Discovery of novel potent ΔF508-CFTR correctors that target the nucleotide binding domain

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

(2012) Juan L. Mendoza et al.   CELL

Correction of Both NBD1 Energetics and Domain Interface Is Required to Restore ΔF508 CFTR Folding and Function

(2012) Wael M. Rabeh et al.   CELL

Correctors of ΔF508 CFTR restore global conformational maturation without thermally stabilizing the mutant protein

(2012) Lihua He et al.   FASEB JOURNAL

Keratin K18 Increases Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Surface Expression by Binding to Its C-terminal Hydrophobic Patch

(2012) Yuanyuan Duan et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Ivacaftor potentiation of multiple CFTR channels with gating mutations

(2012) Haihui Yu et al.   Journal of Cystic Fibrosis

Rescue of the mutant CFTR chloride channel by pharmacological correctors and low temperature analyzed by gene expression profiling

(2011) Elvira Sondo et al.   AMERICAN JOURNAL OF PHYSIOLOGY-CELL PHYSIOLOGY

Resveratrol rescues cAMP-dependent anionic transport in the cystic fibrosis pancreatic cell line CFPAC1

(2011) Nabila Hamdaoui et al.   BRITISH JOURNAL OF PHARMACOLOGY

Identification of a NBD1-Binding Pharmacological Chaperone that Corrects the Trafficking Defect of F508del-CFTR

(2011) Heidi M. Sampson et al.   CHEMISTRY & BIOLOGY

Disruption of cytokeratin-8 interaction with F508del-CFTR corrects its functional defect

(2011) Julien Colas et al.   HUMAN MOLECULAR GENETICS

The testis anion transporter TAT1 (SLC26A8) physically and functionally interacts with the cystic fibrosis transmembrane conductance regulator channel: a potential role during sperm capacitation

(2011) Baptiste Rode et al.   HUMAN MOLECULAR GENETICS

Pharmacological therapy for cystic fibrosis: From bench to bedside

(2011) Frédéric Becq et al.   Journal of Cystic Fibrosis

A CFTR Potentiator in Patients with Cystic Fibrosis and theG551DMutation

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

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

Small molecule correctors of F508del-CFTR discovered by structure-based virtual screening

(2010) Ori Kalid et al.   JOURNAL OF COMPUTER-AIDED MOLECULAR DESIGN

A Chemical Corrector Modifies the Channel Function of F508del-CFTR

(2010) P. K. Chiaw et al.   MOLECULAR PHARMACOLOGY

Correction of the  Phe508 Cystic Fibrosis Transmembrane Conductance Regulator Trafficking Defect by the Bioavailable Compound Glafenine

(2010) R. Robert et al.   MOLECULAR PHARMACOLOGY

Measurement of nasal potential difference in young children with an equivocal sweat test following newborn screening for cystic fibrosis

(2010) I. Sermet-Gaudelus et al.   THORAX

Docking Ligands into Flexible and Solvated Macromolecules. 4. Are Popular Scoring Functions Accurate for this Class of Proteins?

(2009) Pablo Englebienne et al.   Journal of Chemical Information and Modeling

A Small-Molecule Modulator Interacts Directly with  Phe508-CFTR to Modify Its ATPase Activity and Conformational Stability

(2009) L. Wellhauser et al.   MOLECULAR PHARMACOLOGY

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

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

CFTR Delivery to 25% of Surface Epithelial Cells Restores Normal Rates of Mucus Transport to Human Cystic Fibrosis Airway Epithelium

(2009) Liqun Zhang et al.   PLOS BIOLOGY

CFTR Function and Prospects for Therapy

(2008) John R. Riordan   Annual Review of Biochemistry

CSN5 binds to misfolded CFTR and promotes its degradation

(2008) Gaëlle Tanguy et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR CELL RESEARCH

Atomic model of human cystic fibrosis transmembrane conductance regulator: Membrane-spanning domains and coupling interfaces

(2008) J.-P. Mornon et al.   CELLULAR AND MOLECULAR LIFE SCIENCES

ΔF508 mutation increases conformational flexibility of CFTR protein

(2008) G. Wieczorek et al.   Journal of Cystic Fibrosis

Assessment of the CFTR and ENaC association

(2008) Bakhrom K. Berdiev et al.   Molecular BioSystems