| HOME | HELP | FEEDBACK | SUBSCRIPTIONS | ARCHIVE | SEARCH | TABLE OF CONTENTS |
|
|
|
|||||||
|
|||||||||
| ||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
Topical Review |
1 Department of Cell Biology and Physiology
2 Cardiovascular Institute, University of Pittsburgh, Pittsburgh, PA 15213, USA
Congenital long QT syndrome is a rare inherited condition characterized by prolongation of action potential duration (APD) in cardiac myocytes, prolongation of the QT interval on the surface electrocardiogram (ECG), and an increased risk of syncope and sudden death due to ventricular tachyarrhythmias. Mutations of cardiac ion channel genes that affect repolarization cause the majority of the congenital cases. Despite detailed characterizations of the mutated ion channels at the molecular level, a complete understanding of the mechanisms by which individual mutations may lead to arrhythmias and sudden death requires study of the intact heart and its modulation by the autonomic nervous system. Here, we will review studies of molecularly engineered mice with mutations in the genes (a) known to cause long QT syndrome in humans and (b) specific to cardiac repolarization in the mouse. Our goal is to provide the reader with a comprehensive overview of mouse models with long QT syndrome and to emphasize the advantages and limitations of these models.
(Received 5 August 2006;
accepted after revision 3 October 2006;
first published online 5 October 2006)
Corresponding author B. London: Cardiovascular Institute, University of Pittsburgh Medical Center, Scaife S-572, 200 Lothrop Street, Pittsburgh, PA 15213, USA. Email: londonb{at}upmc.edu
This article has been cited by other articles:
|
|
 |
|
  T. K. Roepke, A. Kontogeorgis, C. Ovanez, X. Xu, J. B. Young, K. Purtell, P. A. Goldstein, D. J. Christini, N. S. Peters, F. G. Akar, et al. Targeted deletion of kcne2 impairs ventricular repolarization via disruption of IK,slow1 and Ito,f FASEB J, October 1, 2008; 22(10): 3648 - 3660. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 Y. Etzion, M. Mor, A. Shalev, S. Dror, O. Etzion, A. Dagan, O. Beharier, A. Moran, and A. Katz New insights into the atrial electrophysiology of rodents using a novel modality: the miniature-bipolar hook electrode Am J Physiol Heart Circ Physiol, October 1, 2008; 295(4): H1460 - H1469. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
S. A. Grandy, V. Trepanier-Boulay, and C. Fiset Postnatal development has a marked effect on ventricular repolarization in mice Am J Physiol Heart Circ Physiol, October 1, 2007; 293(4): H2168 - H2177. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 R. Arnaout, T. Ferrer, J. Huisken, K. Spitzer, D. Y. R. Stainier, M. Tristani-Firouzi, and N. C. Chi Zebrafish model for human long QT syndrome PNAS, July 3, 2007; 104(27): 11316 - 11321. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 M. I. Kotlikoff From genotype to phenotype and back J. Physiol., January 1, 2007; 578(1): 7 - 8. [Full Text] [PDF]
|
|
| HOME | HELP | FEEDBACK | SUBSCRIPTIONS | ARCHIVE | SEARCH | TABLE OF CONTENTS |
