© 1999 by European Society of Cardiology
Copyright © 1999, European Society of Cardiology
A plethora of mechanisms in the HERG-related long QT syndrome
Genetics meets electrophysiology
aThe Department of Medicine, Vanderbilt University School of Medicine, Nashville, TN 37232-6602, USA
bThe Department of Pharmacology, Vanderbilt University School of Medicine, Nashville, TN 37232-6602, USA
cThe Department of Anesthesiology, Vanderbilt University School of Medicine, Nashville, TN 37232-6602, USA
* Corresponding author. Division of Clinical Pharmacology, 532 Medical Research Building I, Vanderbilt University School of Medicine, 23d Ave. South at Pierce Avenue, Nashville, TN 37232-6602, USA. Tel.: +1-615-322-0067; fax: +1-615-343-4522 dan.roden@mcmail.vanderbilt.edu
Received 12 July 1999; accepted 12 July 1999
| The first 150 words of the full text of this article appear below. |
See article by Nakajima et al. [15] (pages 283–293) in this issue.
The ether à-go-go-related gene (eag) was cloned from a Drosophila mutant displaying a dance-like movement disorder on exposure to ether [1]. Homologs of eag have been cloned in mouse and rat, but not (at least yet) in human. However, a related gene, the human ether à-go-go-related gene (HERG) was cloned from a human hippocampal cDNA library in 1994 [2]. The function of HERG was initially obscure, but came to dramatic attention in early 1995 when Keating et al. [3] identified mutations in HERG linked to one form of the congenital long QT syndrome (LQT2), and shortly thereafter HERG was identified as the 
-subunit encoding the rapid component of delayed rectifier (IKr) [4,5]. Thus, HERG rapidly became of interest not only to geneticists interested in LQTS, but also to cardiac
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1 HERG mutations cause multiple defects in IKr |
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2 An LQT2 mutation in the voltage sensor |
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3 How many mutations, in how many genes? |
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