Cystic fibrosis transmembrane conductance regulator (CFTR) modulators have differential effects on cystic fibrosis macrophage function

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

(2018) Roxanna Barnaby et al.   AMERICAN JOURNAL OF PHYSIOLOGY-LUNG CELLULAR AND MOLECULAR PHYSIOLOGY

Macrophage plasticity, polarization, and function in health and disease

(2018) Abbas Shapouri-Moghaddam et al.   JOURNAL OF CELLULAR PHYSIOLOGY

Phagocytosis depends on TRPV2-mediated calcium influx and requires TRPV2 in lipids rafts: alteration in macrophages from patients with cystic fibrosis

(2018) Manuella Lévêque et al.   Scientific Reports

An update on new and emerging therapies for cystic fibrosis

(2017) Kristin M. Hudock et al.   EXPERT OPINION ON EMERGING DRUGS

Cystic Fibrosis Transmembrane Conductance Regulator Attaches Tumor Suppressor PTEN to the Membrane and Promotes Anti Pseudomonas aeruginosa Immunity

(2017) Sebastián A. Riquelme et al.   IMMUNITY

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

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

CFTR-dependent defect in alternatively-activated macrophages in cystic fibrosis

(2017) Abdullah A. Tarique 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

Monocyte-derived alveolar macrophages drive lung fibrosis and persist in the lung over the life span

(2017) Alexander V. Misharin et al.   JOURNAL OF EXPERIMENTAL MEDICINE

Human Cystic Fibrosis Macrophages Have Defective Calcium-Dependent Protein Kinase C Activation of the NADPH Oxidase, an Effect Augmented byBurkholderia cenocepacia

(2017) Kaivon Assani et al.   JOURNAL OF IMMUNOLOGY

Cysteamine-mediated clearance of antibiotic-resistant pathogens in human cystic fibrosis macrophages

(2017) Chandra L. Shrestha et al.   PLoS One

Newborn Cystic Fibrosis Pigs Have a Blunted Early Response to an Inflammatory Stimulus

(2016) Jennifer A. Bartlett et al.   AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE

Mutations of Cystic Fibrosis Transmembrane Conductance Regulator Gene Cause a Monocyte-Selective Adhesion Deficiency

(2016) Claudio Sorio et al.   AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE

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

Relationships among CFTR expression, HCO3−secretion, and host defense may inform gene- and cell-based cystic fibrosis therapies

(2016) Viral S. Shah et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Airway acidification initiates host defense abnormalities in cystic fibrosis mice

(2016) V. S. Shah et al.   SCIENCE

Defective Innate Immunity and Hyperinflammation in Newborn Cystic Fibrosis Transmembrane Conductance Regulator–Knockout Ferret Lungs

(2015) Nicholas W. Keiser et al.   AMERICAN JOURNAL OF RESPIRATORY CELL AND MOLECULAR BIOLOGY

Quantitative Proteomics Reveals an Altered Cystic FibrosisIn VitroBronchial Epithelial Secretome

(2015) Jennifer R. Peters-Hall et al.   AMERICAN JOURNAL OF RESPIRATORY CELL AND MOLECULAR BIOLOGY

Host-pathogen interplay in the respiratory environment of cystic fibrosis

(2015) Lael M. Yonker et al.   Journal of Cystic Fibrosis

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

(2015)   NEW ENGLAND JOURNAL OF MEDICINE

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

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

Pharmacological modulation of the AKT/microRNA-199a-5p/CAV1 pathway ameliorates cystic fibrosis lung hyper-inflammation

(2015) Ping-xia Zhang et al.   Nature Communications

Current concepts of immune dysregulation in cystic fibrosis

(2014) N. Rieber et al.   INTERNATIONAL JOURNAL OF BIOCHEMISTRY & CELL BIOLOGY

IFN-γ Stimulates Autophagy-Mediated Clearance of Burkholderia cenocepacia in Human Cystic Fibrosis Macrophages

(2014) Kaivon Assani et al.   PLoS One

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

Impact of azithromycin treatment on macrophage gene expression in subjects with cystic fibrosis

(2013) Theodore J. Cory et al.   Journal of Cystic Fibrosis

Exaggerated inflammatory responses mediated by Burkholderia cenocepacia in human macrophages derived from Cystic fibrosis patients

(2012) Benjamin T. Kopp et al.   BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS

A CFTR Potentiator in Patients with Cystic Fibrosis and theG551DMutation

(2011) Bonnie W. Ramsey et al.   NEW ENGLAND JOURNAL OF MEDICINE

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

(2011) Paola Del Porto et al.   PLoS One

Defective CFTR Expression and Function Are Detectable in Blood Monocytes: Development of a New Blood Test for Cystic Fibrosis

(2011) Claudio Sorio et al.   PLoS One

Macrophages Directly Contribute to the Exaggerated Inflammatory Response in Cystic Fibrosis Transmembrane Conductance Regulator−/−Mice

(2008) Emanuela M. Bruscia et al.   AMERICAN JOURNAL OF RESPIRATORY CELL AND MOLECULAR BIOLOGY