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 and Juan P. Ianowski ()
Additional contact information
Xiaojie Luan: University of Saskatchewan
George Belev: Canadian Light Source Inc.
Julian S. Tam: University of Saskatchewan
Santosh Jagadeeshan: University of Saskatchewan
Noman Hassan: University of Saskatchewan
Paula Gioino: University of Saskatchewan
Nikolay Grishchenko: University of Saskatchewan
Yanyun Huang: Prairie Diagnostic Services Inc.
James L. Carmalt: University of Saskatchewan
Tanya Duke: University of Saskatchewan
Teela Jones: University of Saskatchewan
Bev Monson: University of Saskatchewan
Monique Burmester: University of Saskatchewan
Tomer Simovich: The University of Melbourne
Orhan Yilmaz: University of Saskatchewan
Veronica A. Campanucci: University of Saskatchewan
Terry E. Machen: University of California
L. Dean Chapman: Department of Anatomy and Cell Biology
Juan P. Ianowski: University of Saskatchewan

Nature Communications, 2017, vol. 8, issue 1, 1-9

Abstract: Abstract Cystic fibrosis is caused by mutations in the gene encoding the cystic fibrosis transmembrane conductance regulator (CFTR) channel, which can result in chronic lung disease. The sequence of events leading to lung disease is not fully understood but recent data show that the critical pathogenic event is the loss of the ability to clear bacteria due to abnormal airway surface liquid secretion (ASL). However, whether the inhalation of bacteria triggers ASL secretion and whether this is abnormal in cystic fibrosis has never been tested. Here we show, using a novel synchrotron-based in vivo imaging technique, that wild-type pigs display both a basal and a Toll-like receptor-mediated ASL secretory response to the inhalation of cystic fibrosis relevant bacteria. Both mechanisms fail in CFTR−/− swine, suggesting that cystic fibrosis airways do not respond to inhaled pathogens, thus favoring infection and inflammation that may eventually lead to tissue remodeling and respiratory disease.

Date: 2017
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DOI: 10.1038/s41467-017-00835-7

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