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

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...

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Autores principales: 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
Formato: article
Lenguaje:EN
Publicado: Nature Portfolio 2017
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Acceso en línea:https://doaj.org/article/b1726fa086ae482099aa7acc7f31f83b
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Sumario: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.