Article
Rescue of the mutant CFTR chloride channel by pharmacological correctors and low temperature analyzed by gene expression profiling.
American journal of physiology. Cell physiology - 1 Oct 2011
Sondo Elvira, Tomati Valeria, Caci Emanuela, Esposito Alessia Isabella, Pfeffer Ulrich, Pedemonte Nicoletta, Galietta Luis J V
Abstract excerpt
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 dependent on the cell expression system. We have used a bronchial epithelial cell line, CFBE41o-, to determine the...
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