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长QT综合征:从离子通道到心律失常

Long QT syndrome: from channels to cardiac arrhythmias.

作者信息

Moss Arthur J, Kass Robert S

机构信息

Heart Research Follow-up Program, Department of Medicine, University of Rochester School of Medicine and Dentistry, Rochester, New York 14642, USA.

出版信息

J Clin Invest. 2005 Aug;115(8):2018-24. doi: 10.1172/JCI25537.

DOI:10.1172/JCI25537
PMID:16075042
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC1180552/
Abstract

Long QT syndrome, a rare genetic disorder associated with life-threatening arrhythmias, has provided a wealth of information about fundamental mechanisms underlying human cardiac electrophysiology that has come about because of truly collaborative interactions between clinical and basic scientists. Our understanding of the mechanisms that control the critical plateau and repolarization phases of the human ventricular action potential has been raised to new levels through these studies, which have clarified the manner in which both potassium and sodium channels regulate this critical period of electrical activity.

摘要

长QT综合征是一种与危及生命的心律失常相关的罕见遗传疾病,它提供了大量有关人类心脏电生理学基本机制的信息,这些信息源于临床科学家和基础科学家之间真正的合作互动。通过这些研究,我们对控制人类心室动作电位关键平台期和复极期机制的理解提升到了新的水平,这些研究阐明了钾通道和钠通道调节这一关键电活动时期的方式。

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2
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本文引用的文献

1
Ca(V)1.2 calcium channel dysfunction causes a multisystem disorder including arrhythmia and autism.Ca(V)1.2钙通道功能障碍会引发包括心律失常和自闭症在内的多系统疾病。
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Association of long QT syndrome loci and cardiac events among patients treated with beta-blockers.β受体阻滞剂治疗患者中长QT综合征基因座与心脏事件的关联。
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3
Calmodulin mediates Ca2+ sensitivity of sodium channels.钙调蛋白介导钠通道的钙离子敏感性。
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Mutation site-specific differences in arrhythmic risk and sensitivity to sympathetic stimulation in the LQT1 form of congenital long QT syndrome: multicenter study in Japan.先天性长QT综合征LQT1型心律失常风险及对交感神经刺激敏感性的突变位点特异性差异:日本多中心研究
J Am Coll Cardiol. 2004 Jul 7;44(1):117-25. doi: 10.1016/j.jacc.2004.03.043.
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A cardiac arrhythmia syndrome caused by loss of ankyrin-B function.由锚蛋白B功能丧失引起的一种心律失常综合征。
Proc Natl Acad Sci U S A. 2004 Jun 15;101(24):9137-42. doi: 10.1073/pnas.0402546101. Epub 2004 Jun 3.
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Andersen-Tawil syndrome: a model of clinical variability, pleiotropy, and genetic heterogeneity.安德森-陶威尔综合征:临床变异性、多效性和基因异质性的一个模型。
Ann Med. 2004;36 Suppl 1:92-7. doi: 10.1080/17431380410032490.
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Am J Physiol Heart Circ Physiol. 2004 Aug;287(2):H652-8. doi: 10.1152/ajpheart.00052.2004. Epub 2004 Apr 8.
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J Gen Physiol. 2004 Feb;123(2):155-65. doi: 10.1085/jgp.200308929.
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