Journal article
Chloride and potassium channels in cystic fibrosis airway epithelia
Nature (London), Vol.322(6078), pp.467-470
07/1986
DOI: 10.1038/322467a0
PMID: 2426598
Abstract
Cystic fibrosis, the most common lethal genetic disease in Caucasians, is characterized by a decreased permeability in sweat gland duct and airway epithelia. In sweat duct epithelium, a decreased Cl− permeability accounts for the abnormally increased salt content of sweat1. In airway epithelia a decreased Cl− permeability, and possibly increased sodium absorption, may account for the abnormal respiratory tract fluid2,3. The Cl− impermeability has been localized to the apical membrane of cystic fibrosis airway epithelial cells4. The finding that hormonally regulated Cl− channels make the apical membrane Cl− permeable in normal airway epithelial cells5 suggested abnormal Cl− channel function in cystic fibrosis. Here we report that excised, cell-free patches of membrane from cystic fibrosis epithelial cells contain Cl− channels that have the same conductive properties as Cl− channels from normal cells. However, Cl− channels from cystic fibrosis cells did not open when they were attached to the cell. These findings suggest defective regulation of Cl− channels in cystic fibrosis epithelia; to begin to address this issue, we performed two studies. First, we found that isoprenaline, which stimulates Cl− secretion, increases cellular levels of cyclic AMP in a similar manner in cystic fibrosis and non-cystic fibrosis epithelial cells. Second, we show that adrenergic agonists open calcium-activated potassium channels, indirectly suggesting that calcium-dependent stimulus–response coupling is intact in cystic fibrosis. These data suggest defective regulation of Cl− channels at a site distal to cAMP accumulation.
Details
- Title: Subtitle
- Chloride and potassium channels in cystic fibrosis airway epithelia
- Creators
- Michael J WelshCarole M Liedtke
- Resource Type
- Journal article
- Publication Details
- Nature (London), Vol.322(6078), pp.467-470
- DOI
- 10.1038/322467a0
- PMID
- 2426598
- ISSN
- 0028-0836
- eISSN
- 1476-4687
- Language
- English
- Date published
- 07/1986
- Academic Unit
- Neurology; Molecular Physiology and Biophysics; Pulmonary, Critical Care, and Occupational Medicine; Neurosurgery; Internal Medicine
- Record Identifier
- 9984020784902771
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