Mouse models of long QT syndrome

Guy Salama; Barry London

doi:10.1113/jphysiol.2006.118745

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Journal article

Open access

Peer reviewed

Mouse models of long QT syndrome

Guy Salama and Barry London

The Journal of physiology, Vol.578(Pt 1), pp.43-53

01/01/2007

DOI: 10.1113/jphysiol.2006.118745

PMCID: PMC2075110

PMID: 17038432

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Abstract

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.

Mutation - physiology

Animals

Heart - physiology

Humans

Mice, Transgenic

Mice

Disease Models, Animal

Long QT Syndrome - physiopathology

Details

Title: Subtitle: Mouse models of long QT syndrome
Creators: Guy Salama - Cardiovascular Institute, University of Pittsburgh Medical Center, Scaife S-572, 200 Lothrop Street, Pittsburgh, PA 15213, USA
Barry London
Resource Type: Journal article
Publication Details: The Journal of physiology, Vol.578(Pt 1), pp.43-53
DOI: 10.1113/jphysiol.2006.118745
PMID: 17038432
PMCID: PMC2075110
NLM abbreviation: J Physiol
ISSN: 0022-3751
eISSN: 1469-7793
Publisher: England
Grant note: R01 HL 59614 / NHLBI NIH HHS R01 HL 70722 / NHLBI NIH HHS R01 HL057929 / NHLBI NIH HHS R01 HL070722 / NHLBI NIH HHS R01 HL 66096 / NHLBI NIH HHS R01 HL066096 / NHLBI NIH HHS R01 HL 57929 / NHLBI NIH HHS
Language: English
Date published: 01/01/2007
Academic Unit: Molecular Physiology and Biophysics; Cardiovascular Medicine; Internal Medicine
Record Identifier: 9984025574802771

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