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Type: Journal article
Title: The CF salt controversy: In vivo observations and therapeutic approaches
Author: Tarran, R.
Grubb, B.
Parsons, D.
Picher, M.
Hirsch, A.
Davis, C.
Boucher, R.
Citation: Molecular Cell, 2001; 8(1):149-158
Publisher: Cell Press
Issue Date: 2001
ISSN: 1097-2765
Abstract: There is controversy over whether abnormalities in the salt concentration or volume of airway surface liquid (ASL) initiate cystic fibrosis (CF) airway disease. In vivo studies of CF mouse nasal epithelia revealed an increase in goblet cell number that was associated with decreased ASL volume rather than abnormal [Cl−]. Aerosolization of osmolytes in vivo failed to raise ASL volume. In vitro studies revealed that osmolytes and pharmacological agents were effective in producing isotonic volume responses in human airway epithelia but were typically short acting and less effective in CF cultures with prolonged volume hyperabsorption and mucus accumulation. These data show that (1) therapies can be designed to normalize ASL volume, without producing deleterious compositional changes in ASL, and (2) therapeutic efficacy will likely depend on development of long-acting pharmacologic agents and/or an increased efficiency of osmolyte delivery.
Keywords: Goblet Cells
Nasal Mucosa
Respiratory Mucosa
Cells, Cultured
Mice, Inbred C57BL
Cystic Fibrosis
Disease Models, Animal
Sodium Chloride
Uridine Triphosphate
Pulmonary Surfactants
Microscopy, Confocal
Ion Transport
Osmolar Concentration
Rights: Copyright © 2001 Cell Press. All rights reserved.
DOI: 10.1016/S1097-2765(01)00286-6
Appears in Collections:Aurora harvest
Paediatrics publications

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