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. 2016 Dec 14;97(1):283–409. doi: 10.1152/physrev.00007.2016

Table 7.

Transgenic murine models for catecholaminergic polymorphic ventricular tachycardia (CPVT)

Gene Genetic Modification Phenotype Reference Nos.
RyR2 (NH2-terminal region) RyR2-R176Q/+ CPVT (ARVC2) 534
RyR2-R420W CPVT 851
RyR2 (central region) RyR2-P2328S/+ and RyR2-P2328S/P2328S CPVT 364, 1340
RyR2-P2328S/P2328S AF 569, 832, 996, 1334, 1337
RyR2-R2474S/+ CPVT 585, 629, 1156
RyR2 (COOH-terminal region) RyR2-R4496C/+ CPVT 172, 673
Casq2 Casq2-D307H overexpression CPVT 266
Casq2−/− CPVT and cardiac hypertrophy 581
Casq2+/− No phenotype 199
Casq2-D307H/D307H Spontaneous arrhythmias 1078
Casq2-ΔE9/ΔE9 Spontaneous arrhythmias; cardiac hypertrophy with age 1077
Casq2-R33Q/R33Q Spontaneous arrhythmias 951