Cystic fibrosis bronchial epithelial cells are lipointoxicated by membrane palmitate accumulation.

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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Bibliographic Details
Main Authors: Laurie-Anne Payet, Linette Kadri, Sébastien Giraud, Caroline Norez, Jean Marc Berjeaud, Christophe Jayle, Sandra Mirval, Frédéric Becq, Clarisse Vandebrouck, Thierry Ferreira
Format: Article
Language:English
Published: Public Library of Science (PLoS) 2014-01-01
Series:PLoS ONE
Online Access:http://europepmc.org/articles/PMC3929646?pdf=render

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http://europepmc.org/articles/PMC3929646?pdf=render

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