Lumacaftor (VX-809) restores the ability of CF macrophages to phagocytose and kill Pseudomonas aeruginosa

Impaired innate immune cells in cystic fibrosis: Is it really a surprise?

(2017) Tracey Bonfield et al.   Journal of Cystic Fibrosis

Monocyte derived macrophages from CF pigs exhibit increased inflammatory responses at birth

(2017) Lily Paemka et al.   Journal of Cystic Fibrosis

The impact of impaired macrophage functions in cystic fibrosis disease progression

(2017) Manuella Lévêque et al.   Journal of Cystic Fibrosis

Assessment of safety and efficacy of long-term treatment with combination lumacaftor and ivacaftor therapy in patients with cystic fibrosis homozygous for the F508del-CFTR mutation (PROGRESS): a phase 3, extension study

(2017) Michael W Konstan et al.   Lancet Respiratory Medicine

Combination therapy with cystic fibrosis transmembrane conductance regulator modulators augment the airway functional microanatomy

(2016) Susan E. Birket et al.   AMERICAN JOURNAL OF PHYSIOLOGY-LUNG CELLULAR AND MOLECULAR PHYSIOLOGY

Ivacaftor-Induced Proteomic Changes Suggest Monocyte Defects May Contribute to the Pathogenesis of Cystic Fibrosis

(2016) Katherine B. Hisert et al.   AMERICAN JOURNAL OF RESPIRATORY CELL AND MOLECULAR BIOLOGY

Low free drug concentration prevents inhibition of F508del CFTR functional expression by the potentiator VX-770 (ivacaftor)

(2016) Elizabeth Matthes et al.   BRITISH JOURNAL OF PHARMACOLOGY

Innate and Adaptive Immunity in Cystic Fibrosis

(2016) Emanuela M. Bruscia et al.   CLINICS IN CHEST MEDICINE

Cystic Fibrosis Lung Immunity: The Role of the Macrophage

(2016) Emanuela M. Bruscia et al.   Journal of Innate Immunity

Efficacy and safety of lumacaftor/ivacaftor combination therapy in patients with cystic fibrosis homozygous for Phe508del CFTR by pulmonary function subgroup: a pooled analysis

(2016) J Stuart Elborn et al.   Lancet Respiratory Medicine

Lumacaftor–Ivacaftor in Patients with Cystic Fibrosis Homozygous for Phe508del CFTR

(2015) Claire E. Wainwright et al.   NEW ENGLAND JOURNAL OF MEDICINE

Lumacaftor and ivacaftor in the management of patients with cystic fibrosis: current evidence and future prospects

(2015) Kelly Kuk et al.   Therapeutic Advances in Respiratory Disease

Pseudomonas aeruginosa Reduces VX-809 Stimulated F508del-CFTR Chloride Secretion by Airway Epithelial Cells

(2015) Bruce A. Stanton et al.   PLoS One

CFTR and lung homeostasis

(2014) James F. Collawn et al.   AMERICAN JOURNAL OF PHYSIOLOGY-LUNG CELLULAR AND MOLECULAR PHYSIOLOGY

Pseudomonas aeruginosa flagellar motility activates the phagocyte PI3K/Akt pathway to induce phagocytic engulfment

(2014) Rustin R. Lovewell et al.   AMERICAN JOURNAL OF PHYSIOLOGY-LUNG CELLULAR AND MOLECULAR PHYSIOLOGY

Mechanisms of phagocytosis and host clearance of Pseudomonas aeruginosa

(2014) Rustin R. Lovewell et al.   AMERICAN JOURNAL OF PHYSIOLOGY-LUNG CELLULAR AND MOLECULAR PHYSIOLOGY

Gender Differences in Outcomes of Patients with Cystic Fibrosis

(2014) Cayce L. Harness-Brumley et al.   JOURNAL OF WOMENS HEALTH

Cystic fibrosis genetics: from molecular understanding to clinical application

(2014) Garry R. Cutting   NATURE REVIEWS GENETICS

Some gating potentiators, including VX-770, diminish  F508-CFTR functional expression

(2014) G. Veit et al.   Science Translational Medicine

Potentiator ivacaftor abrogates pharmacological correction of  F508 CFTR in cystic fibrosis

(2014) D. M. Cholon et al.   Science Translational Medicine

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

(2014) Michael P Boyle et al.   Lancet Respiratory Medicine

Optimal Complement-Mediated Phagocytosis ofPseudomonas aeruginosaby Monocytes Is Cystic Fibrosis Transmembrane Conductance Regulator–Dependent

(2013) Pauline B. Van de Weert–van Leeuwen et al.   AMERICAN JOURNAL OF RESPIRATORY CELL AND MOLECULAR BIOLOGY

Low Levels of Insulin-like Growth Factor-1 Contribute to Alveolar Macrophage Dysfunction in Cystic Fibrosis

(2013) J. L. Bessich et al.   JOURNAL OF IMMUNOLOGY

Impaired Functions of Macrophage from Cystic Fibrosis Patients: CD11b, TLR-5 Decrease and sCD14, Inflammatory Cytokines Increase

(2013) Karin Simonin-Le Jeune et al.   PLoS One

Abnormal Trafficking and Degradation of TLR4 Underlie the Elevated Inflammatory Response in Cystic Fibrosis

(2011) E. M. Bruscia et al.   JOURNAL OF IMMUNOLOGY

Dysfunctional CFTR Alters the Bactericidal Activity of Human Macrophages against Pseudomonas aeruginosa

(2011) Paola Del Porto et al.   PLoS One

Correction of the F508del-CFTR protein processing defect in vitro by the investigational drug VX-809

(2011) F. Van Goor et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Results of a phase IIa study of VX-809, an investigational CFTR corrector compound, in subjects with cystic fibrosis homozygous for theF508del-CFTRmutation

(2011) J P Clancy et al.   THORAX

Alterations in Ceramide Concentration and pH Determine the Release of Reactive Oxygen Species by Cftr-Deficient Macrophages on Infection

(2010) Y. Zhang et al.   JOURNAL OF IMMUNOLOGY

Corr4A and VRT325 do not Reduce the Inflammatory Response to P. aeruginosa in Human Cystic Fibrosis Airway Epithelial Cells

(2009) Laleh Talebian et al.   CELLULAR PHYSIOLOGY AND BIOCHEMISTRY