Key result
Dantrolene restored normal Ca(2+) spark properties and rescued the arrhythmogenic phenotype in iPSC-derived cardiomyocytes from a CPVT patient with a novel RYR2 S406L mutation.
Why the study?
Does dantrolene rescue the arrhythmogenic phenotype in iPSC-derived cardiomyocytes from a CPVT patient with an RYR2 S406L mutation?
Population
Induced pluripotent stem cell-derived cardiomyocytes from a CPVT patient carrying a novel RYR2 S406L mutation
Comparison
Dantrolene vs Control myocytes / untreated state
Design
Preclinical
Authors
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Dantrolene merits trial evaluation in RYR2-CPVT; leaves open clinical translation from iPSC models.
Does dantrolene rescue the arrhythmogenic phenotype in iPSC-derived cardiomyocytes from a CPVT patient with an RYR2 S406L mutation?
Dantrolene rescues the arrhythmogenic phenotype in a patient-specific iPSC model of CPVT with an RYR2 S406L mutation, suggesting defective inter-domain interactions as the pathomechanism.
Jung et al. (2011) studied Catecholaminergic polymorphic ventricular tachycardia (CPVT) (n=1). Dantrolene vs. Control myocytes / untreated state was evaluated on Ca(2+) spark properties and arrhythmogenic phenotype (DADs and arrhythmia susceptibility). Dantrolene restored normal Ca(2+) spark properties and rescued the arrhythmogenic phenotype in iPSC-derived cardiomyocytes from a CPVT patient with a novel RYR2 S406L mutation.
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