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Bicarbonate and functional CFTR channel are required for proper mucin secretion and link cystic fibrosis with its mucus phenotype
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2012 (English)In: Journal of Experimental Medicine, ISSN 0022-1007, E-ISSN 1540-9538, Vol. 209, no 7, 1263-1272 p.Article in journal (Refereed) Published
Abstract [en]

Cystic fibrosis (CF) is caused by a nonfunctional chloride and bicarbonate ion channel (CF transmembrane regulator [CFTR]), but the link to the phenomenon of stagnant mucus is not well understood. Mice lacking functional CFTR (CftrΔ508) have no lung phenotype but show similar ileal problems to humans. We show that the ileal mucosa in CF have a mucus that adhered to the epithelium, was denser, and was less penetrable than that of wild-type mice. The properties of the ileal mucus of CF mice were normalized by secretion into a high concentration sodium bicarbonate buffer (~100 mM). In addition, bicarbonate added to already formed CF mucus almost completely restored the mucus properties. This knowledge may provide novel therapeutic options for CF.

Place, publisher, year, edition, pages
2012. Vol. 209, no 7, 1263-1272 p.
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Cell and Molecular Biology
URN: urn:nbn:se:kth:diva-160351DOI: 10.1084/jem.20120562ISI: 000306174300004ScopusID: 2-s2.0-84864313785OAI: diva2:789441
Swedish Research Council, 7461Swedish Research Council, 21027Swedish Cancer SocietyKnut and Alice Wallenberg FoundationEU, FP7, Seventh Framework Programme, 200931Swedish Foundation for Strategic Research

QC 20150414

Available from: 2015-02-18 Created: 2015-02-18 Last updated: 2015-04-14Bibliographically approved

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