Long QT syndrome: Cellular basis and arrhythmia mechanism in LQT2

被引：87

作者：

January, CT ^{[1
]}

Gong, QM ^{[1
]}

Zhou, ZF ^{[1
]}

机构：

[1] Univ Wisconsin, Dept Med, Cardiol Sect, Madison, WI 53706 USA

来源：

JOURNAL OF CARDIOVASCULAR ELECTROPHYSIOLOGY | 2000年 / 11卷 / 12期

关键词：

potassium channels; HERG; arrhythmia; sudden death; cardiac action potentials; long QT syndrome;

D O I：

10.1046/j.1540-8167.2000.01413.x

中图分类号：

R5 [内科学];

学科分类号：

1002 ; 100201 ;

摘要：

LQT2 is one form of the congenital long QT syndrome. It results from mutations in the human ether-a-go-go-related gene (HERG), and more than 80 mutations, usually causing single amino acid substitutions in the HERG protein, are known, HERG encodes the ion channel pore-forming subunit protein for the rapidly activating delayed rectifier K+ channel (I-Kr) in the heart. This review summarizes current findings about mutations causing LQT2, the mechanisms by which mutations may cause the clinical phenotype of a reduction in I-Kr and a prolonged QT interval, and how this may be involved in the generation of ventricular arrhythmias.

引用

页码：1413 / 1418

页数：6

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