HAL will be down for maintenance from Friday, June 10 at 4pm through Monday, June 13 at 9am. More information
Skip to Main content Skip to Navigation
Journal articles

Buserelin alleviates chloride transport defect in human cystic fibrosis nasal epithelial cells

Abstract : Cystic fibrosis (CF) is the most common autosomal recessive disease in Caucasians caused by mutations in the gene encoding the Cystic Fibrosis Transmembrane conduc-tance Regulator (CFTR) chloride (Cl-) channel regulated by protein kinases, phosphatases, divalent cations and by protein-protein interactions. Among protein-protein interactions, we previously showed that Annexin A5 (AnxA5) binds to CFTR and is involved in the channel localization within membranes and in its Cl-channel function. The deletion of phenylalanine at position 508 (F508del) is the most common mutation in CF which leads to an altered protein (F508del-CFTR) folding with a nascent protein retained within the ER and is quickly degraded. We previously showed that AnxA5 binds to F508del-CFTR and that its increased expression due to a Gonadoliberin (GnRH) augments Cl-efflux in cells expressing F508del-CFTR. The aim of the present work was to use the GnRH analog buserelin which is already used in medicine. Human nasal epithelial cells from controls and CF patients (F508del/ F508del) were treated with buserelin and we show here that the treatment alleviates Cl-channel defects in CF cells. Using proteomics we highlighted some proteins explaining this result. Finally, we propose that buserelin is a potential new pharmaceutical compound that can be used in CF and that bronchus can be targeted since we show here that they express GnRH-R.
Document type :
Journal articles
Complete list of metadata

Cited literature [70 references]  Display  Hide  Download

Contributor : Valerian Dormoy Connect in order to contact the contributor
Submitted on : Tuesday, May 26, 2020 - 5:52:48 PM
Last modification on : Thursday, March 31, 2022 - 3:41:43 AM


Publisher files allowed on an open archive




Marie-Laure Calvez, Nathalie Benz, Florentin Huguet, Aude Saint-Pierre, Elise Rouillé, et al.. Buserelin alleviates chloride transport defect in human cystic fibrosis nasal epithelial cells. PLoS ONE, Public Library of Science, 2017, 12 (11), pp.e0187774. ⟨10.1371/journal.pone.0187774⟩. ⟨hal-02448639⟩



Record views


Files downloads