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Mol Cell. 1998 Sep;2(3):397-403.

Loss of CFTR chloride channels alters salt absorption by cystic fibrosis airway epithelia in vitro.

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1
Department of Internal Medicine, University of Iowa College of Medicine, Iowa City 52242, USA.

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.

PMID:
9774978
[Indexed for MEDLINE]
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