Display Settings:

Format

Send to:

Choose Destination
Int J Biochem Cell Biol. 2012 Aug;44(8):1232-5. doi: 10.1016/j.biocel.2012.04.012. Epub 2012 Apr 21.

Airway epithelial cells--hyperabsorption in CF?

Author information

  • 1Institut für Physiologie, Universität Regensburg, Universitätsstraße 31, D-93053 Regensburg, Germany. karl.kunzelmann@vkl.uni-regensburg.de

Abstract

Airway epithelial cells transport electrolytes and are central to the disease cystic fibrosis (CF), which is an inherited transport defect affecting smaller airways and a number of other epithelial organs. Clinically, CF is dominated by a chronic lung disease, the main cause of morbidity and mortality. Airway obstruction by thick mucus and chronic infection by Pseudomonas aeruginosa eventually lead to loss of pulmonary function. Loss of function of CFTR Cl(-) channels was found to be the cause for CF. However, intensive research on the detailed mechanism of CF lung disease for more than 25 years produced a bewildering number of hypotheses and an endless discussion whether reduced Cl(-) secretion, primarily located in airway submucosal glands, or dehydration of the airways, driven by a hyperabsorption of Na(+) ions, is the primary cause of the disease. Recent results suggest a fine-tuned regulation of the airway fluid layer, but how significant really are Cl(-) and Na(+) transport?

Copyright © 2012 Elsevier Ltd. All rights reserved.

PMID:
22542896
[PubMed - indexed for MEDLINE]
PubMed Commons home

PubMed Commons

0 comments
How to join PubMed Commons

    Supplemental Content

    Full text links

    Icon for Elsevier Science
    Loading ...
    Write to the Help Desk