Correctors Enhance Maturation of ΔF508 CFTR by Promoting Interactions between the Two Halves of the Molecule
about
Pharmacological chaperoning: a primer on mechanism and pharmacologyStabilizing rescued surface-localized δf508 CFTR by potentiation of its interaction with Na(+)/H(+) exchanger regulatory factor 1.Restoration of domain folding and interdomain assembly by second-site suppressors of the DeltaF508 mutation in CFTRCystic fibrosis transmembrane conductance regulator protein repair as a therapeutic strategy in cystic fibrosisCalpain inhibition promotes the rescue of F(508)del-CFTR in PBMC from cystic fibrosis patients.Thermally unstable gating of the most common cystic fibrosis mutant channel (ΔF508): "rescue" by suppressor mutations in nucleotide binding domain 1 and by constitutive mutations in the cytosolic loops.Capturing the Direct Binding of CFTR Correctors to CFTR by Using Click Chemistry.Gout-causing Q141K mutation in ABCG2 leads to instability of the nucleotide-binding domain and can be corrected with small moleculesCFTR: folding, misfolding and correcting the ΔF508 conformational defect.Update in cystic fibrosis 2009.Functional Rescue of F508del-CFTR Using Small Molecule CorrectorsThe V510D suppressor mutation stabilizes DeltaF508-CFTR at the cell surface.Increased efficacy of VX-809 in different cellular systems results from an early stabilization effect of F508del-CFTR.Correction of both NBD1 energetics and domain interface is required to restore ΔF508 CFTR folding and function.Pharmacological Chaperones: Beyond Conformational Disorders.Impact of the cystic fibrosis mutation F508del-CFTR on renal cyst formation and growth
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P2860
Correctors Enhance Maturation of ΔF508 CFTR by Promoting Interactions between the Two Halves of the Molecule
description
im Oktober 2009 veröffentlicher wissenschaftlicher Artikel
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scientific article published on 01 October 2009
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wetenschappelijk artikel
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наукова стаття, опублікована в жовтні 2009
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name
Correctors Enhance Maturation ...... the Two Halves of the Molecule
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Correctors Enhance Maturation ...... the Two Halves of the Molecule
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type
label
Correctors Enhance Maturation ...... the Two Halves of the Molecule
@en
Correctors Enhance Maturation ...... the Two Halves of the Molecule
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prefLabel
Correctors Enhance Maturation ...... the Two Halves of the Molecule
@en
Correctors Enhance Maturation ...... the Two Halves of the Molecule
@nl
P356
P1433
P1476
Correctors enhance maturation ...... the two halves of the molecule
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P2093
David M Clarke
M Claire Bartlett
P304
P356
10.1021/BI9004842
P407
P50
P577
2009-10-01T00:00:00Z