Ryanodine receptor-bound calmodulin is essential to protect against catecholaminergic polymorphic ventricular tachycardia

• Published in: JCI insight Vol. 4; no. 11
• Main Authors: Nakamura, Yoshihide, Yamamoto, Takeshi, Kobayashi, Shigeki, Tamitani, Masaki, Hamada, Yoriomi, Fukui, Go, Xu, Xiaojuan, Nishimura, Shigehiko, Kato, Takayoshi, Uchinoumi, Hitoshi, Oda, Tetsuro, Okuda, Shinichi, Yano, Masafumi
• Format: Journal Article
• Language: English
• Published: United States American Society for Clinical Investigation 06.06.2019
• Subjects: Calcium channels; Cardiology; Arrhythmias; Calmodulin

Catecholaminergic polymorphic ventricular tachycardia (CPVT) is caused by a single point mutation in the cardiac type 2 ryanodine receptor (RyR2). Using a knockin (KI) mouse model (R2474S/+), we previously reported that a single point mutation within the RyR2 sensitizes the channel to agonists, prim...