scholarly journals Measuring Airway Surface Liquid Depth in Ex Vivo Mouse Airways by X-Ray Imaging for the Assessment of Cystic Fibrosis Airway Therapies

PLoS ONE ◽  
2013 ◽  
Vol 8 (1) ◽  
pp. e55822 ◽  
Author(s):  
Kaye S. Morgan ◽  
Martin Donnelley ◽  
David M. Paganin ◽  
Andreas Fouras ◽  
Naoto Yagi ◽  
...  
2020 ◽  
Vol 598 (19) ◽  
pp. 4307-4320 ◽  
Author(s):  
Ian M. Thornell ◽  
Tayyab Rehman ◽  
Alejandro A. Pezzulo ◽  
Michael J. Welsh

2020 ◽  
Vol 21 (4) ◽  
pp. 1488 ◽  
Author(s):  
Ambra Gianotti ◽  
Valeria Capurro ◽  
Livia Delpiano ◽  
Marcin Mielczarek ◽  
María García-Valverde ◽  
...  

Cystic fibrosis (CF) is a genetic disease characterized by the lack of cystic fibrosis transmembrane conductance regulator (CFTR) protein expressed in epithelial cells. The resulting defective chloride and bicarbonate secretion and imbalance of the transepithelial homeostasis lead to abnormal airway surface liquid (ASL) composition and properties. The reduced ASL volume impairs ciliary beating with the consequent accumulation of sticky mucus. This situation prevents the normal mucociliary clearance, favouring the survival and proliferation of bacteria and contributing to the genesis of CF lung disease. Here, we have explored the potential of small molecules capable of facilitating the transmembrane transport of chloride and bicarbonate in order to replace the defective transport activity elicited by CFTR in CF airway epithelia. Primary human bronchial epithelial cells obtained from CF and non-CF patients were differentiated into a mucociliated epithelia in order to assess the effects of our compounds on some key properties of ASL. The treatment of these functional models with non-toxic doses of the synthetic anionophores improved the periciliary fluid composition, reducing the fluid re-absorption, correcting the ASL pH and reducing the viscosity of the mucus, thus representing promising drug candidates for CF therapy.


2003 ◽  
Vol 168 (10) ◽  
pp. 1223-1226 ◽  
Author(s):  
Peter G. Middleton ◽  
Katy A. Pollard ◽  
Elizabeth Donohoo ◽  
John R. Wheatley ◽  
Duncan M. Geddes ◽  
...  

2001 ◽  
Vol 281 (2) ◽  
pp. L309-L313 ◽  
Author(s):  
Jean-Marie Zahm ◽  
Sonia Baconnais ◽  
Donald J. Davidson ◽  
Sheila Webb ◽  
Julia Dorin ◽  
...  

The airway surface liquid (ASL) that lines the airway surface epithelium plays a major role in airway antibacterial defense and mucociliary transport efficiency, two key factors in cystic fibrosis (CF) disease. A major difficulty is to collect ASL in native conditions without stimulation or alteration of the underlying airway epithelium. Using a cryoprobe specifically adapted to collect native ASL from the tracheal mouse surface, we analyzed by X-ray microanalysis the complete ASL and plasma ion content in Cftrtm1Hgu/Cftrtm1Hgu mice compared with that in control littermates. ASL ion content from eight Cftrtm1Hgu/Cftrtm1Hgu mice and eight control littermates did not appear significantly different. The mean (±SE) concentrations were 2,352 ± 367 and 2,058 ± 401 mmol/kg dry weight for Na, 1,659 ± 272 and 1,448 ± 281 mmol/kg dry weight for Cl, 357 ± 57 and 337 ± 38 mmol/kg dry weight for S, 1,066 ± 220 and 787 ± 182 mmol/kg dry weight for K, 400 ± 82 and 301 ± 58 mmol/kg dry weight for Ca, 105 ± 31 and 105 ± 20 mmol/kg dry weight for Mg, 33 ± 15 and 29 ± 9 mmol/kg dry weight for P in non-CF and CF mice, respectively. This cryotechnique appears to be a promising technique for analyzing the complete elemental composition of native ASL in CF and non-CF tissues.


2006 ◽  
Vol 69 (4) ◽  
pp. 271-276 ◽  
Author(s):  
V. Vanthanouvong ◽  
I. Kozlova ◽  
M. Johannesson ◽  
E. Nääs ◽  
S.L. Nordvall ◽  
...  

2005 ◽  
Vol 280 (42) ◽  
pp. 35751-35759 ◽  
Author(s):  
Robert Tarran ◽  
Brian Button ◽  
Maryse Picher ◽  
Anthony M. Paradiso ◽  
Carla M. Ribeiro ◽  
...  

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