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Science 9 September 1983: Vol. 221. no. 4615, pp. 1067 - 1070 DOI: 10.1126/science.6308769
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Articles
Science, Vol 221, Issue 4615, 1067-1070
Copyright © 1983 by American Association for the Advancement of Science
Abnormal ion permeation through cystic fibrosis respiratory epithelium
MR Knowles,
MJ Stutts,
A Spock,
N Fischer,
JT Gatzy,
and
RC Boucher
The epithelium of nasal tissue excised from subjects with cystic fibrosis exhibited higher voltage and lower conductance than tissue from control subjects. Basal sodium ion absorption by cystic fibrosis and normal nasal epithelia equaled the short-circuit current and was amiloride-sensitive. Amiloride induced chloride ion secretion in normal but not cystic fibrosis tissue and consequently was more effective in inhibiting the short-circuit current in cystic fibrosis epithelia. Chloride ion-free solution induced a smaller hyperpolarization of cystic fibrosis tissue. The increased voltage and amiloride efficacy in cystic fibrosis reflect absorption of sodium ions across an epithelium that is relatively impermeable to chloride ions.
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