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钠通道在心肌传导中的作用:细微的基因改变如何导致严重的心律失常疾病。

Role of sodium channels in propagation in heart muscle: how subtle genetic alterations result in major arrhythmic disorders.

作者信息

Terrenoire Cecile, Simhaee Daniel, Kass Robert S

机构信息

Department of Pharmacology, College of Physicians and Surgeons of Columbia University, New York, New York 10032, USA.

出版信息

J Cardiovasc Electrophysiol. 2007 Aug;18(8):900-5. doi: 10.1111/j.1540-8167.2007.00838.x. Epub 2007 May 14.

Abstract

Sodium channels play a crucial role in initiation, propagation, and maintenance of cardiac excitation throughout the heart. Indeed, dysfunctional sodium channels have been shown to be responsible for several inherited cardiac electrical disorders, such as Long QT and Brugada syndromes (BrS), potentially leading to fatal arrhythmic events. Genetic approaches and functional experiments using heterologous systems have enabled the characterization of the molecular determinants involved in these disorders and their consequences on ion channel function. The improved understanding of the mechanisms leading to these cardiac arrhythmic events represents a first step in the development of therapeutic treatments.

摘要

钠通道在心脏兴奋的起始、传导和维持过程中起着至关重要的作用。事实上,功能失调的钠通道已被证明是多种遗传性心脏电疾病的病因,如长QT综合征和Brugada综合征(BrS),这些疾病可能导致致命的心律失常事件。利用异源系统的遗传学方法和功能实验,已经能够确定参与这些疾病的分子决定因素及其对离子通道功能的影响。对导致这些心律失常事件的机制的深入了解是开发治疗方法的第一步。

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