Altered fluid transport across airway epithelium in cystic fibrosis

Canwen Jiang, Walter E. Finkbeiner, Jonathan H. Widdicombe, Paul B. McCray, Sneldon S. Miller

Research output: Contribution to journalArticle

177 Citations (Scopus)

Abstract

In cystic fibrosis (CF), absence or dysfunction of a phosphorylation-regulated chloride channel [CF transmembrane conductance regulator (CFTR)] leads to the loss or reduction of chloride secretion into the airways. Active sodium absorption is also increased in CF, and both of these ion transport changes could after fluid transport across the airways. Under baseline conditions, cultured human airway epithelia from normal individuals absorbed fluid, and this absorption was increased in epithelia from patients with CF. In normal and CF epithelial cultures fluid absorption was inhibited by amiloride. Adenosine 3′,5′-monophosphate stimulated fluid secretion in normal epithelial cultures but not in cultures from individuals with CF. In contrast, fluid secretion induced by nucleotide triphosphates (uridine triphosphate or adenosine triphosphate) was unaltered in cultures of epithelia from patients with CF, suggesting an approach to the treatment of CF.

Original languageEnglish (US)
Pages (from-to)424-427
Number of pages4
JournalScience
Volume262
Issue number5132
StatePublished - 1993

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Cystic Fibrosis
Epithelium
Fluids and Secretions
Cystic Fibrosis Transmembrane Conductance Regulator
Uridine Triphosphate
Chloride Channels
Amiloride
Ion Transport
Adenosine
Chlorides
Nucleotides
Adenosine Triphosphate
Sodium
Phosphorylation

ASJC Scopus subject areas

  • General

Cite this

Jiang, C., Finkbeiner, W. E., Widdicombe, J. H., McCray, P. B., & Miller, S. S. (1993). Altered fluid transport across airway epithelium in cystic fibrosis. Science, 262(5132), 424-427.

Altered fluid transport across airway epithelium in cystic fibrosis. / Jiang, Canwen; Finkbeiner, Walter E.; Widdicombe, Jonathan H.; McCray, Paul B.; Miller, Sneldon S.

In: Science, Vol. 262, No. 5132, 1993, p. 424-427.

Research output: Contribution to journalArticle

Jiang, C, Finkbeiner, WE, Widdicombe, JH, McCray, PB & Miller, SS 1993, 'Altered fluid transport across airway epithelium in cystic fibrosis', Science, vol. 262, no. 5132, pp. 424-427.
Jiang C, Finkbeiner WE, Widdicombe JH, McCray PB, Miller SS. Altered fluid transport across airway epithelium in cystic fibrosis. Science. 1993;262(5132):424-427.
Jiang, Canwen ; Finkbeiner, Walter E. ; Widdicombe, Jonathan H. ; McCray, Paul B. ; Miller, Sneldon S. / Altered fluid transport across airway epithelium in cystic fibrosis. In: Science. 1993 ; Vol. 262, No. 5132. pp. 424-427.
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