PMID: 8967432May 1, 1996Paper

In vivo analysis of fluid transport in cystic fibrosis airway epithelia of bronchial xenografts

The American Journal of Physiology
Y ZhangJohn F Engelhardt

Abstract

An in vivo human bronchial xenograft model system was used to simultaneously analyze electrolyte and fluid transport defects in fully differentiated human cystic fibrosis (CF) and non-CF proximal airways. CF airways demonstrated three discernible defects when compared with non-CF, including 1) a lack of adenosine 3',5'-cylic monophosphate (cAMP)-inducible Cl- secretion, 2) a fourfold higher basal fluid absorption rate, and 3) an altered regulation of fluid absorption in response to amiloride-stimulated changes in Na+ transport. A unique finding in this study demonstrated that treatment of epithelia with amiloride led to a greater than threefold decrease in the rate of fluid absorption in CF tissues as contrasted to a greater than threefold increase in the rate of fluid absorption in non-CF tissues. The removal of apical Na+ from amiloride-treated non-CF xenografts was capable of ablating this amiloride-induced increase in fluid absorption. In light of the recent interactions demonstrated between CF transmembrane conductance regulator (CFTR) and the rat epithelial, amiloride-sensitive Na+ channel, these findings implicate additional complexities between the Na+ conductance pathways and fluid transport in normal and CF proximal a...Continue Reading

References

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Citations

Sep 14, 1999·The American Journal of Physiology·M MallK Kunzelmann
Jan 25, 2000·American Journal of Physiology. Lung Cellular and Molecular Physiology·F DupuitE Puchelle
Oct 3, 2006·The Journal of Biological Chemistry·Xuefeng SuHong-Long Ji
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Mar 1, 2008·The Journal of Pharmacology and Experimental Therapeutics·Sabrina NoëlFrédéric Becq
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