The Cystic Fibrosis Transmembrane Conductance Regulator Potentiator Ivacaftor Augments Mucociliary Clearance Abrogating Cystic Fibrosis Transmembrane Conductance Regulator Inhibition by Cigarette Smoke
| العنوان: | The Cystic Fibrosis Transmembrane Conductance Regulator Potentiator Ivacaftor Augments Mucociliary Clearance Abrogating Cystic Fibrosis Transmembrane Conductance Regulator Inhibition by Cigarette Smoke |
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| المؤلفون: | Limbo Liu, John C. Kappes, Peter A. Sloane, Liping Tang, Marina Mazur, Carmel M. McNicholas, Kevin Macon, Lawrence J. DeLucas, Stephen Barnes, Wei Wang, Guillermo J. Tearney, Landon Wilson, Patricia L. Jackson, Suman Karki, Kevin L. Kirk, Steven M. Rowe, S. Vamsee Raju, Vivian Y. Lin |
| المصدر: | American Journal of Respiratory Cell and Molecular Biology. 56:99-108 |
| بيانات النشر: | American Thoracic Society, 2017. |
| سنة النشر: | 2017 |
| مصطلحات موضوعية: | 0301 basic medicine, Pulmonary and Respiratory Medicine, Mucociliary clearance, Clinical Biochemistry, Cystic Fibrosis Transmembrane Conductance Regulator, Bronchi, Quinolones, Pharmacology, Aminophenols, Ivacaftor, Pathogenesis, 03 medical and health sciences, 0302 clinical medicine, parasitic diseases, medicine, Humans, Amino Acid Sequence, Cilia, Acrolein, Molecular Biology, Cells, Cultured, Ion transporter, Original Research, Mucous Membrane, biology, Chemistry, Cilium, Smoking, Mucous membrane, Epithelial Cells, Cell Biology, respiratory system, Potentiator, digestive system diseases, Cystic fibrosis transmembrane conductance regulator, respiratory tract diseases, Trachea, 030104 developmental biology, medicine.anatomical_structure, 030228 respiratory system, Mucociliary Clearance, Immunology, biology.protein, Ion Channel Gating, Tomography, Optical Coherence, medicine.drug |
| الوصف: | Acquired cystic fibrosis transmembrane conductance regulator (CFTR) dysfunction may contribute to chronic obstructive pulmonary disease pathogenesis and is a potential therapeutic target. We sought to determine the acute effects of cigarette smoke on ion transport and the mucociliary transport apparatus, their mechanistic basis, and whether deleterious effects could be reversed with the CFTR potentiator ivacaftor (VX-770). Primary human bronchial epithelial (HBE) cells and human bronchi were exposed to cigarette smoke extract (CSE) and/or ivacaftor. CFTR function and expression were measured in Ussing chambers and by surface biotinylation. CSE-derived acrolein modifications on CFTR were determined by mass spectroscopic analysis of purified protein, and the functional microanatomy of the airway epithelia was measured by 1-μm resolution optical coherence tomography. CSE reduced CFTR-dependent current in HBE cells (P |
| تدمد: | 1535-4989 1044-1549 |
| URL الوصول: | https://explore.openaire.eu/search/publication?articleId=doi_dedup___::715d68d894e7d983a58dd765c6d03b1e https://doi.org/10.1165/rcmb.2016-0226oc |
| حقوق: | OPEN |
| رقم الأكسشن: | edsair.doi.dedup.....715d68d894e7d983a58dd765c6d03b1e |
| قاعدة البيانات: | OpenAIRE |
| تدمد: | 15354989 10441549 |
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