Human ether-a-go-go related gene (hERG) K+ channels: function and dysfunction

Prog Biophys Mol Biol. 2008 Oct-Nov;98(2-3):137-48. doi: 10.1016/j.pbiomolbio.2008.10.006. Epub 2008 Nov 5.

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.

Publication types

  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • ERG1 Potassium Channel
  • Ether-A-Go-Go Potassium Channels / chemistry
  • Ether-A-Go-Go Potassium Channels / genetics*
  • Ether-A-Go-Go Potassium Channels / physiology*
  • Genotype
  • Humans
  • Ion Channel Gating
  • Long QT Syndrome / drug therapy
  • Long QT Syndrome / genetics*
  • Long QT Syndrome / physiopathology*
  • Mutation
  • Phenotype

Substances

  • ERG1 Potassium Channel
  • Ether-A-Go-Go Potassium Channels
  • KCNH2 protein, human