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Low free drug concentration prevents inhibition of F508del CFTR functional expression by the potentiator VX-770 (ivacaftor)
Elizabeth Matthes et al.
BRITISH JOURNAL OF PHARMACOLOGY (2016)
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Emanuela M. Bruscia et al.
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Cystic Fibrosis Lung Immunity: The Rose of the Macrophage
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J. Stuart Elborn et al.
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Lumacaftor-Ivacaftor in Patients with Cystic Fibrosis Homozygous for Phe508del CFTR
C. E. Wainwright et al.
NEW ENGLAND JOURNAL OF MEDICINE (2015)
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Kelly Kuk et al.
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Cystic fibrosis genetics: from molecular understanding to clinical application
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Pseudomonas aeruginosa Reduces VX-809 Stimulated F508del-CFTR Chloride Secretion by Airway Epithelial Cells
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James F. Collawn et al.
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Pseudomonas aeruginosa flagellar motility activates the phagocyte PI3K/Akt pathway to induce phagocytic engulfment
Rustin R. Lovewell et al.
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Mechanisms of phagocytosis and host clearance of Pseudomonas aeruginosa
Rustin R. Lovewell et al.
AMERICAN JOURNAL OF PHYSIOLOGY-LUNG CELLULAR AND MOLECULAR PHYSIOLOGY (2014)
Gender Differences in Outcomes of Patients with Cystic Fibrosis
Cayce L. Harness-Brumley et al.
JOURNAL OF WOMENS HEALTH (2014)
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Guido Veit et al.
SCIENCE TRANSLATIONAL MEDICINE (2014)
Potentiator ivacaftor abrogates pharmacological correction of ΔF508 CFTR in cystic fibrosis
Deborah M. Cholon et al.
SCIENCE TRANSLATIONAL MEDICINE (2014)
A CFTR corrector (lumacaftor) and a CFTR potentiator (ivacaftor) for treatment of patients with cystic fibrosis who have a phe508del CFTR mutation: a phase 2 randomised controlled trial
Michael P. Boyle et al.
LANCET RESPIRATORY MEDICINE (2014)
Optimal Complement-Mediated Phagocytosis of Pseudomonas aeruginosa by Monocytes Is Cystic Fibrosis Transmembrane Conductance Regulator-Dependent
Pauline B. Van de Weert-van Leeuwen et al.
AMERICAN JOURNAL OF RESPIRATORY CELL AND MOLECULAR BIOLOGY (2013)
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Jamie L. Bessich et al.
JOURNAL OF IMMUNOLOGY (2013)
Impaired Functions of Macrophage from Cystic Fibrosis Patients: CD11b, TLR-5 Decrease and sCD14, Inflammatory Cytokines Increase
Karin Simonin-Le Jeune et al.
PLOS ONE (2013)
Results of a phase IIa study of VX-809, an investigational CFTR corrector compound, in subjects with cystic fibrosis homozygous for the F508del-CFTR mutation
J. P. Clancy et al.
THORAX (2012)
Abnormal Trafficking and Degradation of TLR4 Underlie the Elevated Inflammatory Response in Cystic Fibrosis
Emanuela M. Bruscia et al.
JOURNAL OF IMMUNOLOGY (2011)
Dysfunctional CFTR Alters the Bactericidal Activity of Human Macrophages against Pseudomonas aeruginosa
Paola Del Porto et al.
PLOS ONE (2011)
Correction of the F508del-CFTR protein processing defect in vitro by the investigational drug VX-809
Fredrick Van Goor et al.
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA (2011)
Alterations in Ceramide Concentration and pH Determine the Release of Reactive Oxygen Species by Cftr-Deficient Macrophages on Infection
Yang Zhang et al.
JOURNAL OF IMMUNOLOGY (2010)
Corr4A and VRT325 do not Reduce the Inflammatory Response to P. aeruginosa in Human Cystic Fibrosis Airway Epithelial Cells
Laleh Talebian et al.
CELLULAR PHYSIOLOGY AND BIOCHEMISTRY (2009)
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