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Evaluation of autophagy inducers in epithelial cells carrying the ?F508 mutation of the cystic fibrosis transmembrane conductance regulator CFTR.


ABSTRACT: Cystic Fibrosis (CF) due to the ?F508 mutation of cystic fibrosis transmembrane conductance regulator (CFTR) can be treated with a combination of cysteamine and Epigallocatechin gallate (EGCG). Since ECGC is not a clinically approved drug, we attempted to identify other compounds that might favourably interact with cysteamine to induce autophagy and thus rescuing the function of ?F508 CFTR as a chloride channel in the plasma membrane. For this, we screened a compound library composed by chemically diverse autophagy inducers for their ability to enhance autophagic flux in the presence of cysteamine. We identified the antiarrhythmic Ca2+ channel blocker amiodarone, as an FDA-approved drug having the property to cooperate with cysteamine to stimulate autophagy in an additive manner. Amiodarone promoted the re-expression of ?F508 CFTR protein in the plasma membrane of respiratory epithelial cells. Hence, amiodarone might be yet another compound for the etiological therapy of CF in patients bearing the ?F508 CFTR mutation.

SUBMITTER: Zhang S 

PROVIDER: S-EPMC5833759 | biostudies-literature | 2018 Feb

REPOSITORIES: biostudies-literature

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Evaluation of autophagy inducers in epithelial cells carrying the ΔF508 mutation of the cystic fibrosis transmembrane conductance regulator CFTR.

Zhang Shaoyi S   Stoll Gautier G   Pedro José Manuel Bravo San JMBS   Sica Valentina V   Sauvat Allan A   Obrist Florine F   Kepp Oliver O   Li Yousheng Y   Maiuri Luigi L   Zamzami Naoufal N   Kroemer Guido G  

Cell death & disease 20180207 2


Cystic Fibrosis (CF) due to the ΔF508 mutation of cystic fibrosis transmembrane conductance regulator (CFTR) can be treated with a combination of cysteamine and Epigallocatechin gallate (EGCG). Since ECGC is not a clinically approved drug, we attempted to identify other compounds that might favourably interact with cysteamine to induce autophagy and thus rescuing the function of ΔF508 CFTR as a chloride channel in the plasma membrane. For this, we screened a compound library composed by chemical  ...[more]

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