Improvement of chloride transport defect by gonadotropin-releasing hormone (GnRH) in cystic fibrosis epithelial cells.

Cystic fibrosis (CF), the most common autosomal recessive disease in Caucasians, is due to mutations in the CFTR gene. F508del, the most frequent mutation in patients, impairs CFTR protein folding and biosynthesis. The F508del-CFTR protein is retained in the endoplasmic reticulum (ER) and its traffi...

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Bibliographic Details
Main Authors: Nathalie Benz, Sophie Le Hir, Caroline Norez, Mathieu Kerbiriou, Marie-Laure Calvez, Frédéric Becq, Pascal Trouvé, Claude Férec
Format: Article
Language:English
Published: Public Library of Science (PLoS) 2014-01-01
Series:PLoS ONE
Online Access:http://europepmc.org/articles/PMC3929654?pdf=render