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RESCUE OF THE MUTANT CFTR CHLORIDE CHANNEL BY PHARMACOLOGICAL CORRECTORS AND LOW TEMPERATURE ANALYZED BY GENE EXPRESSION PROFILING

The F508del mutation, the most frequent in cystic fibrosis (CF), impairs the maturation of the CFTR chloride channel. The F508del defect can be partially overcome at low temperature (27 °C) or with pharmacological correctors. However, the efficacy of correctors on the mutant protein appears to be de...

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Bibliografiske detaljer
Main Authors: Sondo, Elvira, Tomati, Valeria, Caci, Emanuela, Esposito, Alessia Isabella, Pfeffer, Ulrich, Pedemonte, Nicoletta, Galietta, Luis J.V.
Format: Artigo
Sprog:Inglês
Udgivet: 2011
Fag:
Online adgang:https://ncbi.nlm.nih.gov/pmc/articles/PMC3512166/
https://ncbi.nlm.nih.gov/pubmed/21753184
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1152/ajpcell.00507.2010
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