Article
The structure of a calsequestrin filament reveals mechanisms of familial arrhythmia.
Nature structural & molecular biology - 1 Dec 2020
Titus Erron W, Deiter Frederick H, Shi Chenxu, Wojciak Julianne, Scheinman Melvin, Jura Natalia, Deo Rahul C
Abstract excerpt
Mutations in the calcium-binding protein calsequestrin cause the highly lethal familial arrhythmia catecholaminergic polymorphic ventricular tachycardia (CPVT). In vivo, calsequestrin multimerizes into filaments, but there is not yet an atomic-resolution structure of a calsequestrin filament. We report a crystal structure of a human cardiac calsequestrin filament with supporting mutational analysis and in vitro...
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