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

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

Research output: Contribution to journalArticle

184 Scopus citations


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 CFand 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.

Original languageEnglish (US)
Pages (from-to)397-403
Number of pages7
JournalMolecular Cell
Issue number3
StatePublished - Sep 1998
Externally publishedYes


ASJC Scopus subject areas

  • Molecular Biology

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