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

CFTR and lung homeostasis

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

Understanding how cystic fibrosis mutations disrupt CFTR function: From single molecules to animal models

(2014) Yiting Wang et al.   INTERNATIONAL JOURNAL OF BIOCHEMISTRY & CELL BIOLOGY

Combination of hypothiocyanite and lactoferrin (ALX-109) enhances the ability of tobramycin and aztreonam to eliminate Pseudomonas aeruginosa biofilms growing on cystic fibrosis airway epithelial cells

(2014) S. Moreau-Marquis et al.   JOURNAL OF ANTIMICROBIAL CHEMOTHERAPY

Signature Motifs Identify anAcinetobacterCif Virulence Factor with Epoxide Hydrolase Activity

(2014) Christopher D. Bahl et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

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

The CF-modifying gene EHF promotes p.Phe508del-CFTR residual function by altering protein glycosylation and trafficking in epithelial cells

(2013) Frauke Stanke et al.   EUROPEAN JOURNAL OF HUMAN GENETICS

Pouring Salt on a Wound: Pseudomonas aeruginosa Virulence Factors Alter Na+ and Cl- Flux in the Lung

(2013) A. E. Ballok et al.   JOURNAL OF BACTERIOLOGY

Arsenic Promotes Ubiquitinylation and Lysosomal Degradation of Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Chloride Channels in Human Airway Epithelial Cells

(2012) Jennifer M. Bomberger et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Cystic fibrosis: a mucosal immunodeficiency syndrome

(2012) Taylor Sitarik Cohen et al.   NATURE MEDICINE

An association study on contrasting cystic fibrosis endophenotypes recognizes KRT8 but not KRT18 as a modifier of cystic fibrosis disease severity and CFTR mediated residual chloride secretion

(2011) Frauke Stanke et al.   BMC Medical Genetics

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

A Pseudomonas aeruginosa Toxin that Hijacks the Host Ubiquitin Proteolytic System

(2011) Jennifer M. Bomberger et al.   PLoS Pathogens

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

Rescue of CF airway epithelial cell function in vitro by a CFTR potentiator, VX-770

(2009) F. Van Goor et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Long-Distance Delivery of Bacterial Virulence Factors by Pseudomonas aeruginosa Outer Membrane Vesicles

(2009) Jennifer M. Bomberger et al.   PLoS Pathogens

CFTR Function and Prospects for Therapy

(2008) John R. Riordan   Annual Review of Biochemistry