Interplay between Triadin and Calsequestrin in the Pathogenesis of CPVT in the Mouse.

ABSTRACT: Recessive forms of catecholaminergic polymorphic ventricular tachycardia (CPVT) are induced by mutations in genes encoding triadin or calsequestrin, two proteins that belong to the Ca²⁺ release complex, responsible for intracellular Ca²⁺ release triggering cardiac contractions. To better understand the mechanisms of triadin-induced CPVT and to assay multiple therapeutic interventions, we used a triadin knockout mouse model presenting a CPVT-like phenotype associated with a decrease in calsequestrin protein level. We assessed different approaches to rescue protein expression and to correct intracellular Ca²⁺ release and cardiac function: pharmacological treatment with kifunensine or a viral gene transfer-based approach, using adeno-associated virus serotype

SUBMITTER: Cacheux M 

PROVIDER: S-EPMC6952166 | biostudies-literature | 2020 Jan

REPOSITORIES: biostudies-literature