Requirements for Efficient Correction of ΔF508 CFTR Revealed by Analyses of Evolved Sequences (Q27676826)

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object named as

Chad A Brautigam

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author name string

André Schmidt

2

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Qin Li

3

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Emmanuel Nuvaga

4

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Tyler Barrett

5

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Robert J Bridges

6

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Andrew P Feranchak

7

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Philip J Thomas

Effect of VX-770 in persons with cystic fibrosis and the G551D-CFTR mutation

9

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language of work or name

English

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publication date

20 January 2012

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published in

Cell

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volume

148

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issue

1-2

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page(s)

164-74

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cites work

1 reference

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Rescue of CF airway epithelial cell function in vitro by a CFTR potentiator, VX-770

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Structure of nucleotide-binding domain 1 of the cystic fibrosis transmembrane conductance regulator

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The primary folding defect and rescue of ΔF508 CFTR emerge during translation of the mutant domain

20 March 2017

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Identification of a NBD1-binding pharmacological chaperone that corrects the trafficking defect of F508del-CFTR.

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

29 September 2017

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

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Correction of the Delta phe508 cystic fibrosis transmembrane conductance regulator trafficking defect by the bioavailable compound glafenine.

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Structural analog of sildenafil identified as a novel corrector of the F508del-CFTR trafficking defect

29 September 2017

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Revertant mutants G550E and 4RK rescue cystic fibrosis mutants in the first nucleotide-binding domain of CFTR by different mechanisms

29 September 2017

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Slc26a6 regulates CFTR activity in vivo to determine pancreatic duct HCO3- secretion: relevance to cystic fibrosis

29 September 2017

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Rescue of DeltaF508-CFTR trafficking and gating in human cystic fibrosis airway primary cultures by small molecules

29 September 2017

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Small-molecule correctors of defective DeltaF508-CFTR cellular processing identified by high-throughput screening.

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The DeltaF508 cystic fibrosis mutation impairs domain-domain interactions and arrests post-translational folding of CFTR.

29 September 2017

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Mutations in the nucleotide binding domain 1 signature motif region rescue processing and functional defects of cystic fibrosis transmembrane conductance regulator delta f508.

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29 September 2017

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DOI

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1435260

1435260

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