Sudden cardiac death is a common cause of death in patients with structural heart disease, genetic mutations, or acquired disorders affecting cardiac ion channels. A wide range of platforms exist to model and study disorders associated with sudden cardiac death. Human clinical studies are cumbersome and are thwarted by the extent of investigation that can be performed on human subjects. Animal models are limited by their degree of homology to human cardiac electrophysiology, including ion channel expression. Most commonly used cellular models are cellular transfection models, which are able to mimic the expression of a single-ion channel offering incomplete insight into changes of the action potential profile. Induced pluripotent stem cell-derived cardiomyocytes resemble, but are not identical, adult human cardiomyocytes and provide a new platform for studying arrhythmic disorders leading to sudden cardiac death. A variety of platforms exist to phenotype cellular models, including conventional and automated patch clamp, multielectrode array, and computational modeling. Induced pluripotent stem cell-derived cardiomyocytes have been used to study long QT syndrome, catecholaminergic polymorphic ventricular tachycardia, hypertrophic cardiomyopathy, and other hereditary cardiac disorders. Although induced pluripotent stem cell-derived cardiomyocytes are distinct from adult cardiomyocytes, they provide a robust platform to advance the science and clinical care of sudden cardiac death.

中文翻译：

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寻找心源性猝死的节奏：使用诱导性多能干细胞衍生的心肌细胞的新机会。

心脏性猝死是结构性心脏病，遗传突变或影响心脏离子通道的获得性疾病患者的常见死亡原因。存在各种各样的平台来建模和研究与心脏猝死相关的疾病。人体临床研究很麻烦，并且受可对人体进行的研究程度所阻碍。动物模型受其与人心脏电生理的同源程度（包括离子通道表达）的限制。最常用的细胞模型是细胞转染模型，该模型能够模拟单离子通道的表达，从而提供对动作电位曲线变化的不完全了解。诱导的多能干细胞衍生的心肌细胞类似于但不相同，成人心肌细胞，并为研究导致心源性猝死的心律失常提供了新的平台。存在多种用于表型细胞模型的平台，包括常规的和自动的膜片钳，多电极阵列和计算模型。诱导多能干细胞衍生的心肌细胞已被用于研究长QT综合征，儿茶酚胺能性多形性室性心动过速，肥厚型心肌病和其他遗传性心脏病。尽管诱导的多能干细胞衍生的心肌细胞与成年心肌细胞不同，但它们提供了一个强大的平台来推进心脏猝死的科学和临床治疗。包括常规和自动膜片钳，多电极阵列以及计算模型。诱导多能干细胞衍生的心肌细胞已被用于研究长QT综合征，儿茶酚胺能性多形性室性心动过速，肥厚型心肌病和其他遗传性心脏病。尽管诱导的多能干细胞衍生的心肌细胞与成年心肌细胞不同，但它们提供了一个强大的平台来推进心脏猝死的科学和临床治疗。包括常规和自动膜片钳，多电极阵列以及计算模型。诱导多能干细胞衍生的心肌细胞已被用于研究长QT综合征，儿茶酚胺能性多形性室性心动过速，肥厚型心肌病和其他遗传性心脏病。尽管诱导的多能干细胞衍生的心肌细胞与成年心肌细胞不同，但它们提供了一个强大的平台来推进心脏猝死的科学和临床治疗。