Molecular, subcellular, and arrhythmogenic mechanisms in genetic RyR2 disease

ED Fowler, S Zissimopoulos - Biomolecules, 2022 - mdpi.com
The ryanodine receptor (RyR2) has a critical role in controlling Ca2+ release from the
sarcoplasmic reticulum (SR) throughout the cardiac cycle. RyR2 protein has multiple …

[HTML][HTML] Cardiac disease and arrhythmogenesis: Mechanistic insights from mouse models

L Choy, JM Yeo, V Tse, SP Chan, G Tse - IJC heart & vasculature, 2016 - Elsevier
The mouse is the second mammalian species, after the human, in which substantial amount
of the genomic information has been analyzed. With advances in transgenic technology …

Increased RyR2 activity is exacerbated by calcium leak-induced mitochondrial ROS

S Hamilton, R Terentyeva, B Martin, F Perger… - Basic research in …, 2020 - Springer
Cardiac disease is associated with deleterious emission of mitochondrial reactive oxygen
species (mito-ROS), as well as enhanced oxidation and activity of the sarcoplasmic …

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 …

Evaluation of changes in morphology and function of human induced pluripotent stem cell derived cardiomyocytes (HiPSC-CMs) cultured on an aligned-nanofiber …

M Khan, Y Xu, S Hua, J Johnson, A Belevych… - PloS one, 2015 - journals.plos.org
Introduction Dilated cardiomyopathy is a major cause of progressive heart failure. Utilization
of stem cell therapy offers a potential means of regenerating viable cardiac tissue. However …

Superresolution modeling of calcium release in the heart

MA Walker, GSB Williams, T Kohl, SE Lehnart… - Biophysical …, 2014 - cell.com
Stable calcium-induced calcium release (CICR) is critical for maintaining normal cellular
contraction during cardiac excitation-contraction coupling. The fundamental element of …

Pharmacological Modulation of Mitochondrial Ca2+ Content Regulates Sarcoplasmic Reticulum Ca2+ Release via Oxidation of the Ryanodine Receptor by …

S Hamilton, R Terentyeva, TY Kim, P Bronk… - Frontiers in …, 2018 - frontiersin.org
In a physiological setting, mitochondria increase oxidative phosphorylation during periods of
stress to meet increased metabolic demand. This in part is mediated via enhanced …

[HTML][HTML] Altered calcium handling produces reentry-promoting action potential alternans in atrial fibrillation–remodeled hearts

T Liu, F Xiong, XY Qi, J Xiao, L Villeneuve, I Abu-Taha… - JCI insight, 2020 - ncbi.nlm.nih.gov
Atrial fibrillation (AF) alters atrial cardiomyocyte (ACM) Ca 2+ handling, promoting ectopic
beat formation. We examined the effects of AF-associated remodeling on Ca 2+-related …

MCU overexpression evokes disparate dose-dependent effects on mito-ROS and spontaneous Ca2+ release in hypertrophic rat cardiomyocytes

S Hamilton, R Terentyeva, F Perger… - American Journal …, 2021 - journals.physiology.org
Cardiac dysfunction in heart failure (HF) and diabetic cardiomyopathy (DCM) is associated
with aberrant intracellular Ca2+ handling and impaired mitochondrial function accompanied …

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 …