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• W2118098701 abstract "Rationale : Catecholaminergic polymorphic ventricular tachycardia (CPVT) is caused by a single point mutation in a well-defined region of the cardiac type 2 ryanodine receptor (RyR)2. However, the underlying mechanism by which a single mutation in such a large molecule produces drastic effects on channel function remains unresolved. Objective : Using a knock-in (KI) mouse model with a human CPVT-associated RyR2 mutation (R2474S), we investigated the molecular mechanism by which CPVT is induced by a single point mutation within the RyR2. Methods and Results : The R2474S/+ KI mice showed no apparent structural or histological abnormalities in the heart, but they showed clear indications of other abnormalities. Bidirectional or polymorphic ventricular tachycardia was induced after exercise on a treadmill. The interaction between the N-terminal (amino acids 1 to 600) and central (amino acids 2000 to 2500) domains of the RyR2 (an intrinsic mechanism to close Ca 2+ channels) was weakened (domain unzipping). On protein kinase A-mediated phosphorylation of the RyR2, this domain unzipping further increased, resulting in a significant increase in the frequency of spontaneous Ca 2+ transients. cAMP-induced aberrant Ca 2+ release events (Ca 2+ sparks/waves) occurred at much lower sarcoplasmic reticulum Ca 2+ content as compared to the wild type. Addition of a domain-unzipping peptide, DPc10 (amino acids 2460 to 2495), to the wild type reproduced the aforementioned abnormalities that are characteristic of the R2474S/+ KI mice. Addition of DPc10 to the (cAMP-treated) KI cardiomyocytes produced no further effect. Conclusions : A single point mutation within the RyR2 sensitizes the channel to agonists and reduces the threshold of luminal [Ca 2+ ] for activation, primarily mediated by defective interdomain interaction within the RyR2." @default.
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• W2118098701 date "2010-04-30" @default.
• W2118098701 modified "2023-10-17" @default.
• W2118098701 title "Catecholaminergic Polymorphic Ventricular Tachycardia Is Caused by Mutation-Linked Defective Conformational Regulation of the Ryanodine Receptor" @default.
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• W2118098701 doi "https://doi.org/10.1161/circresaha.109.209312" @default.
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