Human iPSC-derived cardiomyocytes offer a promising, patient-specific in vitro platform for modeling inherited arrhythmias and testing novel therapeutics, though their immature electrophysiological phenotype remains a key limitation.
Inherited arrhythmia disorders (IADs) are a group of potentially lethal diseases that remain diagnostic and management challenges. Although the genetic basis for many of these disorders is well known, the pathogenicity of individual mutations and the resulting clinical outcomes are difficult to predict. Treatment options remain imperfect, and optimizing therapy for individual patients can be difficult. Recent advances in the derivation of induced pluripotent stem cells (iPSCs) from patients and creation of genetically engineered human models using CRISPR/Cas9 has the potential to dramatically advance translational arrhythmia research. In this review, we discuss the current state of modeling IADs using human iPSC-derived cardiomyocytes. We also discuss current limitations and areas for further study.
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Bezzerides et al. (2016) studied this question.
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