Rescue of NBD2 Mutants N1303K and S1235R of CFTR by Small-Molecule Correctors and Transcomplementation
Published 2015 View Full Article
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Title
Rescue of NBD2 Mutants N1303K and S1235R of CFTR by Small-Molecule Correctors and Transcomplementation
Authors
Keywords
HEK 293 cells, Cell membranes, Chlorides, Proteasomes, Lysosomes, Membrane proteins, Cystic fibrosis, Transfection
Journal
PLoS One
Volume 10, Issue 3, Pages e0119796
Publisher
Public Library of Science (PLoS)
Online
2015-03-24
DOI
10.1371/journal.pone.0119796
References
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Related references
Note: Only part of the references are listed.- Correcting the Cystic Fibrosis Disease Mutant, A455E CFTR
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- Requirements for Efficient Correction of ΔF508 CFTR Revealed by Analyses of Evolved Sequences
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- Correction of the F508del-CFTR protein processing defect in vitro by the investigational drug VX-809
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- Results of a phase IIa study of VX-809, an investigational CFTR corrector compound, in subjects with cystic fibrosis homozygous for theF508del-CFTRmutation
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- CFTR expression and activity from the human CFTR locus in BAC vectors, with regulatory regions, isolated by a single-step procedure
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- 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
- Effect of VX-770 in Persons with Cystic Fibrosis and the G551D-CFTRMutation
- (2010) Frank J. Accurso et al. NEW ENGLAND JOURNAL OF MEDICINE
- 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
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- Cystic Fibrosis Transmembrane Regulator Missing the First Four Transmembrane Segments Increases Wild Type and ΔF508 Processing
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