Induced pluripotent stem cell technology and inherited arrhythmia syndromes

Induced pluripotent stem cell technology and inherited arrhythmia syndromes.

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Item Type: Article
Status: Published
Official URL:
Journal or Publication Title: Heart Rhythm
Volume: 15
Number: 1
Page Range: pp. 137-144
Date: 2018
Divisions: Molecular Cardiology
Depositing User: General Admin
Identification Number: 10.1016/j.hrthm.2017.08.013
ISSN: 15475271
Date Deposited: 04 Jan 2021 22:57

Inherited arrhythmia syndromes, including familial long QT syndrome, catecholaminergic polymorphic ventricular tachycardia, and Brugada syndrome, can cause life-threatening arrhythmias and are responsible for a significant proportion of sudden deaths in the young. Identification of genetic mutations and pathophysiological changes that underlie disease development can inform clinical practice and guide novel drug development. However, disease mechanisms in a large number of patients remain elusive and pharmacologic treatment is suboptimal, so many patients rely on implantable cardioverter–defibrillator therapy. Induced pluripotent stem cell models of disease facilitate analysis of disease mechanisms in patient-specific cardiomyocytes, overcoming limitations of animal models and human tissue restrictions. This review outlines how studies using induced pluripotent stem cell–derived cardiomyocytes are contributing to our understanding of the mechanisms that underpin disease pathogenesis and their potential to facilitate new pharmacologic therapies and personalized medicine.

Ross, Samantha Barratt
Fraser, Stuart T.
Semsarian, Christopher
Last Modified: 04 Jan 2021 22:57

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