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

被引：284

作者：

Gustafsson, Jenny K. ^{[1
]}

Ermund, Anna ^{[1
]}

Ambort, Daniel ^{[1
]}

Johansson, Malin E. V. ^{[1
]}

Nilsson, Harriet E. ^{[3
]}

Thorell, Kaisa ^{[1
]}

Hebert, Hans ^{[3
]}

Sjovall, Henrik ^{[2
]}

Hansson, Gunnar C. ^{[1
]}

机构：

[1] Univ Gothenburg, Dept Med Biochem, S-40530 Gothenburg, Sweden

[2] Univ Gothenburg, Dept Internal Med, S-40530 Gothenburg, Sweden

[3] Karolinska Inst, Dept Biosci & Nutr, S-14157 Huddinge, Sweden

来源：

JOURNAL OF EXPERIMENTAL MEDICINE | 2012年 / 209卷 / 07期

基金：

瑞典研究理事会; 欧盟第七框架计划;

关键词：

MOUSE SMALL-INTESTINE; IN-VIVO; MECHANISM; RELEASE; LAYER; IDENTIFICATION; GLYCOPROTEINS; EXPRESSION; THICKNESS; TRANSPORT;

D O I：

10.1084/jem.20120562

中图分类号：

R392 [医学免疫学]; Q939.91 [免疫学];

学科分类号：

100102 ;

摘要：

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 (Cftr Delta 508) 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 (similar to 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.

引用

页码：1263 / 1272

页数：10

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