Loss of CFTR Chloride Channels Alters Salt Absorption by Cystic Fibrosis Airway Epithelia In Vitro

Joseph Zabner; Jeffrey J Smith; Philip H Karp; Jonathan H Widdicombe; Michael J Welsh

doi:10.1016/S1097-2765(00)80284-1

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Loss of CFTR Chloride Channels Alters Salt Absorption by Cystic Fibrosis Airway Epithelia In Vitro

Journal article

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Peer reviewed

Loss of CFTR Chloride Channels Alters Salt Absorption by Cystic Fibrosis Airway Epithelia In Vitro

Joseph Zabner, Jeffrey J Smith, Philip H Karp, Jonathan H Widdicombe and Michael J Welsh

Molecular cell, Vol.2(3), pp.397-403

09/1998

DOI: 10.1016/S1097-2765(00)80284-1

PMID: 9774978

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Abstract

Cystic fibrosis (CF) is caused by the loss of functional CFTR Cl− channels. However, it is not understood how this defect disrupts salt and liquid movement in the airway or whether it alters the NaCl concentration in the thin liquid film covering the airway surface. Using a new approach, we found that CF airway surface liquid had a higher NaCl concentration than normal. Both CF and non-CF epithelia absorbed salt and liquid; however, expression of CFTR Cl− channels was required for maximal absorption. Thus, loss of CFTR elevates the salt concentration in CF airway surface liquid and in sweat by related mechanisms; the elevated NaCl concentration is due to a block in transcellular Cl− movement. The high NaCl may predispose CF airways to bacterial infections by inhibiting endogenous antibacterial defenses.

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Title: Subtitle: Loss of CFTR Chloride Channels Alters Salt Absorption by Cystic Fibrosis Airway Epithelia In Vitro
Creators: Joseph Zabner - Department of Internal Medicine, Howard Hughes Medical Institute, University of Iowa College of Medicine, Iowa City, Iowa 52242, USA
Jeffrey J Smith - Department of Pediatrics, Howard Hughes Medical Institute, University of Iowa College of Medicine, Iowa City, Iowa 52242, USA
Philip H Karp - Department of Internal Medicine, Howard Hughes Medical Institute, University of Iowa College of Medicine, Iowa City, Iowa 52242, USA
Jonathan H Widdicombe - Cardiovascular Research Institute, University of California at San Francisco, San Francisco, California 94143, USA
Michael J Welsh - Department of Internal Medicine, Howard Hughes Medical Institute, University of Iowa College of Medicine, Iowa City, Iowa 52242, USA
Resource Type: Journal article
Publication Details: Molecular cell, Vol.2(3), pp.397-403
Publisher: Elsevier Inc
DOI: 10.1016/S1097-2765(00)80284-1
PMID: 9774978
ISSN: 1097-2765
eISSN: 1097-4164
Language: English
Date published: 09/1998
Academic Unit: Neurology; Molecular Physiology and Biophysics; Pulmonary, Critical Care, and Occupational Medicine; Stead Family Department of Pediatrics; Neurosurgery; Internal Medicine
Record Identifier: 9984020626302771

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