{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,5]],"date-time":"2026-09-05T00:11:43Z","timestamp":1788567103224,"version":"build-2803163510"},"reference-count":183,"publisher":"Frontiers Media SA","license":[{"start":{"date-parts":[[2024,4,18]],"date-time":"2024-04-18T00:00:00Z","timestamp":1713398400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["frontiersin.org"],"crossmark-restriction":true},"short-container-title":["Front. Physiol."],"abstract":"<jats:p>Dysfunction of the cystic fibrosis transmembrane conductance regulator (CFTR) anion channel by genetic mutations causes the inherited disease cystic fibrosis (CF). CF lung disease that involves multiple disorders of epithelial function likely results from loss of CFTR function as an anion channel conducting chloride and bicarbonate ions and its function as a cellular regulator modulating the activity of membrane and cytosol proteins. In the absence of CFTR activity, abundant mucus accumulation, bacterial infection and inflammation characterize CF airways, in which inflammation-associated tissue remodeling and damage gradually destroys the lung. Deciphering the link between CFTR dysfunction and bacterial infection in CF airways may reveal the pathogenesis of CF lung disease and guide the development of new treatments. Research efforts towards this goal, including high salt, low volume, airway surface liquid acidosis and abnormal mucus hypotheses are critically reviewed.<\/jats:p>","DOI":"10.3389\/fphys.2024.1385661","type":"journal-article","created":{"date-parts":[[2024,4,18]],"date-time":"2024-04-18T06:15:07Z","timestamp":1713420907000},"update-policy":"https:\/\/doi.org\/10.3389\/crossmark-policy","source":"Crossref","is-referenced-by-count":5,"title":["CFTR dysfunction leads to defective bacterial eradication on cystic fibrosis airways"],"prefix":"10.3389","volume":"15","author":[{"given":"Min","family":"Wu","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jeng-Haur","family":"Chen","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1965","published-online":{"date-parts":[[2024,4,18]]},"reference":[{"key":"B1","doi-asserted-by":"publisher","first-page":"e89752","DOI":"10.1172\/jci.insight.89752","article-title":"Defective postsecretory maturation of MUC5B mucin in cystic fibrosis airways","volume":"2","author":"Abdullah","year":"2017","journal-title":"JCI Insight"},{"key":"B2","doi-asserted-by":"publisher","first-page":"373","DOI":"10.1016\/j.jcf.2013.12.006","article-title":"Neonates with cystic fibrosis have a reduced nasal liquid pH; a small pilot study","volume":"13","author":"Abou Alaiwa","year":"","journal-title":"J. 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