Zobrazeno 1 - 10 of 27 pro vyhledávání: '"Cystic Fibrosis/pathology"'
1
ENaC-mediated sodium influx exacerbates NLRP3-dependent inflammation in cystic fibrosis
Autor: Daniel Peckham, Fabio Martinon, C. Wong, Heledd H. Jarosz-Griffiths, Shelly Pathak, Samuel Lara-Reyna, Sinisa Savic, T. Scambler, Michael F. McDermott, J. Holbrook
Publikováno v: eLife, vol. 8
eLife, Vol 8 (2019)
eLife
Cystic Fibrosis (CF) is a monogenic disease caused by mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene, resulting in defective CFTR-mediated chloride and bicarbonate transport, with dysregulation of epithelial sodium c
Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::ae263e191a20b0d408b31ae8aa082153
https://doi.org/10.7554/elife.49248
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2
In vitro and in vivo evidence for an inflammatory role of the calcium channel TRPV4 in lung epithelium: Potential involvement in cystic fibrosis
Autor: Rainer Kiefmann, Lhousseine Touqui, Marie Potier-Cartereau, Christelle Coraux, Cristina Plata, Clémence Henry, Eric Morello, Mustapha Si-Tahar, Antoine Guillon, Emilie Dalloneau, Christophe Vandier, Rose-France Aimar, Maria-Teresa Pérez-Berezo, Frédéric Esnard, Nicolas Cenac, Yongzheng Wu, Miguel A. Valverde, Christian Börnchen
Publikováno v: American Journal of Physiology-Lung Cellular and Molecular Physiology
American Journal of Physiology-Lung Cellular and Molecular Physiology, American Physiological Society, 2016, 311 (3), pp.L664-L675. ⟨10.1152/ajplung.00442.2015⟩
American Journal of Physiology-Lung Cellular and Molecular Physiology, 2016, 311 (3), pp.L664-L675. ⟨10.1152/ajplung.00442.2015⟩
Cystic fibrosis (CF) is an inherited disease associated with chronic severe lung inflammation, leading to premature death. To develop innovative anti-inflammatory treatments, we need to characterize new cellular and molecular components contributing
Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::ca96eb875d5e73f302cf82f923967ddb
https://hal.univ-reims.fr/hal-02448649
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3
Airway surface liquid volume regulation determines different airway phenotypes in liddle compared with betaENaC-overexpressing mice
Autor: Ray A. Caldwell, Alessandra Livraghi, Brian Button, Susanne C. Schubert, Bernard C. Rossier, Edith Hummler, Zhe Zhou, Marcus A. Mall, Sylvain Pradervand, Richard C. Boucher, Wanda K. O'Neal, Barbara R. Grubb, Bjarki Johannesson, Carsten Schultz
Publikováno v: Journal of Biological Chemistry, vol. 285, no. 35, pp. 26945-26955
The Journal of Biological Chemistry
Studies in cystic fibrosis patients and mice overexpressing the epithelial Na(+) channel beta-subunit (betaENaC-Tg) suggest that raised airway Na(+) transport and airway surface liquid (ASL) depletion are central to the pathogenesis of cystic fibrosi
Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::baab53229adfb1f253ead965b9cc11d7
https://serval.unil.ch/notice/serval:BIB_44A5A1B88EA4
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4
Dysfunction of mitochondria Ca2+ uptake in cystic fibrosis airway epithelial cells
Autor: Maud Frieden, Dorothée Raveau, Clarisse Vandebrouck, Fabrice Antigny, Frédéric Becq, Nathalie C Girardin
Publikováno v: Mitochondrion
Mitochondrion, Elsevier, 2009, 9 (4), pp.232-241. ⟨10.1016/j.mito.2009.02.003⟩
Mitochondrion, Vol. 9, No 4 (2009) pp. 232-41
(IF : 3,209); International audience; In the genetic disease cystic fibrosis (CF), the most common mutation F508del promotes the endoplasmic reticulum (ER) retention of misfolded CF proteins. Furthermore, in homozygous F508del-CFTR airway epithelial
Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::b8d3557c638af43919e8ae92dd37b331
https://hal.archives-ouvertes.fr/hal-00391081
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6
Discrimination between cystic fibrosis and CFTR-corrected epithelial cells by a membrane potential-sensitive probe
Autor: Serge Poitry, Marc Chanson, Thierry Rochat, Joelle Coclet-Ninin
Publikováno v: Experimental Lung Research, Vol. 28, No 3 (2002) pp. 181-99
Methods to detect functional cystic fibrosis transmembrane conductance regulator (CFTR) are needed for the assessment of new therapies in cystic fibrosis (CF). We have combined patch-clamp and fluorimetric techniques to investigate whether the fluore
Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::bcc43357b7bcac22a2f22c781f8ce1a7
https://archive-ouverte.unige.ch/unige:27039
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7
Lung disease in mice with cystic fibrosis
Autor: Cameron Ackerley, Geraldine Kent, R. Iles, Danuta Radzioch, Uta Griesenbach, Manuel Buchwald, A. K. Tanswell, Christine E. Bear, Lap-Chee Tsui, Colin McKerlie, Hugh O'Brodovich, L J Huan, Diane Gosselin, Helena Frndova
Publikováno v: Scopus-Elsevier
The leading cause of mortality and morbidity in humans with cystic fibrosis is lung disease. Advances in our understanding of the pathogenesis of the lung disease of cystic fibrosis, as well as development of innovative therapeutic interventions, hav
Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::78b49fa2e494962da913295c1a152d58
https://europepmc.org/articles/PMC508519/
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8
Akademický článek
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9
[MicroRNA control biosynthesis of motile cilia in vertebrates]
Autor: Chevalier B, Kodjabachian L, Coraux C, Pascal Barbry, Marcet B
Publikováno v: Europe PubMed Central
médecine/sciences
médecine/sciences, 2011, 27 (6-7), pp.671-673. ⟨10.1051/medsci/2011276022⟩
médecine/sciences, EDP Sciences, 2011, 27 (6-7), pp.671-673. ⟨10.1051/medsci/2011276022⟩
médecine/sciences, EDP Sciences, 2011, 27 (6-7), pp.671-3. ⟨10.1051/medsci/2011276022⟩
médecine/sciences, 2011, 27 (6-7), pp.671-3. ⟨10.1051/medsci/2011276022⟩
International audience
Externí odkaz: https://explore.openaire.eu/search/publication?articleId=dedup_wf_001::56a3f77c005ec27323decbf9d7b9ce7b
http://europepmc.org/abstract/med/21718654
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10
Akademický článek
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