Deletion of Phenylalanine 508 in the First Nucleotide-binding Domain of the Cystic Fibrosis Transmembrane Conductance Regulator Increases Conformational Exchange and Inhibits Dimerization

Restoration of NBD1 Thermal Stability Is Necessary and Sufficient to Correct ∆F508 CFTR Folding and Assembly

(2015) Lihua He et al.   JOURNAL OF MOLECULAR BIOLOGY

Genetic, cell biological and clinical interrogation of the CFTR mutation c.3700 A>G (p.Ile1234Val) informs strategies for future medical intervention

(2014) Steven V. Molinski et al.   GENETICS IN MEDICINE

Conserved Allosteric Hot Spots in the Transmembrane Domains of Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Channels and Multidrug Resistance Protein (MRP) Pumps

(2014) Shipeng Wei et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Revertant mutants modify, but do not rescue, the gating defect of the cystic fibrosis mutant G551D-CFTR

(2014) Zhe Xu et al.   JOURNAL OF PHYSIOLOGY-LONDON

Mechanistic determinants of the directionality and energetics of active export by a heterodimeric ABC transporter

(2014) Nina Grossmann et al.   Nature Communications

Effect of ivacaftor on CFTR forms with missense mutations associated with defects in protein processing or function

(2013) Fredrick Van Goor et al.   Journal of Cystic Fibrosis

VX-809 corrects folding defects in cystic fibrosis transmembrane conductance regulator protein through action on membrane-spanning domain 1

(2013) Hong Yu Ren et al.   MOLECULAR BIOLOGY OF THE CELL

Allosteric Coupling between the Intracellular Coupling Helix 4 and Regulatory Sites of the First Nucleotide-binding Domain of CFTR

(2013) Jennifer E. Dawson et al.   PLoS One

Gout-causing Q141K mutation in ABCG2 leads to instability of the nucleotide-binding domain and can be corrected with small molecules

(2013) O. M. Woodward et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

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

Conformational Changes Relevant to Channel Activity and Folding within the first Nucleotide Binding Domain of the Cystic Fibrosis Transmembrane Conductance Regulator

(2012) Rhea P. Hudson et al.   JOURNAL OF BIOLOGICAL 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

The most common cystic fibrosis-associated mutation destabilizes the dimeric state of the nucleotide-binding domains of CFTR

(2011) Kang-Yang Jih et al.   JOURNAL OF PHYSIOLOGY-LONDON

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

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

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

Structures of a minimal human CFTR first nucleotide-binding domain as a monomer, head-to-tail homodimer, and pathogenic mutant

(2010) Shane Atwell et al.   PROTEIN ENGINEERING DESIGN & SELECTION

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

(2010) Irina Protasevich et al.   PROTEIN SCIENCE

Integrated biophysical studies implicate partial unfolding of NBD1 of CFTR in the molecular pathogenesis of F508del cystic fibrosis

(2010) Chi Wang et al.   PROTEIN SCIENCE

NMR evidence for differential phosphorylation-dependent interactions in WT and ΔF508 CFTR

(2009) Voula Kanelis et al.   EMBO JOURNAL

TALOS+: a hybrid method for predicting protein backbone torsion angles from NMR chemical shifts

(2009) Yang Shen et al.   JOURNAL OF BIOMOLECULAR NMR

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

Relationship between nucleotide binding and ion channel gating in cystic fibrosis transmembrane conductance regulator

(2009) Andrei A. Aleksandrov et al.   JOURNAL OF PHYSIOLOGY-LONDON

Solubilizing Mutations Used to Crystallize One CFTR Domain Attenuate the Trafficking and Channel Defects Caused by the Major Cystic Fibrosis Mutation

(2008) Luísa S. Pissarra et al.   CHEMISTRY & BIOLOGY

ΔF508 mutation increases conformational flexibility of CFTR protein

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