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The hERG Chanel Trafficking and Endoplasmic Reticulum Stress
Chen Jiayuan1,2, Lian Jiangfang1,2*
1Medical School of Ningbo University, Ningbo 315000, China; 2Ningbo Medical Center, Lihuili Hospital, Ningbo 315000, China
Abstract: Hereditary long QT syndrome is a fatal arrhythmia with an increased risk for syncope， ventricular tachycardia and the potentially fatal tachyarrhythmia Torsades de pointes. It is characterized by an abnormally long QT interval of ≥ 450 ms on the ECG. LQTS stype 2 (LQT2) associated with hERG gene mutations is the most common type of LQTS. Most of the mutant hERG proteins in LQT2 have folding deficiency and trafficking defects so that they are retained in the endoplasmic reticulum (ER) by cellular quality control mechanisms. Therefore， stabilization (facilitation of folding and alleviation of degradation) of the mutant proteins to ameliorate trafficking defects and increase IKr might be of therapeutic value for patients with LQT2. This review focuses on the quality control mechanisms in the ER that contribute to the folding and ERAD of hERG proteins.
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