Molecular and tissue mechanisms of catecholaminergic polymorphic ventricular tachycardia

MJ Wleklinski, PJ Kannankeril… - The Journal of …, 2020 - Wiley Online Library
Catecholaminergic polymorphic ventricular tachycardia (CPVT) is a stress‐induced cardiac
channelopathy that has a high mortality in untreated patients. Our understanding has grown …

Redox dependent modifications of ryanodine receptor: basic mechanisms and implications in heart diseases

R Nikolaienko, E Bovo, AV Zima - Frontiers in physiology, 2018 - frontiersin.org
Heart contraction vitally depends on tightly controlled intracellular Ca regulation. Because
contraction is mainly driven by Ca released from the sarcoplasmic reticulum (SR), this …

Cardiac ryanodine receptor calcium release deficiency syndrome

B Sun, J Yao, M Ni, J Wei, X Zhong, W Guo… - Science translational …, 2021 - science.org
Cardiac ryanodine receptor (RyR2) gain-of-function mutations cause catecholaminergic
polymorphic ventricular tachycardia, a condition characterized by prominent ventricular …

Gene Therapy for Catecholaminergic Polymorphic Ventricular Tachycardia by Inhibition of Ca2+/Calmodulin-Dependent Kinase II

VJ Bezzerides, A Caballero, S Wang, Y Ai, RJ Hylind… - Circulation, 2019 - Am Heart Assoc
Background: Catecholaminergic polymorphic ventricular tachycardia (CPVT), an inherited
cardiac arrhythmia characterized by adrenergically triggered arrhythmias, is inadequately …

An International Multicenter Evaluation of Inheritance Patterns, Arrhythmic Risks, and Underlying Mechanisms of CASQ2-Catecholaminergic Polymorphic Ventricular …

K Ng, EW Titus, KV Lieve, TM Roston, A Mazzanti… - Circulation, 2020 - Am Heart Assoc
Background: Genetic variants in calsequestrin-2 (CASQ2) cause an autosomal recessive
form of catecholaminergic polymorphic ventricular tachycardia (CPVT), although isolated …

Precision Medicine in Catecholaminergic Polymorphic Ventricular Tachycardia: JACC Focus Seminar 5/5

SG Priori, A Mazzanti, DJ Santiago, D Kukavica… - Journal of the American …, 2021 - jacc.org
In this final of a 5-part Focus Seminar series on precision medicine, we focus on
catecholaminergic polymorphic ventricular tachycardia (CPVT). This focus on CPVT allows …

AAV-mediated delivery of Plakophilin-2a arrests progression of arrhythmogenic right ventricular cardiomyopathy in murine hearts: preclinical evidence supporting …

CJM van Opbergen, B Narayanan… - Circulation: Genomic …, 2024 - Am Heart Assoc
BACKGROUND: Pathogenic variants in PKP2 (plakophilin-2) cause arrhythmogenic right
ventricular cardiomyopathy, a disease characterized by life-threatening arrhythmias and …

The CaMKII inhibitor KN93-calmodulin interaction and implications for calmodulin tuning of NaV1. 5 and RyR2 function

CN Johnson, R Pattanayek, F Potet, RT Rebbeck… - Cell Calcium, 2019 - Elsevier
Here we report the structure of the widely utilized calmodulin (CaM)-dependent protein
kinase II (CaMKII) inhibitor KN93 bound to the Ca 2+-sensing protein CaM. KN93 is widely …

Ca2+-CaM Dependent Inactivation of RyR2 Underlies Ca2+ Alternans in Intact Heart

J Wei, J Yao, D Belke, W Guo, X Zhong, B Sun… - Circulation …, 2021 - Am Heart Assoc
Rationale: Ca2+ alternans plays an essential role in cardiac alternans that can lead to
ventricular fibrillation, but the mechanism underlying Ca2+ alternans remains undefined …

Proarrhythmic remodeling of calcium homeostasis in cardiac disease; implications for diabetes and obesity

S Hamilton, D Terentyev - Frontiers in physiology, 2018 - frontiersin.org
A rapid growth in the incidence of diabetes and obesity has transpired to a major heath
issue and economic burden in the postindustrial world, with more than 29 million patients …