Abnormal ion content, hydration and granule expansion of the secretory granules from cystic fibrosis airway glandular cells
| Autor: | Vincent Banchet, Noël Bonnet, Gérard Balossier, Jean Michel, J.M. Zahm, E. Puchelle, S. Baconnais, Karl Zierold, F. Delavoi, Christine Terryn, Nicolas Castillon, Olivier Danos, Marc Merten, Magali Milliot, T. Chinet |
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| Přispěvatelé: | Interfaces biomatériaux/tissus hôtes, Université de Reims Champagne-Ardenne (URCA)-IFR53-Institut National de la Santé et de la Recherche Médicale (INSERM), Dynamique cellulaire et moléculaire de la muqueuse respiratoire, Centre de recherche et d'applications sur les thérapies géniques (CRATG), Université d'Évry-Val-d'Essonne (UEVE)-Centre National de la Recherche Scientifique (CNRS)-Généthon, Biologie Cellulaire et Moleculaire du Transport des Nutriments, Université Henri Poincaré - Nancy 1 (UHP)-Institut National de la Santé et de la Recherche Médicale (INSERM), Laboratoire de Biologie et Pharmacologie des Epithéliums Respiratoires (LBPER), Université Paris Descartes - Paris 5 (UPD5), Laboratory for Analytical Microscopy, Max-Planck-Institut, Birembaut, Philippe, Université d'Évry-Val-d'Essonne (UEVE)-Généthon-Centre National de la Recherche Scientifique (CNRS) |
| Jazyk: | angličtina |
| Rok vydání: | 2005 |
| Předmět: |
Cytoplasm
Cystic Fibrosis MESH: Secretory Vesicles MESH: Cystic Fibrosis Cystic Fibrosis Transmembrane Conductance Regulator Respiratory Mucosa Cystic fibrosis [SDV.MHEP.PSR]Life Sciences [q-bio]/Human health and pathology/Pulmonology and respiratory tract Exocytosis 03 medical and health sciences 0302 clinical medicine Exocrine Glands Chlorides Fibrosis MESH: Respiratory Mucosa medicine MESH: Water Humans Microscopy Phase-Contrast MESH: Cell Line Transformed MESH: Chlorides Magnesium ion 030304 developmental biology Cell Line Transformed MESH: Microscopy Phase-Contrast MESH: Cystic Fibrosis Transmembrane Conductance Regulator 0303 health sciences MESH: Humans biology Secretory Vesicles MESH: Cytoplasm Granule (cell biology) Water Cell Biology Anatomy Glandular Cell medicine.disease Molecular biology Cystic fibrosis transmembrane conductance regulator Trachea 030228 respiratory system Cell culture MESH: Exocrine Glands biology.protein [SDV.MHEP.PSR] Life Sciences [q-bio]/Human health and pathology/Pulmonology and respiratory tract MESH: Trachea |
| Zdroj: | Experimental Cell Research Experimental Cell Research, 2005, 309 (2), pp.296-304. ⟨10.1016/j.yexcr.2005.06.010⟩ Experimental Cell Research, Elsevier, 2005, 309 (2), pp.296-304. ⟨10.1016/j.yexcr.2005.06.010⟩ |
| ISSN: | 0014-4827 1090-2422 |
| DOI: | 10.1016/j.yexcr.2005.06.010⟩ |
| Popis: | The absence or decreased expression of cystic fibrosis transmembrane conductance regulator (CFTR) induces increased Na{sup +} absorption and hyperabsorption of the airway surface liquid (ASL) resulting in a dehydrated and hyperviscous ASL. Although the implication of abnormal airway submucosal gland function has been suggested, the ion and water content in the Cystic Fibrosis (CF) glandular secretory granules, before exocytosis, is unknown. We analyzed, in non-CF and CF human airway glandular cell lines (MM-39 and KM4, respectively), the ion content in the secretory granules by electron probe X-ray microanalysis and the water content by quantitative dark field imaging on freeze-dried cryosections. We demonstrated that the ion content (Na{sup +}, Mg{sup 2+}, P, S and Cl{sup -}) is significantly higher and the water content significantly lower in secretory granules from the CF cell line compared to the non-CF cell line. Using videomicroscopy, we observed that the secretory granule expansion was deficient in CF glandular cells. Transfection of CF cells with CFTR cDNA or inhibition of non-CF cells with CFTR{sub inh}-172, respectively restored or decreased the water content and granule expansion, in parallel with changes in ion content. We hypothesize that the decreased water and increased ion content in glandular secretory granules maymore » contribute to the dehydration and increased viscosity of the ASL in CF.« less |
| Databáze: | OpenAIRE |
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