Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Potentiator VX-770 (Ivacaftor) Opens the Defective Channel Gate of Mutant CFTR in a Phosphorylation-dependent but ATP-independent Manner
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Title
Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Potentiator VX-770 (Ivacaftor) Opens the Defective Channel Gate of Mutant CFTR in a Phosphorylation-dependent but ATP-independent Manner
Authors
Keywords
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Journal
JOURNAL OF BIOLOGICAL CHEMISTRY
Volume 287, Issue 44, Pages 36639-36649
Publisher
American Society for Biochemistry & Molecular Biology (ASBMB)
Online
2012-09-02
DOI
10.1074/jbc.m112.393637
References
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Related references
Note: Only part of the references are listed.- A Unified View of Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Gating: Combining the Allosterism of a Ligand-gated Channel with the Enzymatic Activity of an ATP-binding Cassette (ABC) Transporter
- (2011) Kevin L. Kirk et al. JOURNAL OF BIOLOGICAL CHEMISTRY
- Synthesis and Properties of Molecular Probes for the Rescue Site on Mutant Cystic Fibrosis Transmembrane Conductance Regulator
- (2011) Bashar Alkhouri et al. JOURNAL OF MEDICINAL 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
- 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
- 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
- Amphiphile regulation of ion channel function by changes in the bilayer spring constant
- (2010) J. A. Lundbaek et al. PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA
- ATP-independent CFTR channel gating and allosteric modulation by phosphorylation
- (2010) Wei Wang et al. PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA
- Peripheral Protein Quality Control Removes Unfolded CFTR from the Plasma Membrane
- (2010) T. Okiyoneda et al. SCIENCE
- Curcumin Cross-links Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Polypeptides and Potentiates CFTR Channel Activity by Distinct Mechanisms
- (2009) Karen Bernard et al. JOURNAL OF BIOLOGICAL CHEMISTRY
- Functional Reconstitution of Purified Human Hv1 H+ Channels
- (2009) Seok-Yong Lee et al. JOURNAL OF MOLECULAR BIOLOGY
- Gating of the CFTR Cl−channel by ATP-driven nucleotide-binding domain dimerisation
- (2009) Tzyh-Chang Hwang et al. JOURNAL OF PHYSIOLOGY-LONDON
- A Small-Molecule Modulator Interacts Directly with Phe508-CFTR to Modify Its ATPase Activity and Conformational Stability
- (2009) L. Wellhauser et al. MOLECULAR PHARMACOLOGY
- 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
- The intact CFTR protein mediates ATPase rather than adenylate kinase activity
- (2008) Mohabir Ramjeesingh et al. BIOCHEMICAL JOURNAL
- Direct interaction of a small-molecule modulator with G551D-CFTR, a cystic fibrosis-causing mutation associated with severe disease
- (2008) Stan Pasyk et al. BIOCHEMICAL JOURNAL
- What’s new in cystic fibrosis? From treating symptoms to correction of the basic defect
- (2008) Marijke Proesmans et al. EUROPEAN JOURNAL OF PEDIATRICS
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