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Molecular Bases of Disease
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- Molecular Bases of DiseaseOpen Access
Combination of Correctors Rescue ΔF508-CFTR by Reducing Its Association with Hsp40 and Hsp27
Journal of Biological ChemistryVol. 290Issue 42p25636–25645Published online: September 2, 2015- Miquéias Lopes-Pacheco
- Clément Boinot
- Inna Sabirzhanova
- Marcelo M. Morales
- William B. Guggino
- Liudmila Cebotaru
Cited in Scopus: 30Background: Mutations in nucleotide binding domain 1 of cystic fibrosis (CF) transmembrane conductance regulator cause severe CF.Results: Correctors rescue trafficking of ΔF508 by altering interaction with Hsp27 and −40.Conclusion: Rescue of trafficking mutants can be accomplished by combining correctors from different classes.Significance: ΔF508 is the most common mutation. Information on the mechanism of corrector action is critical for treating the majority of patients.