Journal
JOURNAL OF PHYSIOLOGY-LONDON
Volume 578, Issue 1, Pages 43-53Publisher
WILEY
DOI: 10.1113/jphysiol.2006.118745
Keywords
-
Categories
Funding
- NHLBI NIH HHS [R01 HL066096, R01 HL 57929, R01 HL070722, R01 HL 66096, R01 HL 59614, R01 HL057929, R01 HL 70722] Funding Source: Medline
- NATIONAL HEART, LUNG, AND BLOOD INSTITUTE [R01HL057929, R01HL066096, R01HL070722, R01HL059614] Funding Source: NIH RePORTER
Ask authors/readers for more resources
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.
Authors
I am an author on this paper
Click your name to claim this paper and add it to your profile.
Reviews
Recommended
No Data Available