ArticleFrontiers in physiology2012
Induced pluripotent stem cell derived cardiomyocytes as models for cardiac arrhythmias.
Article in Frontiers in physiology, 2012. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 117 papers.
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Who cites it
117 citing papers in PubMed, 207 citations in OpenAlex.
- Flexible Sensing Technology for Myocardial Tissue Contractile Force With Integrated Magnetically Actuated Mechanical Stimulation.Advanced healthcare materials · 2026Article
- Modular bioreactor for multi-well electrical stimulation ofLab on a chip · 2026Article
- Biomimetic electrospun scaffolds for engineered heart tissue: from design parameters to drug testing platforms.Frontiers in bioengineering and biotechnology · 2026Review
- Advanced cardiotoxicity profiling using field potential imaging with UHD-CMOS-MEA in human iPSC-derived cardiomyocytes.Toxicological sciences : an official journal of the Society of Toxicology · 2025Article
- Decoding Cardiac Maturation Program: Insights from RNA Splicing Regulation.Current cardiology reports · 2025Review
- Review
- Modelling neurocardiac physiology and diseases using human pluripotent stem cells: current progress and future prospects.The Journal of physiology · 2025Review
- Alleviating the Effects of Short QT Syndrome Type 3 by Allele-Specific Suppression of theInternational journal of molecular sciences · 2024Article
- The Current State of Realistic Heart Models for Disease Modelling and Cardiotoxicity.International journal of molecular sciences · 2024Review
- hiPSC-CM electrophysiology: impact of temporal changes and study parameters on experimental reproducibility.American journal of physiology. Heart and circulatory physiology · 2024Article
- A red-emitting carborhodamine for monitoring and measuring membrane potential.Proceedings of the National Academy of Sciences of the United States of America · 2024Article
- Human Induced Pluripotent Spheroids' Growth Is Driven by Viscoelastic Properties and Macrostructure of 3D Hydrogel Environment.Bioengineering (Basel, Switzerland) · 2023Article
- A red-emitting carborhodamine for monitoring and measuring membrane potential.bioRxiv : the preprint server for biology · 2023Article
- New drug discovery of cardiac anti-arrhythmic drugs: insights in animal models.Scientific reports · 2023Article
- Article
- Adapting to a new environment: postnatal maturation of the human cardiomyocyte.The Journal of physiology · 2023Review
- Article
- Pro-arrhythmic effects of gain-of-function potassium channel mutations in the short QT syndrome.Philosophical transactions of the Royal Society of London. Series B, Biological sciences · 2023Review
- Electrophysiological and calcium-handling development during long-term culture of human-induced pluripotent stem cell-derived cardiomyocytes.Basic research in cardiology · 2023Article
- Maturation of hiPSC-derived cardiomyocytes promotes adult alternative splicing of SCN5A and reveals changes in sodium current associated with cardiac arrhythmia.Cardiovascular research · 2023Article
57 more citing papers are in PubMed but not listed here.
Corrections and comments
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Authors and funding
5 authors at 2 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Cardiac arrhythmias are a major cause of morbidity and mortality. In younger patients, the majority of sudden cardiac deaths have an underlying Mendelian genetic cause. Over the last 15 years, enormous progress has been made in identifying the distinct clinical phenotypes and in studying the basic cellular and genetic mechanisms associated with the primary Mendelian (monogenic) arrhythmia syndromes. Investigation of the electrophysiological consequences of an ion channel mutation is ideally done in the native cardiomyocyte (CM) environment. However, the majority of such studies so far have relied on heterologous expression systems in which single ion channel genes are expressed in non-cardiac cells. In some cases, transgenic mouse models have been generated, but these also have significant shortcomings, primarily related to species differences. The discovery that somatic cells can be reprogrammed to pluripotency as induced pluripotent stem cells (iPSC) has generated much interest since it presents an opportunity to generate patient- and disease-specific cell lines from which normal and diseased human CMs can be obtained These genetically diverse human model systems can be studied in vitro and used to decipher mechanisms of disease and identify strategies and reagents for new therapies. Here, we review the present state of the art with respect to cardiac disease models already generated using IPSC technology and which have been (partially) characterized. Human iPSC (hiPSC) models have been described for the cardiac arrhythmia syndromes, including LQT1, LQT2, LQT3-Brugada Syndrome, LQT8/Timothy syndrome and catecholaminergic polymorphic ventricular tachycardia (CPVT). In most cases, the hiPSC-derived cardiomyoctes recapitulate the disease phenotype and have already provided opportunities for novel insight into cardiac pathophysiology. It is expected that the lines will be useful in the development of pharmacological agents for the management of these disorders.
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Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.