The cardiac conduction system.
The cardiac conduction system.
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DOI：
10.1161/circulationaha.110.942284
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发表时间：
2011-03-01
期刊：
影响因子：
37.8
通讯作者：
Fishman GI
中科院分区：
文献类型：
--
作者：
Park DS;Fishman GI
人类的心脏在正常寿命内跳动25亿次，这是心脏传导系统(CCS)细胞完成的一项壮举。CCS的功能组件大致可分为脉冲产生节点和脉冲传播的His-Purkinje系统。人类的传导系统疾病已被确定为改变脉冲产生、脉冲传播或两者兼而有之的疾病。CCS功能障碍主要是由获得性条件引起的，如心肌缺血/梗死、年龄相关性退变、手术并发症和药物毒性。遗传形式的CCS病很少见，但每个新的突变都为CCS的发育和功能提供了宝贵的分子机制。应用包括人类遗传筛选、生物物理分析和转基因小鼠技术在内的多学科方法，已经产生了与维持正常CCS生理有关的广泛的基因家族(图1)。在这篇综述中，我们讨论了与人类CCS疾病有关的节律、传导阻滞、副传导和发育相关的基因家族(表)。我们还研究了可作为当前植入式起搏器的辅助或替代疗法的不断发展的治疗策略。
The human heart beats 2.5 billion times during a normal lifespan, a feat accomplished by cells of the cardiac conduction system (CCS). The functional components of the CCS can be broadly divided into the impulse-generating nodes and the impulse-propagating His-Purkinje system. Human diseases of the conduction system have been identified that alter impulse generation, impulse propagation, or both. CCS dysfunction is primarily due to acquired conditions such as myocardial ischemia/infarct, age-related degeneration, procedural complications, and drug toxicity. Inherited forms of CCS disease are rare, but each new mutation provides invaluable insight into the molecular mechanisms governing CCS development and function. Applying a multidisciplinary approach, which includes human genetic screening, biophysical analysis, and transgenic mouse technology, has yielded a broad array of gene families involved in maintaining normal CCS physiology (Figure 1). In this review, we discuss gene families that have been implicated in human CCS diseases of rhythm, conduction block, accessory conduction, and development (Table). We also investigate evolving therapeutic strategies that may serve as adjuvant or replacement therapy to current implantable pacemakers.