The Advantages, Challenges, and Future of Human-Induced Pluripotent Stem Cell Lines in Type 2 Long QT Syndrome.

Abstract

Type 2 long QT syndrome (LQT2) is the second most common subtype of long QT syndrome and is caused by mutations in KCHN2 encoding the rapidly activating delayed rectifier potassium channel vital for ventricular repolarization. Sudden cardiac death is a sentinel event of LQT2. Preclinical diagnosis by genetic testing is potentially life-saving.Traditional LQT2 models cannot wholly recapitulate genetic and phenotypic features; therefore, there is a demand for a reliable experimental model. Human-induced pluripotent stem cell-derived cardiomyocytes (hiPSC-CMs) meet this challenge. This review introduces the advantages of the hiPSC-CM model over the traditional model and discusses how hiPSC-CM and gene editing are used to decipher mechanisms of LQT2, screen for cardiotoxicity, and identify therapeutic strategies, thus promoting the realization of precision medicine for LQT2 patients.

• Authors: Dihui Cai, Zequn Zheng, Xiaojun Jin, Yin Fu, Lichao Cen, Jiachun Ye, Yongfei Song, Jiangfang Lian
• Journal: Journal of cardiovascular translational research (J Cardiovasc Transl Res) Vol. 16 Issue 1 Pg. 209-220 (02 2023) ISSN: 1937-5395 [Electronic] United States
• PMID: 35976484 (Publication Type: Journal Article, Review, Research Support, Non-U.S. Gov't)
• Copyright: © 2022. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.

Chemical References

• ERG1 Potassium Channel

Topics

• Humans
• Induced Pluripotent Stem Cells (metabolism)
• Long QT Syndrome (drug therapy, genetics)
• Mutation
• Genetic Testing
• Myocytes, Cardiac (metabolism)
• ERG1 Potassium Channel (genetics, metabolism)
• Action Potentials