Ion and fluid transport properties of small airways in cystic fibrosis
| Autor: | Emmanuel Naline, Sabine Blouquit, Luc Dannhoffer, Richard C. Boucher, Thierry Chinet, Agathe Regnier, Christophe Fermanian |
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| Jazyk: | angličtina |
| Rok vydání: | 2006 |
| Předmět: |
Pulmonary and Respiratory Medicine
Pathology medicine.medical_specialty Bronchiole Cystic Fibrosis Bronchi Critical Care and Intensive Care Medicine chemistry.chemical_compound Body Water Chlorides Reference Values Intensive care Electric Impedance Humans Medicine Cells Cultured Ion transporter Ion Transport Microscopy Confocal Lung Forskolin biology business.industry Colforsin Epithelial Cells Middle Aged respiratory system Fluid transport Cystic fibrosis transmembrane conductance regulator Amiloride respiratory tract diseases medicine.anatomical_structure chemistry biology.protein business medicine.drug |
| DOI: | 10.17615/539v-7s95 |
| Popis: | Rationale: Small airways constitute amajor site of pathology in cystic fibrosis (CF) and provide most of the surface area of the conducting airways of the lung. Little is known, however, about the impact of CF on ion and fluid transport in small (bronchiolar) airways. Objectives: To describe the ion and fluid transport properties of CF bronchiolar epithelium. Methods: Primary cultures of human bronchial and bronchiolar (non-CF and CF) epithelial cells were obtained. The bioelectric properties were studied in Ussing chambers and the airway surface liquid (ASL) height was measured with confocal microscopy. Main Results: Primary cultures of ΔF508 CF bronchiolar epithelial cells displayed higher transepithelial resistance than non-CF cultures, whereas baseline short circuit current and amiloride-inhibitable short circuit current were similar in both preparations. The ASL height was significantly smaller in CF compared with non-CF preparations. In the presence of amiloride, addition of forskolin increased short circuit current in non-CF but not in CF bronchiolar cultures, and the ATP-induced increase in short circuit current was lower in CF than in non-CF cultures. Non-CF bronchiolar preparations displayed larger short circuit current and fluid secretion in responses to forskolin than non-CF bronchial preparations, suggesting that CFTR-dependent Cl- transport may play a more important role in the regulation of fluid transport in small airways than in large airways. Conclusion: In CF small airways, defective Cl- secretion combined with unregulated (persistent) Na+ absorption results in ASLdepletion. |
| Databáze: | OpenAIRE |
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