Verkerk Leonie, Verkerk Arie O, Wilders Ronald
Department of Medical Biology, Amsterdam Cardiovascular Sciences, Amsterdam UMC, University of Amsterdam, 1105 AZ Amsterdam, The Netherlands.
Department of Experimental Cardiology, Heart Center, Amsterdam Cardiovascular Sciences, Amsterdam UMC, University of Amsterdam, 1105 AZ Amsterdam, The Netherlands.
Rev Cardiovasc Med. 2024 Sep 5;25(9):313. doi: 10.31083/j.rcm2509313. eCollection 2024 Sep.
Brugada syndrome (BrS) is an inheritable cardiac arrhythmogenic disease, associated with an increased risk of sudden cardiac death. It is most common in males around the age of 40 and the prevalence is higher in Asia than in Europe and the United States. The pathophysiology underlying BrS is not completely understood, but several hypotheses have been proposed. So far, the best effective treatment is the implantation of an implantable cardioverter-defibrillator (ICD), but device-related complications are not uncommon. Therefore, there is an urgent need to improve diagnosis and risk stratification and to find new treatment options. To this end, research should further elucidate the genetic basis and pathophysiological mechanisms of BrS. Several experimental models are being used to gain insight into these aspects. The zebrafish () is a widely used animal model for the study of cardiac arrhythmias, as its cardiac electrophysiology shows interesting similarities to humans. However, zebrafish have only been used in a limited number of studies on BrS, and the potential role of zebrafish in studying the mechanisms of BrS has not been reviewed. Therefore, the present review aims to evaluate zebrafish as an animal model for BrS. We conclude that zebrafish can be considered as a valuable experimental model for BrS research, not only for gene editing technologies, but also for screening potential BrS drugs.
Brugada综合征（BrS）是一种遗传性致心律失常性心脏病，与心脏性猝死风险增加相关。它在40岁左右的男性中最为常见，亚洲的患病率高于欧洲和美国。BrS的病理生理学尚未完全明确，但已提出了几种假说。到目前为止，最有效的治疗方法是植入植入式心脏复律除颤器（ICD），但与设备相关的并发症并不少见。因此，迫切需要改进诊断和风险分层，并寻找新的治疗选择。为此，研究应进一步阐明BrS的遗传基础和病理生理机制。目前正在使用几种实验模型来深入了解这些方面。斑马鱼是一种广泛用于研究心律失常的动物模型，因为其心脏电生理学与人类有有趣的相似之处。然而，斑马鱼仅在有限数量的BrS研究中使用过，并且斑马鱼在研究BrS机制中的潜在作用尚未得到综述。因此，本综述旨在评估斑马鱼作为BrS的动物模型。我们得出结论，斑马鱼可被视为BrS研究的有价值的实验模型，不仅适用于基因编辑技术，也适用于筛选潜在的BrS药物。
