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Chinese Journal of Cell and Stem Cell(Electronic Edition) ›› 2026, Vol. 16 ›› Issue (04): 233-239. doi: 10.3877/cma.j.issn.2095-1221.2026.04.006

• Review • Previous Articles    

Advances in induced pluripotent stem cells-derived cardiomyocytes in inherited primary arrhythmia syndrome models

Mingqi Yan, Qiuling Xiang()   

  1. Zhongshan School of Medicine, Sun Yat-sen University, Guangzhou 510080, China
  • Received:2025-09-04 Online:2026-08-01 Published:2026-08-07
  • Contact: Qiuling Xiang

Abstract:

Inherited primary arrhythmia syndromes (IPAS) encompass a group of disorders stemming from genetic anomalies in cardiac ion channels or their regulatory proteins, frequently associated with an elevated risk of sudden cardiac death. These diseases manifest a broad spectrum of clinical presentations, ranging from asymptomatic states to syncope, arrhythmias, and conduction disturbances, potentially leading to unexplained sudden cardiac death in phenotypically healthy individuals. Induced pluripotent stem cells (iPSCs) and induced pluripotent stem cells-derived cardiomyocytes (iPSC-CMs) offer robust in vitro disease models. It not only helps to analyze the mechanism of patient-specific diseases, but also avoids species differences in animal models and restrictions on the acquisition of human tissue samples. This article briefly introduces the current research status of iPSCs and iPSC-CMs, and summarizes the progress in the construction of patient-specific iPSC-CMs models, drug screening, and mechanistic studies for long QT syndrome, Brugada syndrome, early repolarization syndrome, and catecholaminergic polymorphic ventricular tachycardia.

Key words: Inherited primary arrhythmia syndromes, Induced pluripotent stem cells, Cardiomyocytes, Disease model, Drug screening

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