Post-Translational Modifications and Diastolic Calcium Leak Associated to the Novel RyR2-D3638A Mutation Lead to CPVT in Patient-Specific hiPSC-Derived Cardiomyocytes

Post-Translational Modifications and Diastolic Calcium Leak Associated to the Novel RyR2-D3638A Mutation Lead to CPVT in Patient-Specific hiPSC-Derived Cardiomyocytes

Background: Sarcoplasmic reticulum Ca<sup>2+</sup> leak and post-translational modifications under stress have been implicated in catecholaminergic polymorphic ventricular tachycardia (CPVT), a highly lethal inherited arrhythmogenic disorder. Human induced pluripotent stem cells (hiPSCs)...

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Bibliographic Details
Main Authors: Ivana Acimovic, Marwan M. Refaat, Adrien Moreau, Anton Salykin, Steve Reiken, Yvonne Sleiman, Monia Souidi, Jan Přibyl, Andrey V. Kajava, Sylvain Richard, Jonathan T. Lu, Philippe Chevalier, Petr Skládal, Petr Dvořak, Vladimir Rotrekl, Andrew R. Marks, Melvin M. Scheinman, Alain Lacampagne, Albano C. Meli
Format: Article
Language:English
Published: MDPI AG 2018-11-01
Series:Journal of Clinical Medicine
Subjects:
ryanodine receptor
CPVT
hiPSC-derived cardiomyocytes
calcium
β-adrenergic receptor blockade
flecainide
post-translational modifications
Online Access:https://www.mdpi.com/2077-0383/7/11/423

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https://www.mdpi.com/2077-0383/7/11/423

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