Journal of Pharmacological Reports
Open Access
+44-20-4587-4809
+44-20-4587-4809
Chen B
China
Research Article
The Association of Endoplasmic Reticulum Stress Response and Clearance Pathways with Trafficking Deficiency of HERG Mutation and Pharmacological Rescue
Author(s): Lian JF, Fang, Zheng M, Huang X, Yang X, Guo J, Ba Y, Zhou J, Huang G, Wang Y, Chen J, Chen B and Zhuang KLian JF, Fang, Zheng M, Huang X, Yang X, Guo J, Ba Y, Zhou J, Huang G, Wang Y, Chen J, Chen B and Zhuang K
Background: The human ether-a-go-go related gene (HERG) gene mutation leads to type II hereditary long QT syndrome (LQTS2), which is a main cause of sudden cardiac death of young teenagers. Previous studies have demonstrated that most Type 2 Long QT syndrome caused by trafficking deficiency of HERG Mutation. Although many studies have been performed to elucidate the mechanisms of mutant HERG channel trafficking defects, the players and their exact roles in these process is still largely unknown. Herein, we investigated the HERG-A561V and HERG-L539fs/47, two HERG mutants with different trafficking process leading to LQTS2, studied the roles that chaperones Calnexin/Calreticulin and ATF6 play and proposed to treat LQTS through restoring protein trafficking as well as discuss if these molecules are involved in these process.
Methods: We transie.. View More»