The voltage-gated channel accessory protein KCNE2: multiple ion channel partners, multiple ways to long QT syndrome

Expert Rev Mol Med. 2011 Dec 14:13:e38. doi: 10.1017/S1462399411002092.

Abstract

The single-pass transmembrane protein KCNE2 or MIRP1 was once thought to be the missing accessory protein that combined with hERG to fully recapitulate the cardiac repolarising current IKr. As a result of this role, it was an easy next step to associate mutations in KCNE2 to long QT syndrome, in which there is delayed repolarisation of the heart. Since that time however, KCNE2 has been shown to modify the behaviour of several other channels and currents, and its role in the heart and in the aetiology of long QT syndrome has become less clear. In this article, we review the known interactions of the KCNE2 protein and the resulting functional effects, and the effects of mutations in KCNE2 and their clinical role.

Publication types

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

MeSH terms

  • Action Potentials
  • Amino Acid Sequence
  • ERG1 Potassium Channel
  • Ether-A-Go-Go Potassium Channels / metabolism
  • Ether-A-Go-Go Potassium Channels / physiology
  • Humans
  • Long QT Syndrome / genetics*
  • Molecular Sequence Data
  • Mutation
  • Potassium Channels, Voltage-Gated / chemistry
  • Potassium Channels, Voltage-Gated / genetics*
  • Potassium Channels, Voltage-Gated / physiology
  • Shab Potassium Channels / metabolism
  • Shab Potassium Channels / physiology

Substances

  • ERG1 Potassium Channel
  • Ether-A-Go-Go Potassium Channels
  • KCNB1 protein, human
  • KCNE2 protein, human
  • KCNH2 protein, human
  • Potassium Channels, Voltage-Gated
  • Shab Potassium Channels