Article
Influence of cell background on pharmacological rescue of mutant CFTR.
American journal of physiology. Cell physiology - 1 Apr 2010
Pedemonte Nicoletta, Tomati Valeria, Sondo Elvira, Galietta Luis J V
Abstract excerpt
Cystic fibrosis (CF) is caused by mutations in the CFTR chloride channel. Deletion of phenylalanine 508 (F508del), the most frequent CF mutation, impairs the maturation and gating of the CFTR protein. Such defects may be corrected in vitro by pharmacological modulators named as correctors and potentiators, respectively. We have evaluated a panel of correctors and potentiators derived from various sources to...
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