Associated Papers
Ryanodine receptor 2 mutations in catecholaminergic polymorphic ventricular tachycardia: From molecular mechanisms to precision medicine.
Sharma V
World Journal of Cardiology, 2026
Human-engineered heart tissues recapitulate tissue-scale mechanisms underlying ventricular tachycardia.
Fiedler M et al.
Biorxiv : the Preprint Server for Biology, 2026
Caenorhabditis elegans as an in vivo model system for human inherited primary arrhythmia syndromes.
Delinière A et al.
The Journal of Physiology, 2025
Disruption of ventricular activation by subthreshold delayed afterdepolarizations in RyR2-R420Q catecholaminergic polymorphic ventricular tachycardia.
Fowler ED et al.
Journal of Molecular and Cellular Cardiology Plus, 2025
RYR2 Variants in Catecholaminergic Polymorphic Ventricular Tachycardia Patients: Insights From Protein Structure and Clinical Data.
Chang A et al.
Circulation. Arrhythmia and Electrophysiology, 2025
Catecholaminergic polymorphic ventricular tachycardia: A narrative review of recent advances in genetics, mechanisms, diagnosis, and treatment.
Luo S et al.
Asian Heart Journal, 2025
Interplay Between Ryanodine Receptor Arrangement and Function: Implications for (Patho)physiological Control of Calcium Release.
Gonano LA et al.
Circulation Research, 2025
SMIM20 promotes complex IV biogenesis and Ca2+ signaling in mice heart.
Boshnakovska A et al.
Cell Reports, 2025
Understanding CPVT pathogenic mechanisms based on mutation location within RyR2: towards personalized medicine?
Benitah JP et al.
The Journal of Physiology, 2025
Structural instability of ryanodine receptor 2 causes endoplasmic reticulum (ER) dysfunction as well as sarcoplasmic reticulum (SR) dysfunction.
Uchinoumi H et al.
Journal of Cardiology, 2025
Catecholaminergic polymorphic ventricular tachycardia-linked ryanodine receptor variants exhibit domain-specific calcium leak and calmodulin affinity properties.
Uchinoumi H et al.
The Journal of Physiology, 2025
Development of selective RyR2 inhibitors with a pharmacophore containing a parabanic acid skeleton.
Ishida R et al.
Rsc Medicinal Chemistry, 2025
Cardiac sympathetic neurons are additional cells affected in genetically determined arrhythmogenic cardiomyopathy.
Vanaja IP et al.
The Journal of Physiology, 2025
The Role of RyR2 Mutations in Congenital Heart Diseases: Insights Into Cardiac Electrophysiological Mechanisms.
Lv T et al.
Journal of Cardiovascular Electrophysiology, 2025
Dual calcium-voltage optical mapping of regional voltage and calcium signals in intact murine RyR2-R2474S hearts.
Li Y et al.
Journal of Molecular and Cellular Cardiology Plus, 2024
Treatment Outcomes in Children With Catecholaminergic Polymorphic Ventricular Tachycardia: A Single Institutional Experience.
Lee J et al.
Korean Circulation Journal, 2024
Structural basis for ryanodine receptor type 2 leak in heart failure and arrhythmogenic disorders.
Miotto MC et al.
Nature Communications, 2024
Propagation of conformational instability in FK506-binding protein FKBP12.
LeMaster DM et al.
Biochimica Et Biophysica Acta. Proteins and Proteomics, 2024
RYR2 receptor gene mutation associated with catecholaminergic polymorphic ventricular tachycardia in children: a case report & literature review.
Abdullah NM et al.
Translational Pediatrics, 2024
Discovery and Structure-Activity Relationship of a Ryanodine Receptor 2 Inhibitor.
Ishida R et al.
Chemical & Pharmaceutical Bulletin, 2024
Screening for Novel Type 2 Ryanodine Receptor Inhibitors by Endoplasmic Reticulum Ca2+ Monitoring.
Takenaka M et al.
