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
出版年份 2012 全文链接
标题
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
作者
关键词
-
出版物
JOURNAL OF BIOLOGICAL CHEMISTRY
Volume 287, Issue 44, Pages 36639-36649
出版商
American Society for Biochemistry & Molecular Biology (ASBMB)
发表日期
2012-09-02
DOI
10.1074/jbc.m112.393637
参考文献
相关参考文献
注意:仅列出部分参考文献,下载原文获取全部文献信息。- 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
Add your recorded webinar
Do you already have a recorded webinar? Grow your audience and get more views by easily listing your recording on Peeref.
Upload NowAsk a Question. Answer a Question.
Quickly pose questions to the entire community. Debate answers and get clarity on the most important issues facing researchers.
Get Started