Cysteamine re-establishes the clearance of Pseudomonas aeruginosa by macrophages bearing the cystic fibrosis-relevant F508del-CFTR mutation

A novel treatment of cystic fibrosis acting on-target: cysteamine plus epigallocatechin gallate for the autophagy-dependent rescue of class II-mutated CFTR

(2016) A Tosco et al.   CELL DEATH AND DIFFERENTIATION

The Approach to Pseudomonas aeruginosa in Cystic Fibrosis

(2016) Jaideep S. Talwalkar et al.   CLINICS IN CHEST MEDICINE

New and emerging targeted therapies for cystic fibrosis

(2016) Bradley S Quon et al.   BMJ-British Medical Journal

Animal Models of Cystic Fibrosis Pathology: Phenotypic Parallels and Divergences

(2016) Gillian M. Lavelle et al.   Biomed Research International

New and emerging targeted therapies for cystic fibrosis

(2016) Bradley S Quon et al.   BMJ-British Medical Journal

Progress in therapies for cystic fibrosis

(2016) Kris De Boeck et al.   Lancet Respiratory Medicine

The Evolution of Cystic Fibrosis Care

(2015) Jessica E. Pittman et al.   CHEST

Repairing the basic defect in cystic fibrosis - one approach is not enough

(2015) Carlos M. Farinha et al.   FEBS Journal

Hallmarks of therapeutic management of the cystic fibrosis functional landscape

(2015) Margarida D. Amaral et al.   Journal of Cystic Fibrosis

Novel personalized therapies for cystic fibrosis: treating the basic defect in all patients

(2015) M. D. Amaral   JOURNAL OF INTERNAL MEDICINE

Another Beginning for Cystic Fibrosis Therapy

(2015) Pamela B. Davis   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

Lumacaftor/ivacaftor for patients homozygous for Phe508del-CFTR: should we curb our enthusiasm?

(2015) A. M. Jones et al.   THORAX

Restoration of CFTR function in patients with cystic fibrosis carrying the F508del-CFTR mutation

(2014) Daniela De Stefano et al.   Autophagy

The relative frequency of CFTR mutation classes in European patients with cystic fibrosis

(2014) K. De Boeck et al.   Journal of Cystic Fibrosis

Tissue Transglutaminase Activation Modulates Inflammation in Cystic Fibrosis via PPAR  Down-Regulation

(2014) L. Maiuri et al.   JOURNAL OF IMMUNOLOGY

Cysteamine (Lynovex®), a novel mucoactive antimicrobial & antibiofilm agent for the treatment of cystic fibrosis

(2014) Cedric Charrier et al.   Orphanet Journal of Rare Diseases

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

Disease-relevant proteostasis regulation of cystic fibrosis transmembrane conductance regulator

(2013) V R Villella et al.   CELL DEATH AND DIFFERENTIATION

Rescuing Mutant CFTR: A Multi-task Approach to a Better Outcome in Treating Cystic Fibrosis

(2013) Margarida D. Amaral et al.   CURRENT PHARMACEUTICAL DESIGN

Reduced Caveolin-1 Promotes Hyperinflammation due to Abnormal Heme Oxygenase-1 Localization in Lipopolysaccharide-Challenged Macrophages with Dysfunctional Cystic Fibrosis Transmembrane Conductance Regulator

(2013) P.-X. Zhang et al.   JOURNAL OF IMMUNOLOGY

Ivacaftor in a G551D Homozygote with Cystic Fibrosis

(2013) Michael J. Harrison et al.   NEW ENGLAND JOURNAL OF MEDICINE

Detection of Phospho-Sites Generated by Protein Kinase CK2 in CFTR: Mechanistic Aspects of Thr1471 Phosphorylation

(2013) Andrea Venerando et al.   PLoS One

Targeting autophagy as a novel strategy for facilitating the therapeutic action of potentiators on ΔF508 cystic fibrosis transmembrane conductance regulator

(2012) Alessandro Luciani et al.   Autophagy

Depletion of the Ubiquitin-binding Adaptor Molecule SQSTM1/p62 from Macrophages HarboringcftrΔF508 Mutation Improves the Delivery ofBurkholderia cenocepaciato the Autophagic Machinery

(2012) Basant A. Abdulrahman et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

The Role of Atg Proteins in Autophagosome Formation

(2011) Noboru Mizushima et al.   Annual Review of Cell and Developmental Biology

Autophagy stimulation by rapamycin suppresses lung inflammation and infection byBurkholderia cenocepaciain a model of cystic fibrosis

(2011) Basant A. Abdulrahman et al.   Autophagy

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

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

Autophagy and disease: always two sides to a problem

(2011) Sunandini Sridhar et al.   JOURNAL OF PATHOLOGY

Autophagy in immunity and inflammation

(2011) Beth Levine et al.   NATURE

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

(2011) Paola Del Porto et al.   PLoS One

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

Autophagy and the Integrated Stress Response

(2010) Guido Kroemer et al.   MOLECULAR CELL

Eaten alive: a history of macroautophagy

(2010) Zhifen Yang et al.   NATURE CELL BIOLOGY

Defective CFTR induces aggresome formation and lung inflammation in cystic fibrosis through ROS-mediated autophagy inhibition

(2010) Alessandro Luciani et al.   NATURE CELL BIOLOGY

Cystic fibrosis transmembrane conductance regulator and caveolin-1 regulate epithelial cell internalization of Pseudomonas aeruginosa

(2009) Milan Bajmoczi et al.   AMERICAN JOURNAL OF PHYSIOLOGY-CELL PHYSIOLOGY

Disease-causing Mutations in the Cystic Fibrosis Transmembrane Conductance Regulator Determine the Functional Responses of Alveolar Macrophages

(2009) Ludmila V. Deriy et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

SUMOylation of Tissue Transglutaminase as Link between Oxidative Stress and Inflammation

(2009) A. Luciani et al.   JOURNAL OF IMMUNOLOGY

Cystic fibrosis

(2009) Brian P O'Sullivan et al.   LANCET

Reduced histone deacetylase 7 activity restores function to misfolded CFTR in cystic fibrosis

(2009) Darren M Hutt et al.   Nature Chemical Biology

Cystic Fibrosis: Pathogenesis and Future Treatment Strategies

(2009) Felix A Ratjen   Respiratory Care

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