Journal
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
Volume 22, Issue 6, Pages -Publisher
MDPI
DOI: 10.3390/ijms22062825
Keywords
brugada syndrome; inherited cardiac arrhythmia; induced pluripotent stem cells; iPSC-cardiomyocytes; electrophysiology
Funding
- University of Antwerp (GOA)
- Research Foundation-Flanders (FWO, Belgium) [G.0356.17]
- European Research Council [Genomia-ERC-COG-2017-771945]
- FWO [1S24317N, 12R5610N]
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Brugada syndrome is an inherited cardiac arrhythmia that can lead to ventricular fibrillation and sudden cardiac death. The use of induced pluripotent stem cell-derived cardiomyocytes (iPSC-CMs) offers new opportunities for studying complex cardiac arrhythmia syndromes. iPSC-CMs are considered the best in vitro model for researching Brugada syndrome and other arrhythmias.
Brugada syndrome (BrS) is an inherited cardiac arrhythmia that predisposes to ventricular fibrillation and sudden cardiac death. It originates from oligogenic alterations that affect cardiac ion channels or their accessory proteins. The main hurdle for the study of the functional effects of those variants is the need for a specific model that mimics the complex environment of human cardiomyocytes. Traditionally, animal models or transient heterologous expression systems are applied for electrophysiological investigations, each of these models having their limitations. The ability to create induced pluripotent stem cell-derived cardiomyocytes (iPSC-CMs), providing a source of human patient-specific cells, offers new opportunities in the field of cardiac disease modelling. Contemporary iPSC-CMs constitute the best possible in vitro model to study complex cardiac arrhythmia syndromes such as BrS. To date, thirteen reports on iPSC-CM models for BrS have been published and with this review we provide an overview of the current findings, with a focus on the electrophysiological parameters. We also discuss the methods that are used for cell derivation and data acquisition. In the end, we critically evaluate the knowledge gained by the use of these iPSC-CM models and discuss challenges and future perspectives for iPSC-CMs in the study of BrS and other arrhythmias.
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