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Cystic Fibrosis Bronchial Epithelial Cells Are Lipointoxicated by Membrane Palmitate Accumulation

The F508del-CFTR mutation, responsible for Cystic Fibrosis (CF), leads to the retention of the protein in the endoplasmic reticulum (ER). The mistrafficking of this mutant form can be corrected by pharmacological chaperones, but these molecules showed limitations in clinical trials. We therefore hyp...

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Bibliographische Detailangaben
Hauptverfasser: Payet, Laurie-Anne, Kadri, Linette, Giraud, Sébastien, Norez, Caroline, Berjeaud, Jean Marc, Jayle, Christophe, Mirval, Sandra, Becq, Frédéric, Vandebrouck, Clarisse, Ferreira, Thierry
Format: Artigo
Sprache:Inglês
Veröffentlicht: Public Library of Science 2014
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Online Zugang:https://ncbi.nlm.nih.gov/pmc/articles/PMC3929646/
https://ncbi.nlm.nih.gov/pubmed/24586495
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1371/journal.pone.0089044
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