DOI: 10.1126/science.6308769. Abnormal Ion Permeation Through Cystic Fibrosis Respiratory Epithelium

Abnormal Ion Permeation Through Cystic Fibrosis Respiratory Epithelium

10.1126/science.6308769

Crossref journal-article

American Association for the Advancement of Science (AAAS)

Science (221)

Abstract

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.

Bibliography

Knowles, M. R., Stutts, M. J., Spock, A., Fischer, N., Gatzy, J. T., & Boucher, R. C. (1983). Abnormal Ion Permeation Through Cystic Fibrosis Respiratory Epithelium. Science, 221(4615), 1067â1070.

Authors 6

M. R. Knowles (first)
M. J. Stutts (additional)
A. Spock (additional)
N. Fischer (additional)
J. T. Gatzy (additional)
R. C. Boucher (additional)

References 15 Referenced 349

BOUCHER, R.C., FLUID ELECTROLYTE AB: 271 (1982). / FLUID ELECTROLYTE AB (1982)
BOUCHER, R, J APPL PHYSL 51: 206 (1981). / J APPL PHYSL (1981)
CUTHBERT, A.W., DRUGS TRANSPORT PROC: 172 (1974). / DRUGS TRANSPORT PROC (1974)
GATZY, J.T., PATHS OF HYDROPHILIC SOLUTE FLOW ACROSS EXCISED CANINE AIRWAYS, FEDERATION PROCEEDINGS 41: 1244 (1982). / FEDERATION PROCEEDINGS (1982)
GATZY, J.T., ION-TRANSPORT ACROSS EXCISED BULLFROG LUNG, AMERICAN JOURNAL OF PHYSIOLOGY 228: 1162 (1975). (10.1152/ajplegacy.1975.228.4.1162) / AMERICAN JOURNAL OF PHYSIOLOGY (1975)
KILBURN, K.H., A HYPOTHESIS FOR PULMONARY CLEARANCE AND ITS IMPLICATIONS, AMERICAN REVIEW OF RESPIRATORY DISEASE 98: 449 (1968). / AMERICAN REVIEW OF RESPIRATORY DISEASE (1968)
KNOWLES, M.R., ION-TRANSPORT IN EXCISED HUMAN BRONCHI AND ITS NEUROHUMORAL CONTROL, CHEST 81: S11 (1982). / CHEST (1982)
KNOWLES, M, AMILORIDE SENSITIVITY AND ION PERMEABILITIES OF CYSTIC-FIBROSIS AND NORMAL HUMAN BRONCHI, CLINICAL RESEARCH 30: A861 (1982). / CLINICAL RESEARCH (1982)
KNOWLES, M, RELATIVE ION PERMEABILITY OF NORMAL AND CYSTIC-FIBROSIS NASAL EPITHELIUM, JOURNAL OF CLINICAL INVESTIGATION 71: 1410 (1983). (10.1172/JCI110894) / JOURNAL OF CLINICAL INVESTIGATION (1983)
KNOWLES, M, INCREASED BIOELECTRIC POTENTIAL DIFFERENCE ACROSS RESPIRATORY EPITHELIA IN CYSTIC-FIBROSIS, NEW ENGLAND JOURNAL OF MEDICINE 305: 1489 (1981). (10.1056/NEJM198112173052502) / NEW ENGLAND JOURNAL OF MEDICINE (1981)
LEGRIS, G, ANN NY ACAD SCI 372: 345 (1981). (10.1111/j.1749-6632.1981.tb15485.x) / ANN NY ACAD SCI (1981)
OPPENHEIMER, E.H., DIFFERENTIAL PATHOLOGY OF NASAL POLYPS IN CYSTIC-FIBROSIS AND ATOPY, LABORATORY INVESTIGATION 40: 445 (1979). / LABORATORY INVESTIGATION (1979)
QUINTON, P.M., CHLORIDE IMPERMEABILITY IN CYSTIC-FIBROSIS, NATURE 301: 421 (1983). (10.1038/301421a0) / NATURE (1983)
SCHULZ, I.J., MICROPUNCTURE STUDIES OF SWEAT FORMATION IN CYSTIC FIBROSIS PATIENTS, JOURNAL OF CLINICAL INVESTIGATION 48: 1470 (1969). (10.1172/JCI106113) / JOURNAL OF CLINICAL INVESTIGATION (1969)
WOOD, R.E., CYSTIC-FIBROSIS, AMERICAN REVIEW OF RESPIRATORY DISEASE 113: 833 (1976). / AMERICAN REVIEW OF RESPIRATORY DISEASE (1976)

Dates

Type	When
Created	18 years, 10 months ago (Oct. 5, 2006, 2:37 p.m.)
Deposited	1 year, 7 months ago (Jan. 14, 2024, 5:05 p.m.)
Indexed	2 months, 1 week ago (June 27, 2025, 6:14 a.m.)
Issued	41 years, 11 months ago (Sept. 9, 1983)
Published	41 years, 11 months ago (Sept. 9, 1983)
Published Print	41 years, 11 months ago (Sept. 9, 1983)

Funders 0

None

BibTeX

@article{Knowles_1983, title={Abnormal Ion Permeation Through Cystic Fibrosis Respiratory Epithelium}, volume={221}, ISSN={1095-9203}, url={http://dx.doi.org/10.1126/science.6308769}, DOI={10.1126/science.6308769}, number={4615}, journal={Science}, publisher={American Association for the Advancement of Science (AAAS)}, author={Knowles, M. R. and Stutts, M. J. and Spock, A. and Fischer, N. and Gatzy, J. T. and Boucher, R. C.}, year={1983}, month=sep, pages={1067–1070} }

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