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

• Published in: The Journal of experimental medicine Vol. 209; no. 7; pp. 1263 - 1272
• Main Authors: Gustafsson, Jenny K, Ermund, Anna, Ambort, Daniel, Johansson, Malin E V, Nilsson, Harriet E, Thorell, Kaisa, Hebert, Hans, Sjövall, Henrik, Hansson, Gunnar C
• Format: Journal Article
• Language: English
• Published: United States The Rockefeller University Press 02.07.2012
• Subjects: Animals; Basic Medicine; Bicarbonates; Bicarbonates - pharmacology; Cell and Molecular Biology; Cell- och molekylärbiologi; Confocal; Cystic Fibrosis; Cystic Fibrosis - genetics; Cystic Fibrosis - metabolism; Cystic Fibrosis - pathology; Cystic Fibrosis Transmembrane Conductance Regulator; Cystic Fibrosis Transmembrane Conductance Regulator - genetics; Cystic Fibrosis Transmembrane Conductance Regulator - metabolism; Dose-Response Relationship; Dose-Response Relationship, Drug; Drug; drug effects; Electron; Epithelium; Epithelium - drug effects; Epithelium - metabolism; Epithelium - pathology; Female; genetics; Ileum; Ileum - drug effects; Ileum - metabolism; Ileum - ultrastructure; Immunohistochemistry; In Vitro Techniques; Inbred C57BL; Intestinal Mucosa; Intestinal Mucosa - drug effects; Intestinal Mucosa - metabolism; Intestinal Mucosa - ultrastructure; Intestine; Intestine, Small - drug effects; Intestine, Small - metabolism; Intestine, Small - pathology; Knockout; Male; Medicin och hälsovetenskap; Medicinska grundvetenskaper; metabolism; Mice; Mice, Inbred C57BL; Mice, Knockout; Microscopy; Microscopy, Confocal; Microscopy, Electron, Transmission; Mucins; Mucins - secretion; Mucus; Mucus - drug effects; Mucus - metabolism; pathology; pharmacology; Phenotype; secretion; Small; Transmission; ultrastructure

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 human...