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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Detalles Bibliográficos
Autores principales: Payet, Laurie-Anne, Kadri, Linette, Giraud, Sébastien, Norez, Caroline, Berjeaud, Jean Marc, Jayle, Christophe, Mirval, Sandra, Becq, Frédéric, Vandebrouck, Clarisse, Ferreira, Thierry
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Public Library of Science 2014
Materias:
Research Article
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

Internet

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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