Efficacy of RyR2 inhibitor EL20 in induced pluripotent stem cell‐derived cardiomyocytes from a patient with catecholaminergic polymorphic ventricular tachycardia

TA Word, AP Quick, CY Miyake… - Journal of cellular …, 2021 - Wiley Online Library
Catecholaminergic polymorphic ventricular tachycardia (CPVT) is an inherited cardiac
arrhythmia syndrome that often leads to sudden cardiac death. The most common form of …

Pernicious attrition and inter-RyR2 CICR current control in cardiac muscle

D Gillespie, M Fill - Biophysical Journal, 2013 - cell.com
In cardiac muscle cells, ryanodine receptor (RyR) mediated Ca release from the
sarcoplasmic reticulum (SR) drives the contractile apparatus. Spontaneous bouts of inter …

An inherited life-threatening arrhythmia model established by screening randomly mutagenized mice

Y Okabe, N Murakoshi… - Proceedings of the …, 2024 - National Acad Sciences
Inherited arrhythmia syndromes (IASs) can cause life-threatening arrhythmias and are
responsible for a significant proportion of sudden cardiac deaths (SCDs). Despite progress …

Unnatural verticilide enantiomer inhibits type 2 ryanodine receptor-mediated calcium leak and is antiarrhythmic

SM Batiste, DJ Blackwell, K Kim… - Proceedings of the …, 2019 - National Acad Sciences
Ca2+ leak via ryanodine receptor type 2 (RyR2) can cause potentially fatal arrhythmias in a
variety of heart diseases and has also been implicated in neurodegenerative and seizure …

Accelerated development of pressure overload–induced cardiac hypertrophy and dysfunction in an RyR2-R176Q knockin mouse model

RJ van Oort, JL Respress, N Li, C Reynolds… - …, 2010 - Am Heart Assoc
In response to chronic hypertension, the heart compensates by hypertrophic growth, which
frequently progresses to heart failure. Although intracellular calcium (Ca2+) has a central …

[HTML][HTML] Ventricular arrhythmogenesis: insights from murine models

IN Sabir, MJ Killeen, AA Grace, CLH Huang - Progress in biophysics and …, 2008 - Elsevier
Ventricular arrhythmias are the key underlying cause of sudden cardiac death, a common
cause of mortality and a significant public health burden. Insights into the …

Ventricular voltage‐gated ion channels: Detection, characteristics, mechanisms, and drug safety evaluation

L Chen, Y He, X Wang, J Ge, H Li - Clinical and Translational …, 2021 - Wiley Online Library
Cardiac voltage‐gated ion channels (VGICs) play critical roles in mediating cardiac
electrophysiological signals, such as action potentials, to maintain normal heart excitability …

Atrial arrhythmia, triggering events and conduction abnormalities in isolated murine RyR2‐P2328S hearts

JH King, Y Zhang, M Lei, AA Grace… - Acta …, 2013 - Wiley Online Library
Aim R y R 2 mutations are associated with catecholaminergic polymorphic tachycardia, a
condition characterized by ventricular and atrial arrhythmias. The present experiments …

[HTML][HTML] MIR448 antagomir reduces arrhythmic risk after myocardial infarction by upregulating the cardiac sodium channel

GJ Kang, A Xie, H Liu, SC Dudley Jr - JCI insight, 2020 - ncbi.nlm.nih.gov
Cardiac ischemia is associated with arrhythmias; however, effective therapies are currently
limited. The cardiac voltage-gated sodium channel α subunit (SCN5A), encoding the Na v …

Abnormal propagation of calcium waves and ultrastructural remodeling in recessive catecholaminergic polymorphic ventricular tachycardia

N Liu, M Denegri, W Dun, S Boncompagni… - Circulation …, 2013 - Am Heart Assoc
Rationale: The recessive form of catecholaminergic polymorphic ventricular tachycardia is
caused by mutations in the cardiac calsequestrin-2 gene; this variant of catecholaminergic …