Bicarbonate and functional CFTR channel are required for proper mucin secretion and link cystic fibrosis with its mucus phenotype.
Artikel i vetenskaplig tidskrift, 2012

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.

metabolism

metabolism

Mucus

metabolism

Bicarbonates

Intestinal Mucosa

Mice

Transmission

Drug

Inbred C57BL

Dose-Response Relationship

Intestine

Epithelium

drug effects

metabolism

Mucins

Confocal

Animals

pathology

Mice

drug effects

Female

pathology

Electron

Cystic Fibrosis Transmembrane Conductance Regulator

drug effects

Knockout

Male

ultrastructure

pharmacology

metabolism

pathology

Small

drug effects

drug effects

Phenotype

metabolism

Microscopy

Ileum

Immunohistochemistry

Mice

secretion

ultrastructure

metabolism

genetics

genetics

Microscopy

Cystic Fibrosis

Författare

Jenny K Gustafsson

Göteborgs universitet

Anna Ermund

Göteborgs universitet

Daniel Ambort

Göteborgs universitet

Malin E V Johansson

Göteborgs universitet

Harriet E Nilsson

Kaisa Thorell

Göteborgs universitet

Hans Hebert

Henrik Sjövall

Göteborgs universitet

Gunnar C. Hansson

Göteborgs universitet

The Journal of experimental medicine

1540-9538 (ISSN)

Vol. 209 1263-72

Ämneskategorier

Medicinska grundvetenskaper

Cell- och molekylärbiologi

DOI

10.1084/jem.20120562

PubMed

22711878