• Port-a-Patch

    Smallest patch clamp setup in the world
  • Port-a-Patch

    Easy to learn - ideal for teaching
  • Port-a-Patch

    Records from cells, organelles and bilayers
  • Port-a-Patch

    First planar patch clamp device on the market
  • Port-a-Patch

    Ideal for internal solution exchange applications

2014 - Improvement of Chloride Transport Defect by Gonadotropin-Releasing Hormone (GnRH) in Cystic Fibrosis Epithelial Cells

icon pap  Port-a-Patch publication in PLoS One (2014)

Authors: 
Benz N., Le Hir S., Norez C., Kerbiriou M., Calvez M.-L., Becq F., Trouvé P. , Férec C.

 

Journal: 
PLoS One (2014) 9(2):e88964


Abstract: 

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 traffic to the plasma membrane is altered. Nevertheless, if it reaches the cell surface, it exhibits a Cl channel function despite a short half-life. Pharmacological treatments may target the F508del-CFTR defect directly by binding to the mutant protein or indirectly by altering cellular proteostasis, and promote its plasma membrane targeting and stability. We previously showed that annexine A5 (AnxA5) directly binds to F508del-CFTR and, when overexpressed, promotes its membrane stability, leading to the restoration of some Cl channel function in cells. Because Gonadotropin-Releasing Hormone (GnRH) increases AnxA5 expression in some cells, we tested it in CF cells. We showed that human epithelial cells express GnRH-receptors (GnRH-R) and that GnRH induces an AnxA5 overexpression and an increased Cl channel function in F508del-CFTR cells, due to an increased stability of the protein in the membranes. Beside the numerous physiological implications of the GnRH-R expression in epithelial cells, we propose that a topical use of GnRH is a potential treatment in CF.


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