Bicarbonate and functional CFTR channel are required for proper mucin secretion and link cystic fibrosis with its mucus phenotype-Reference-Cited by-同舟云学术

Bicarbonate and functional CFTR channel are required for proper mucin secretion and link cystic fibrosis with its mucus phenotype

Published:2012-06-18 Issue:7 Volume:209 Page:1263-1272
ISSN:1540-9538
Container-title:Journal of Experimental Medicine
language:en
Short-container-title:

Author:

Gustafsson Jenny K.¹,Ermund Anna¹,Ambort Daniel¹,Johansson Malin E.V.¹,Nilsson Harriet E.²,Thorell Kaisa¹,Hebert Hans²,Sjövall Henrik¹,Hansson Gunnar C.¹

Affiliation:

1. Department of Medical Biochemistry and Department of Internal Medicine, University of Gothenburg, 405 30 Gothenburg, Sweden

2. Department of Biosciences and Nutrition, Karolinska Institutet, 14157 Huddinge, Sweden

Abstract

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.

Publisher

Rockefeller University Press

Subject

Immunology,Immunology and Allergy

Link

http://rupress.org/jem/article-pdf/209/7/1263/1208652/jem_20120562.pdf

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