Molecular Pharmacology, 2023
New drug discovery of cardiac anti-arrhythmic drugs: insights in animal models.
Sharma AK et al.
Scientific Reports, 2023
Cysteines 1078 and 2991 cross-linking plays a critical role in redox regulation of cardiac ryanodine receptor (RyR).
Nikolaienko R et al.
Nature Communications, 2023
Arrhythmogenic mechanism of a novel ryanodine receptor mutation underlying sudden cardiac death.
Qian Y et al.
Europace : European Pacing, Arrhythmias, and Cardiac Electrophysiology : Journal of the Working Groups on Cardiac Pacing, Arrhythmias, and Cardiac Cellular Electrophysiology of the European Society of Cardiology, 2023
Ryanodine receptor 2 promotes colorectal cancer metastasis by the ROS/BACH1 axis.
Chen T et al.
Molecular Oncology, 2023
Molecular Aspects Implicated in Dantrolene Selectivity with Respect to Ryanodine Receptor Isoforms.
Gaburjakova J et al.
International Journal of Molecular Sciences, 2023
Gene mutations in comorbidity of epilepsy and arrhythmia.
Yu C et al.
Journal of Neurology, 2023
Three-dimensional perspective on ryanodine receptor mutations causing skeletal and cardiac muscle-related diseases.
Iyer KA et al.
Current Opinion in Pharmacology, 2023
A gain of function ryanodine receptor 2 mutation (R1760W-RyR2) in catecholaminergic polymorphic ventricular tachycardia.
Li S et al.
Clinical and Experimental Pharmacology & Physiology, 2023
The Role of RYR2 in Atrial Fibrillation.
Boehm BM et al.
Case Reports in Cardiology, 2023
Cytosolic Ca2+-dependent Ca2+ release activity primarily determines the ER Ca2+ level in cells expressing the CPVT-linked mutant RYR2.
Kurebayashi N et al.
The Journal of General Physiology, 2022
Do CPVT-linked mutations alter RYR2 regulation by cytosolic Ca2+ in cardiomyocytes?
Yamaguchi N
The Journal of General Physiology, 2022
Z16b, a natural compound from Ganoderma cochlear is a novel RyR2 stabilizer preventing catecholaminergic polymorphic ventricular tachycardia.
Wan JF et al.
Acta Pharmacologica Sinica, 2022
Increased susceptibility to ventricular arrhythmia at low-normal and moderately low levels of extracellular potassium in catecholaminergic polymorphic ventricular tachycardia.
Robinson VM et al.
Heart Rhythm, 2022
Molecular, Subcellular, and Arrhythmogenic Mechanisms in Genetic RyR2 Disease.
Fowler ED et al.
Biomolecules, 2022
Structural analyses of human ryanodine receptor type 2 channels reveal the mechanisms for sudden cardiac death and treatment.
Miotto MC et al.
Science Advances, 2022
The role of junctophilin proteins in cellular function.
Lehnart SE et al.
Physiological Reviews, 2022
Animal Models to Study Cardiac Arrhythmias.
Blackwell DJ et al.
Circulation Research, 2022
Outcomes of Patients With Catecholaminergic Polymorphic Ventricular Tachycardia Treated With β-Blockers.
Mazzanti A et al.
Jama Cardiology, 2022
Molecular Changes in the Cardiac RyR2 With Catecholaminergic Polymorphic Ventricular Tachycardia (CPVT).
Dulhunty AF
Frontiers in Physiology, 2022
An Optogenetic Arrhythmia Model-Insertion of Several Catecholaminergic Polymorphic Ventricular Tachycardia Mutations Into Caenorhabditis elegans UNC-68 Disturbs Calstabin-Mediated Stabilization of the Ryanodine Receptor Homolog.
Engel MA et al.
Frontiers in Physiology, 2022
Identifying Features of Cardiac Disease Phenotypes Based on Mechanical Function in a Catecholaminergic Polymorphic Ventricular Tachycardia Model.
Stempien A et al.
Frontiers in Bioengineering and Biotechnology, 2022