Probing Conformational Rescue Induced by a Chemical Corrector of F508del-Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Mutant
Published 2011 View Full Article
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Title
Probing Conformational Rescue Induced by a Chemical Corrector of F508del-Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Mutant
Authors
Keywords
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Journal
JOURNAL OF BIOLOGICAL CHEMISTRY
Volume 286, Issue 28, Pages 24714-24725
Publisher
American Society for Biochemistry & Molecular Biology (ASBMB)
Online
2011-05-23
DOI
10.1074/jbc.m111.239699
References
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Related references
Note: Only part of the references are listed.- Identification of a NBD1-Binding Pharmacological Chaperone that Corrects the Trafficking Defect of F508del-CFTR
- (2011) Heidi M. Sampson et al. CHEMISTRY & BIOLOGY
- 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
- Regulatory Insertion Removal Restores Maturation, Stability and Function of ΔF508 CFTR
- (2010) Andrei A. Aleksandrov et al. JOURNAL OF MOLECULAR BIOLOGY
- A Chemical Corrector Modifies the Channel Function of F508del-CFTR
- (2010) P. K. Chiaw et al. MOLECULAR PHARMACOLOGY
- 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
- 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
- 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
- Functional Rescue of DeltaF508-CFTR by Peptides Designed to Mimic Sorting Motifs
- (2009) Patrick Kim Chiaw et al. CHEMISTRY & BIOLOGY
- A truncated CFTR protein rescues endogenous ΔF508-CFTR and corrects chloride transport in mice
- (2009) Estelle Cormet-Boyaka et al. FASEB JOURNAL
- 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
- Interplay between ER Exit Code and Domain Conformation in CFTR Misprocessing and Rescue
- (2009) Gargi Roy et al. MOLECULAR BIOLOGY OF THE CELL
- A Small-Molecule Modulator Interacts Directly with Phe508-CFTR to Modify Its ATPase Activity and Conformational Stability
- (2009) L. Wellhauser et al. MOLECULAR PHARMACOLOGY
- Strict coupling between CFTR's catalytic cycle and gating of its Cl- ion pore revealed by distributions of open channel burst durations
- (2009) L. Csanady et al. PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA
- 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
- Structure of P-Glycoprotein Reveals a Molecular Basis for Poly-Specific Drug Binding
- (2009) S. G. Aller et al. SCIENCE
- Correctors promote folding of the CFTR in the endoplasmic reticulum
- (2008) Tip W. Loo et al. BIOCHEMICAL JOURNAL
- 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
- 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
- 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
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