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

Thermal Instability of ΔF508 Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Channel Function: Protection by Single Suppressor Mutations and Inhibiting Channel Activity

(2012) Xuehong Liu et al.   BIOCHEMISTRY

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

New Model of Cystic Fibrosis Transmembrane Conductance Regulator Proposes Active Channel-like Conformation

(2012) James Dalton et al.   Journal of Chemical Information and Modeling

Ivacaftor potentiation of multiple CFTR channels with gating mutations

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

Structure–Activity Relationships of Cyanoquinolines with Corrector–Potentiator Activity in ΔF508 Cystic Fibrosis Transmembrane Conductance Regulator Protein

(2012) John M. Knapp et al.   JOURNAL OF MEDICINAL CHEMISTRY

Allosteric Modulation Balances Thermodynamic Stability and Restores Function of ΔF508 CFTR

(2012) Andrei A. Aleksandrov et al.   JOURNAL OF MOLECULAR BIOLOGY

Thermally Unstable Gating of the Most Common Cystic Fibrosis Mutant Channel (ΔF508)

(2011) Wei Wang et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Cyanoquinolines with Independent Corrector and Potentiator Activities Restore  Phe508-Cystic Fibrosis Transmembrane Conductance Regulator Chloride Channel Function in Cystic Fibrosis

(2011) P.-W. Phuan et al.   MOLECULAR PHARMACOLOGY

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

CFTR: folding, misfolding and correcting the ΔF508 conformational defect

(2011) Gergely L. Lukacs et al.   TRENDS IN MOLECULAR MEDICINE

Modifier genes in Mendelian disorders: the example of cystic fibrosis

(2010) Garry R. Cutting   Annals of the New York Academy of Sciences

The V510D Suppressor Mutation Stabilizes ΔF508-CFTR at the Cell Surface

(2010) Tip W. Loo et al.   BIOCHEMISTRY

Restoration of domain folding and interdomain assembly by second-site suppressors of the ΔF508 mutation in CFTR

(2010) Lihua He et al.   FASEB JOURNAL

The Cystic Fibrosis-causing Mutation ΔF508 Affects Multiple Steps in Cystic Fibrosis Transmembrane Conductance Regulator Biogenesis

(2010) Patrick H. Thibodeau et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Rapid Flexible Docking Using a Stochastic Rotamer Library of Ligands

(2010) Feng Ding et al.   Journal of Chemical Information and Modeling

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

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

Regulatory Insertion Removal Restores Maturation, Stability and Function of ΔF508 CFTR

(2010) Andrei A. Aleksandrov et al.   JOURNAL OF MOLECULAR BIOLOGY

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

(2010) Hanneke Hoelen et al.   PLoS One

Thermal unfolding studies show the disease causing F508del mutation in CFTR thermodynamically destabilizes nucleotide-binding domain 1

(2010) Irina Protasevich et al.   PROTEIN SCIENCE

Cystic Fibrosis Transmembrane Conductance Regulator: Using Differential Reactivity toward Channel-Permeant and Channel-Impermeant Thiol-Reactive Probes To Test a Molecular Model for the Pore

(2009) Christopher Alexander et al.   BIOCHEMISTRY

Molecular models of the open and closed states of the whole human CFTR protein

(2009) Jean-Paul Mornon et al.   CELLULAR AND MOLECULAR LIFE SCIENCES

Modifier gene study of meconium ileus in cystic fibrosis: statistical considerations and gene mapping results

(2009) Ruslan Dorfman et al.   HUMAN GENETICS

Genetic Modifiers of Liver Disease in Cystic Fibrosis

(2009) Jaclyn R. Bartlett   JAMA-JOURNAL OF THE AMERICAN MEDICAL ASSOCIATION

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

Multiple Membrane-Cytoplasmic Domain Contacts in the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Mediate Regulation of Channel Gating

(2008) Lihua He et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

MedusaScore: An Accurate Force Field-Based Scoring Function for Virtual Drug Screening

(2008) Shuangye Yin et al.   Journal of Chemical Information and Modeling

Phenylalanine-508 mediates a cytoplasmic-membrane domain contact in the CFTR 3D structure crucial to assembly and channel function

(2008) A. W. R. Serohijos et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Chemical and Biological Folding Contribute to Temperature-Sensitive ΔF508 CFTR Trafficking

(2008) Xiaodong Wang et al.   TRAFFIC