Related Experiment Video
Updated: Jun 27, 2026

Recapitulation of an Ion Channel IV Curve Using Frequency Components
Published on: February 8, 2011
Human ether-a-go-go related gene (hERG) K+ channels: function and dysfunction
Mark J Perrin1, Rajesh N Subbiah, Jamie I Vandenberg
1Victor Chang Cardiac Research Institute, 405 Liverpool Street, Darlinghurst, NSW 2010, Australia.
Insights
Mutations in the human Ether-a-go-go Related Gene (hERG) potassium channel cause long QT syndrome. Understanding hERG channel function and drug interactions is key to preventing cardiac arrhythmias and sudden death.
Area of Science:
- Cardiovascular Physiology
- Molecular Cardiology
- Pharmacology
Background:
- The human Ether-a-go-go Related Gene (hERG) potassium channel is crucial for cardiac electrical activity and rhythm regulation.
- hERG channel dysfunction is implicated in both congenital and acquired long QT syndrome, leading to potentially fatal ventricular arrhythmias.
- The hERG channel is a primary target for drugs that induce acquired long QT syndrome.
Purpose of the Study:
- To review current knowledge on hERG channel function and its role in cardiac disorders.
- To summarize advances in understanding hERG gating, trafficking, and drug interactions.
- To highlight research directions for preventing and treating hERG-associated long QT syndrome.
Main Methods:
- Literature review of current research on hERG channel.
- Analysis of structural and functional studies related to hERG gating and drug block.
- Synthesis of information on hERG channel trafficking and its clinical implications.
Main Results:
- Detailed understanding of hERG channel's role in cardiac repolarization.
- Identification of hERG mutations as a cause for congenital long QT syndrome.
- Recognition of hERG as the molecular target for drug-induced long QT syndrome.
Conclusions:
- Advances in hERG channel research provide a basis for developing targeted therapies.
- Further investigation into hERG structure-function relationships is essential for clinical applications.
- Understanding hERG dysfunction is critical for preventing sudden cardiac death.
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.
Related Concept Videos
G-Protein Gated Ion Channels
Sensory organs,...
Cardiomyopathy III: Hypertrophic Cardiomyopathy
Human Genetics
The complex relationship between genetics and psychology is observable through common biological components such...
Pathophysiology of Heart Failure
Export of Misfolded Proteins out of the ER
Ligand-Gated Ion Channel Receptor: Gating Mechanism

