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Cardiovascular Research 2003 60(2):235-241; doi:10.1016/j.cardiores.2003.08.002
© 2003 by European Society of Cardiology
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Copyright © 2003, European Society of Cardiology

Defective protein trafficking in hERG-associated hereditary long QT syndrome (LQT2): molecular mechanisms and restoration of intracellular protein processing

Dierk Thomas*, Johann Kiehn, Hugo A Katus and Christoph A Karle

Department of Cardiology, Medical University Hospital Heidelberg, Bergheimerstrasse 58, D-69115 Heidelberg, Germany

*Corresponding author. Tel.: +49-6221-568476; fax: +49-6221-565515. Email address: dierk_thomas{at}med.uni-heidelberg.de

Human hereditary long QT syndrome is a cardiac disease characterized by prolongation of the QT interval and increased susceptibility to ventricular arrhythmias and sudden cardiac death. Mutations in the human-ether-a-go-go-related gene (hERG), encoding the protein underlying the repolarizing cardiac IKr potassium current, cause chromosome 7-linked long QT syndrome 2. Loss of function of mutant hERG channels may be caused by several mechanisms, including altered current kinetics, altered ion selectivity, or defective intracellular protein trafficking. Especially the latter category has become a focus of particular interest recently, because some of the mutant subunits display wild type current properties when normal trafficking is restored and channels are inserted in the cell membrane in vitro. This review summarizes the current knowledge on hERG channel trafficking under physiological and pathological conditions. In addition, therapeutic approaches to restore normal hERG trafficking in vitro and in vivo are discussed.

KEYWORDS Antiarrhythmic agents; Arrhythmia (mechanisms); Ion channels; K-channel; Long QT syndrome

Abbreviations: CF, cystic fibrosis • CFTR, cystic fibrosis transmembrane conductance regulator • HERG, human ether-a-go-go-related gene • MiRP1, mink-related peptide 1 • IKr, rapidly activating component of the cardiac delayed rectifier potassium current • LQTS, long QT syndrome

Time for primary review 19 days


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