Calmodulin Mutants Linked to Catecholaminergic Polymorphic Ventricular Tachycardia Fail to Inhibit Human RyR2 Channels.

Kafa Walweel,Nieves Gomez-Hurtado, Ye Wint Oo,Nicole A Beard,Cris Dos Remedios,Christopher N Johnson,Walter J Chazin,Dirk F van Helden,Björn C Knollmann,Derek R Laver

Journal of the American College of Cardiology（2017）

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摘要

Calmodulin (CaM) is a calcium-binding protein that can directly inhibit cardiac ryanodine receptor calcium release channels (ryanodine receptor 2 [RyR2]) [(1)][1]. CaM mutations can cause an autosomal-dominant form of catecholaminergic polymorphic ventricular tachycardia (CPVT), a syndrome

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