Human ether-a-go-go related gene (hERG) K channels: Function and dysfunction |
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| Authors: | Mark J. Perrin Rajesh N. Subbiah Jamie I. Vandenberg Adam P. Hill |
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| Affiliation: | aVictor Chang Cardiac Research Institute, 405 Liverpool Street, Darlinghurst, NSW 2010, Australia;bSt Vincent's Clinical School, University of New South Wales, Victoria Street, Darlinghurst, NSW 2010, Australia;cDepartment of Cardiology, St Vincent's Hospital, Victoria Street, Darlinghurst, NSW 2010, Australia |
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| Abstract: | The human Ether-a-go-go Related Gene (hERG) potassium channel plays a central role in regulating cardiac excitability and maintenance of normal cardiac rhythm. Mutations in hERG cause a third of all cases of congenital long QT syndrome, a disorder of cardiac repolarisation characterised by prolongation of the QT interval on the surface electrocardiogram, abnormal T waves, and a risk of sudden cardiac death due to ventricular arrhythmias. Additionally, the hERG channel protein is the molecular target for almost all drugs that cause the acquired form of long QT syndrome. Advances in understanding the structural basis of hERG gating, its traffic to the cell surface, and the molecular architecture involved in drug-block of hERG, are providing the foundation for rational treatment and prevention of hERG associated long QT syndrome. This review summarises the current knowledge of hERG function and dysfunction, and the areas of ongoing research. |
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| Keywords: | Long QT syndrome Arrhythmias Electrophysiology Voltage-gated K+ channel |
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