Abnormal termination of Ca2+ release is a common defect of RyR2 mutations associated with cardiomyopathies
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TMEM43 mutation p.S358L alters intercalated disc protein expression and reduces conduction velocity in arrhythmogenic right ventricular cardiomyopathyExome Analyses of Long QT Syndrome Reveal Candidate Pathogenic Mutations in Calmodulin-Interacting GenesHeterogeneity of ryanodine receptor dysfunction in a mouse model of catecholaminergic polymorphic ventricular tachycardiaPolycystin-2 mutations lead to impaired calcium cycling in the heart and predispose to dilated cardiomyopathyLeaky RyR2 channels unleash a brainstem spreading depolarization mechanism of sudden cardiac death.RNA-binding protein RBM20 represses splicing to orchestrate cardiac pre-mRNA processing.Generation and characterization of a mouse model harboring the exon-3 deletion in the cardiac ryanodine receptor.The H29D Mutation Does Not Enhance Cytosolic Ca2+ Activation of the Cardiac Ryanodine ReceptorArray comparative genomic hybridization identifies a heterozygous deletion of exon 3 of the RYR2 geneCytosolic Ca²⁺ buffering determines the intra-SR Ca²⁺ concentration at which cardiac Ca²⁺ sparks terminateInvestigation of Pathogenic Genes in Chinese sporadic Hypertrophic Cardiomyopathy Patients by Whole Exome SequencingThe EF-hand Ca2+ Binding Domain Is Not Required for Cytosolic Ca2+ Activation of the Cardiac Ryanodine Receptor.Abnormal Ca(2+) homeostasis, atrial arrhythmogenesis, and sinus node dysfunction in murine hearts modeling RyR2 modificationThe expression of Lamin A mutant R321X leads to endoplasmic reticulum stress with aberrant Ca2+ handling.N-terminus oligomerization is conserved in intracellular calcium release channels.Inherited calcium channelopathies in the pathophysiology of arrhythmias.Regulation of RYR2 by sarcoplasmic reticulum Ca(2+).Traditional vs. genetic pathogenesis of arrhythmogenic right ventricular cardiomyopathy.Recent Developments in Heart Failure.Murine Electrophysiological Models of Cardiac Arrhythmogenesis.The Cytoplasmic Region of Inner Helix S6 Is an Important Determinant of Cardiac Ryanodine Receptor Channel Gating.Enhanced Cytosolic Ca2+ Activation Underlies a Common Defect of Central Domain Cardiac Ryanodine Receptor Mutations Linked to Arrhythmias.Oxidation of RyR2 Has a Biphasic Effect on the Threshold for Store Overload-Induced Calcium Release.Extensive Ca2+ leak through K4750Q cardiac ryanodine receptors caused by cytosolic and luminal Ca2+ hypersensitivity.The N-Terminal Region of the Ryanodine Receptor Affects Channel Activation.Roles of the NH2-terminal domains of cardiac ryanodine receptor in Ca2+ release activation and termination.Role of Cys³⁶⁰² in the function and regulation of the cardiac ryanodine receptor.Crystal structures of wild type and disease mutant forms of the ryanodine receptor SPRY2 domain.CPVT-associated cardiac ryanodine receptor mutation G357S with reduced penetrance impairs Ca2+ release termination and diminishes protein expression.The CPVT-associated RyR2 mutation G230C enhances store overload-induced Ca2+ release and destabilizes the N-terminal domains.Reduced Expression of Cardiac Ryanodine Receptor Protects against Stress-induced Ventricular Tachyarrhythmia, but Increases the Susceptibility to Cardiac Alternans.Simultaneous Measurement of Contraction and Calcium Transients in Stem Cell Derived Cardiomyocytes.Control of cardiac ryanodine receptor by sarcoplasmic reticulum luminal Ca2.Reduced threshold for store overload-induced Ca2+ release is a common defect of RyR1 mutations associated with malignant hyperthermia and central core disease.Exon 3 deletion of ryanodine receptor causes left ventricular noncompaction, worsening catecholaminergic polymorphic ventricular tachycardia, and sudden cardiac arrest.Antiarrhythmic Effects of Carvedilol and Flecainide in Cardiomyocytes Derived from Catecholaminergic Polymorphic Ventricular Tachycardia Patients.Exploring the Crosstalk Between and Splicing Machinery Gene Mutations in Dilated Cardiomyopathy
P2860
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P2860
Abnormal termination of Ca2+ release is a common defect of RyR2 mutations associated with cardiomyopathies
description
2012 nî lūn-bûn
@nan
2012年の論文
@ja
2012年論文
@yue
2012年論文
@zh-hant
2012年論文
@zh-hk
2012年論文
@zh-mo
2012年論文
@zh-tw
2012年论文
@wuu
2012年论文
@zh
2012年论文
@zh-cn
name
Abnormal termination of Ca2+ r ...... sociated with cardiomyopathies
@ast
Abnormal termination of Ca2+ r ...... sociated with cardiomyopathies
@en
type
label
Abnormal termination of Ca2+ r ...... sociated with cardiomyopathies
@ast
Abnormal termination of Ca2+ r ...... sociated with cardiomyopathies
@en
prefLabel
Abnormal termination of Ca2+ r ...... sociated with cardiomyopathies
@ast
Abnormal termination of Ca2+ r ...... sociated with cardiomyopathies
@en
P2093
P2860
P1433
P1476
Abnormal termination of Ca2+ r ...... sociated with cardiomyopathies
@en
P2093
Michael Fill
Ruiwu Wang
S R Wayne Chen
Yijun Tang
P2860
P304
P356
10.1161/CIRCRESAHA.111.256560
P577
2012-02-28T00:00:00Z