A pharmacologic approach to acquired cystic fibrosis transmembrane conductance regulator dysfunction in smoking related lung disease

Mucus stasis in chronic obstructive pulmonary disease (COPD) is a significant contributor to morbidity and mortality. Potentiators of cystic fibrosis transmembrane conductance regulator (CFTR) activity pharmacologically enhance CFTR function; ivacaftor is one such agent approved to treat CF patients...

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Veröffentlicht in:	PloS one 2012-06, Vol.7 (6), p.e39809-e39809
Hauptverfasser:	Sloane, Peter A, Shastry, Suresh, Wilhelm, Andrew, Courville, Clifford, Tang, Li Ping, Backer, Kyle, Levin, Elina, Raju, S Vamsee, Li, Yao, Mazur, Marina, Byan-Parker, Suzanne, Grizzle, William, Sorscher, Eric J, Dransfield, Mark T, Rowe, Steven M
Format:	Artikel
Sprache:	eng
Schlagworte:	Adult Aged Aged, 80 and over Aminophenols - pharmacology Biology Biophysics Bronchitis Chloride transport Chlorides Chronic obstructive pulmonary disease Cigarette smoke Cigarette smoking Cigarettes Cough Critical care Cystic fibrosis Cystic Fibrosis Transmembrane Conductance Regulator - metabolism Demography Epithelial cells Epithelial Cells - drug effects Epithelial Cells - metabolism Epithelial Cells - pathology Female Fibrosis Gating Health aspects Humans Ion Transport - drug effects Lung diseases Lung Diseases - metabolism Lung Diseases - physiopathology Male Medicine Membrane Potentials - drug effects Middle Aged Morbidity Mucus Mucus - drug effects Mucus - metabolism Mutation Obstructive lung disease Pathology Patients Pharmacology Physiology Quinolones - pharmacology Resistance Respiratory tract Smoke Smoking Smoking - metabolism Smoking - physiopathology Sputum Studies Transport
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Beschreibung
Zusammenfassung:	Mucus stasis in chronic obstructive pulmonary disease (COPD) is a significant contributor to morbidity and mortality. Potentiators of cystic fibrosis transmembrane conductance regulator (CFTR) activity pharmacologically enhance CFTR function; ivacaftor is one such agent approved to treat CF patients with the G551D-CFTR gating mutation. CFTR potentiators may also be useful for other diseases of mucus stasis, including COPD. In primary human bronchial epithelial cells, exposure to cigarette smoke extract diminished CFTR-mediated anion transport (65.8±0.2% of control, P
ISSN:	1932-6203 1932-6203
DOI:	10.1371/journal.pone.0039809