ArticleCirculation. Arrhythmia and electrophysiology2009
Sudden infant death syndrome in mice with an inherited mutation in RyR2.
Article in Circulation. Arrhythmia and electrophysiology, 2009. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 18 papers.
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Who cites it
18 citing papers in PubMed, 26 citations in OpenAlex.
- An RYR2-interacting fragment of MYBPC3 mitigates arrhythmia in human iPSC-CM and mouse models of CPVT.Molecular therapy : the journal of the American Society of Gene Therapy · 2026Article
- Cardiac Genetic Investigation of Sudden Infant and Early Childhood Death: A Study From Victims to Families.Journal of the American Heart Association · 2023Article
- Sex and Gene Influence Arrhythmia Susceptibility in Murine Models of Calmodulinopathy.Circulation. Arrhythmia and electrophysiology · 2023Article
- Tocotrienols-enriched Self-nanoemulsifying Drug Delivery System Enhances the Antileukemic Activity of All-trans Retinoic Acid but not Electrocardiogram Alterations Evoked by Its Combination with Arsenic Trioxide.AAPS PharmSciTech · 2023Article
- Gene Therapy for Catecholaminergic Polymorphic Ventricular Tachycardia by Inhibition of CaCirculation · 2019Article
- In Vivo Ryr2 Editing Corrects Catecholaminergic Polymorphic Ventricular Tachycardia.Circulation research · 2018Article
- Alterations in the carnitine cycle in a mouse model of Rett syndrome.Scientific reports · 2017Article
- Leaky RyR2 channels unleash a brainstem spreading depolarization mechanism of sudden cardiac death.Proceedings of the National Academy of Sciences of the United States of America · 2016Article
- Cardiac BIN1 folds T-tubule membrane, controlling ion flux and limiting arrhythmia.Nature medicine · 2014Article
- An introduction to murine models of atrial fibrillation.Frontiers in physiology · 2012Article
- Cardiac ion channelopathies and the sudden infant death syndrome.ISRN cardiology · 2012Article
- Cardiac electrophysiology in mice: a matter of size.Frontiers in physiology · 2012Article
- Cardiac conduction system anomalies and sudden cardiac death: insights from murine models.Frontiers in physiology · 2012Article
- Pathogenesis of lethal cardiac arrhythmias in Mecp2 mutant mice: implication for therapy in Rett syndrome.Science translational medicine · 2011Article
- Targeting ryanodine receptors for anti-arrhythmic therapy.Acta pharmacologica Sinica · 2011Review
- Ryanodine receptor phosphorylation, calcium/calmodulin-dependent protein kinase II, and life-threatening ventricular arrhythmias.Trends in cardiovascular medicine · 2011Review
- Programmed electrical stimulation in mice.Journal of visualized experiments : JoVE · 2010Article
- The ryanodine receptor in cardiac physiology and disease.Advances in pharmacology (San Diego, Calif.) · 2010Review
Corrections and comments
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Authors and funding
10 authors at 1 institution in 1 country.
Funding
Abstract
backgroundMutations in the cardiac ryanodine receptor gene (RyR2) have been recently identified in victims of sudden infant death syndrome. The aim of this study was to determine whether a gain-of-function mutation in RyR2 increases the propensity to cardiac arrhythmias and sudden death in young mice. METHODS AND
resultsIncidence of sudden death was monitored prospectively in heterozygous knock-in mice with mutation R176Q in RyR2 (R176Q/+). Young R176Q/+ mice exhibited a higher incidence of sudden death compared with wild-type littermates. Optical mapping of membrane potentials and intracellular calcium in 1- to 7-day-old R176Q/+ and wild-type mice revealed an increased incidence of ventricular ectopy and spontaneous calcium releases in neonatal R176Q/+ mice. Surface ECGs in 3- to 10-day-old mice showed that R176Q/+ mice developed more ventricular arrhythmias after provocation with epinephrine and caffeine. Intracardiac pacing studies in 12- to 18-day-old mice revealed the presence of an arrhythmogenic substrate in R176Q/+ compared with wild-type mice. Reverse transcription-polymerase chain reaction and Western blotting showed that expression levels of other calcium handling proteins were unaltered, suggesting that calcium leak through mutant RyR2 underlies arrhythmogenesis and sudden death in young R176Q/+ mice.
conclusionsOur findings demonstrate that a gain-of-function mutation in RyR2 confers an increased risk of cardiac arrhythmias and sudden death in young mice and that young R176Q/+ mice may be used as a model for elucidating the complex interplay between genetic and environmental risk factors associated with sudden infant death syndrome.
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