CFTR-/- swine exhibited a complete failure of both basal and stimulated ASL secretion.
  • This suggests CF airways do not adequately respond to inhaled pathogens.

Conclusions:

  • The study reveals a critical defect in the airway's response to inhaled bacteria in cystic fibrosis.
  • This impaired pathogen sensing and ASL secretion likely contributes to increased infection and inflammation in CF lungs.
  • The findings highlight a potential therapeutic target for preventing CF lung disease progression.

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