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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Bibliographic Details
Published in:PloS one 2012-06, Vol.7 (6), p.e39809-e39809
Main Authors: 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: Article
Language:English
Subjects:
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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Summary: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