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DOI10.1038/s41467-019-09959-4
Cystic fibrosis swine fail to secrete airway surface liquid in response to inhalation of pathogens
Luan, Xiaojie1; Belev, George2; Tam, Julian S.3; Jagadeeshan, Santosh1; Hassan, Noman1; Gioino, Paula1; Grishchenko, Nikolay1; Huang, Yanyun4; Carmalt, James L.5; Duke, Tanya6; Jones, Teela6; Monson, Bev7; Burmester, Monique7; Simovich, Tomer8; Yilmaz, Orhan1; Campanucci, Veronica A.1; Machen, Terry E.9; Chapman, L. Dean10; Ianowski, Juan P.1
2019-08-13
发表期刊NATURE COMMUNICATIONS
ISSN2041-1723
出版年2017
卷号8
文章类型Article
语种英语
国家Canada; Australia; USA
英文摘要

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.


领域资源环境
收录类别SCI-E
WOS记录号WOS:000412362400001
WOS关键词TRACHEAL SUBMUCOSAL GLANDS ; MUCUS SECRETION ; FLUID SECRETION ; INFECTION ; PARTICLES ; MODEL ; PIG
WOS类目Multidisciplinary Sciences
WOS研究方向Science & Technology - Other Topics
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文献类型期刊论文
条目标识符http://119.78.100.173/C666/handle/2XK7JSWQ/204495
专题资源环境科学
作者单位1.Univ Saskatchewan, Dept Physiol, Hlth Sci Bldg,Room 2D01,107 Wiggins Rd, Saskatoon, SK S7N 5E5, Canada;
2.Canadian Light Source Inc, 44 Innovat Blvd, Saskatoon, SK S7N 2V3, Canada;
3.Univ Saskatchewan, Div Respirol Crit Care & Sleep Med, Dept Med, Royal Univ Hosp, 103 Hosp Dr, Saskatoon, SK S7N 0W8, Canada;
4.Prairie Diagnost Serv Inc, 52 Campus Dr, Saskatoon, SK S7N 5B4, Canada;
5.Univ Saskatchewan, Western Coll Vet Med, Dept Large Anim Clin Sci, 52 Campus Dr, Saskatoon, SK S7N 5B4, Canada;
6.Univ Saskatchewan, Western Coll Vet Med, Dept Small Anim Clin Sci, 52 Campus Dr, Saskatoon, SK S7N 5B4, Canada;
7.Univ Saskatchewan, Western Coll Vet Med, Anim Care Unit, 52 Campus Dr, Saskatoon, SK S7N 5B4, Canada;
8.Univ Melbourne, Sch Chem, Surface Sci & Technol Grp, Parkville, Vic 3010, Australia;
9.Univ Calif Berkeley, Dept Mol & Cell Biol, 231 LSA, Berkeley, CA 94720 USA;
10.Univ Saskatchewan, Dept Anat & Cell Biol, Hlth Sci Bldg,Room 2D01,107 Wiggins Rd, Saskatoon, SK S7N 5E5, Canada
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GB/T 7714
Luan, Xiaojie,Belev, George,Tam, Julian S.,et al. Cystic fibrosis swine fail to secrete airway surface liquid in response to inhalation of pathogens[J]. NATURE COMMUNICATIONS,2019,8.
APA Luan, Xiaojie.,Belev, George.,Tam, Julian S..,Jagadeeshan, Santosh.,Hassan, Noman.,...&Ianowski, Juan P..(2019).Cystic fibrosis swine fail to secrete airway surface liquid in response to inhalation of pathogens.NATURE COMMUNICATIONS,8.
MLA Luan, Xiaojie,et al."Cystic fibrosis swine fail to secrete airway surface liquid in response to inhalation of pathogens".NATURE COMMUNICATIONS 8(2019).
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