Related Experiment Video
Updated: Jun 3, 2026

Assessment of Sarcoplasmic Reticulum Calcium Reserve and Intracellular Diastolic Calcium Removal in Isolated Ventricular Cardiomyocytes
Published on: September 18, 2017
Inherited dysfunction of sarcoplasmic reticulum Ca2+ handling and arrhythmogenesis
Silvia G Priori1, S R Wayne Chen
1Molecular Cardiology Laboratories, IRCCS Fondazione Salvatore Maugeri, Via Maugeri 10/10A, Pavia, Italy. silvia.priori@fsm.it
None:
Catecholaminergic polymorphic ventricular tachycardia (CPVT) is an inherited arrhythmogenic disease occurring in patients with a structurally normal heart: the disease is characterized by life-threatening arrhythmias elicited by stress and emotion. In 2001, the ryanodine receptor was identified as the gene that is linked to CPVT; shortly thereafter, cardiac calsequestrin was implicated in the recessive form of the same disease. It became clear that abnormalities in intracellular Ca(2+) regulation could profoundly disrupt the electrophysiological properties of the heart. In this article, we discuss the molecular basis of the disease and the pathophysiological mechanisms that are impacting clinical diagnosis and management of affected individuals. As of today, the interaction between basic scientists and clinicians to understand CPVT and identify new therapeutic strategies is one of the most compelling examples of the importance of translational research in cardiology.
More Related Videos
09:36Dual-Dye Optical Mapping of Hearts from RyR2R2474S Knock-In Mice of Catecholaminergic Polymorphic Ventricular Tachycardia
Published on: December 22, 2023
09:26Optical Mapping of Intra-Sarcoplasmic Reticulum Ca2+ and Transmembrane Potential in the Langendorff-perfused Rabbit Heart
Published on: September 10, 2015
Related Concept Videos
Mechanism of Cardiac Arrhythmias
Cardiomyopathy I: Introduction and Classification
Cardiomyopathy III: Hypertrophic Cardiomyopathy
Disturbances in Heart Rhythm
Arrhythmias are categorized by their speed, rhythm, and origin. A slow heart...
Dysrhythmias I: Introduction
Cardiomyopathy IV: Restrictive Cardiomyopathy