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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...
Autores principales: | Payet, Laurie-Anne, Kadri, Linette, Giraud, Sébastien, Norez, Caroline, Berjeaud, Jean Marc, Jayle, Christophe, Mirval, Sandra, Becq, Frédéric, Vandebrouck, Clarisse, Ferreira, Thierry |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Public Library of Science
2014
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3929646/ https://www.ncbi.nlm.nih.gov/pubmed/24586495 http://dx.doi.org/10.1371/journal.pone.0089044 |
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