作者: Bert Vandendriessche , Ewa Sieliwonczyk , Maaike Alaerts , Bart L. Loeys , Dirk Snyders
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摘要: Inherited cardiac arrhythmias contribute substantially to sudden death in the young. The underlying pathophysiology remains incompletely understood because of lack representative study models and labour-intensive nature electrophysiological patch clamp experiments. Whereas is still considered gold standard for investigating electrical properties a cell, optical mapping voltage calcium transients has paved way high-throughput studies. Moreover, development human-induced pluripotent stem-cell-derived cardiomyocytes (hiPSC-CMs) enabled patient specific cell lines capturing full genomic background. Nevertheless, hiPSC-CMs do not fully address complex interactions between various types heart. Studies using vivo models, are therefore necessary. Given analogies human zebrafish cardiovascular system, emerged as cost-efficient model arrhythmogenic diseases. In this review, we describe how hiPSC-CM employed primary disorders. We provide an overview contemporary phenotyping tools discuss more depth different strategies available mapping. consider current advantages disadvantages both tool propose further improvement. Overall, combination experimental readouts at cellular (hiPSC-CM) whole organ (zebrafish) level can raise our understanding complexity inherited arrhythmia disorders next level.