Combination of Correctors Rescue ΔF508-CFTR by Reducing Its Association with Hsp40 and Hsp27

Correcting the Cystic Fibrosis Disease Mutant, A455E CFTR

(2014) Liudmila Cebotaru et al.   PLoS One

Molecular Chaperone Functions in Protein Folding and Proteostasis

(2013) Yujin E. Kim et al.   Annual Review of Biochemistry

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

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

Transcomplementation by a Truncation Mutant of Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Enhances ΔF508 Processing through a Biomolecular Interaction

(2013) Liudmila Cebotaru et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Mechanism-based corrector combination restores ΔF508-CFTR folding and function

(2013) Tsukasa Okiyoneda et al.   Nature Chemical Biology

Requirements for Efficient Correction of ΔF508 CFTR Revealed by Analyses of Evolved Sequences

(2012) Juan L. Mendoza et al.   CELL

Small heat shock proteins target mutant cystic fibrosis transmembrane conductance regulator for degradation via a small ubiquitin-like modifier–dependent pathway

(2012) Annette Ahner et al.   MOLECULAR BIOLOGY OF THE CELL

Managing Cystic Fibrosis

(2011) Malena Cohen-Cymberknoh et al.   AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE

Sensitivity of Chloride Efflux vs. Transepithelial Measurements in Mixed CF and Normal Airway Epithelial Cell Populations

(2011) Beate Illek et al.   CELLULAR PHYSIOLOGY AND BIOCHEMISTRY

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

Modeling general proteostasis: proteome balance in health and disease

(2010) Daniela M Roth et al.   CURRENT OPINION IN CELL BIOLOGY

Effect of VX-770 in Persons with Cystic Fibrosis and the G551D-CFTRMutation

(2010) Frank J. Accurso et al.   NEW ENGLAND JOURNAL OF MEDICINE

The Primary Folding Defect and Rescue of ΔF508 CFTR Emerge during Translation of the Mutant Domain

(2010) Hanneke Hoelen et al.   PLoS One

Emergent Properties of Proteostasis in Managing Cystic Fibrosis

(2010) W. E. Balch et al.   Cold Spring Harbor Perspectives in Biology

Structure and Dynamics of NBD1 from CFTR Characterized Using Crystallography and Hydrogen/Deuterium Exchange Mass Spectrometry

(2009) H.A. Lewis et al.   JOURNAL OF MOLECULAR BIOLOGY

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

CFTR Function and Prospects for Therapy

(2008) John R. Riordan   Annual Review of Biochemistry

Cystic Fibrosis Transmembrane Regulator Missing the First Four Transmembrane Segments Increases Wild Type and ΔF508 Processing

(2008) Liudmila Cebotaru et al.   JOURNAL OF BIOLOGICAL CHEMISTRY