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

Marine Cacheux; Jérémy Fauconnier; Jérôme Thireau; Alexis Osseni; Jacques Brocard; Nathalie Roux-Buisson; Julie Brocard; Julien Fauré; Alain Lacampagne; Isabelle Marty

doi:10.1016/j.ymthe.2019.09.012

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

Marine Cacheux, Jérémy Fauconnier, Jérôme Thireau, Alexis Osseni, Jacques Brocard, Nathalie Roux-Buisson, Julie Brocard, Julien Fauré, Alain Lacampagne, Isabelle Marty

Research output: Contribution to journal › Article › peer-review

22 Scopus citations

Abstract

Catecholaminergic polymorphic ventricular tachycardia (CPVT) is induced by mutations in triadin or calsequestrin. Deletion/mutation of triadin is associated with reduction in calsequestrin and leads to CPVT. Cacheux and colleagues show that partial re-expression of triadin and calsequestrin through gene or pharmacological therapy reverses this cardiac arrhythmia.

Original language	English
Pages (from-to)	171-179
Number of pages	9
Journal	Molecular Therapy
Volume	28
Issue number	1
DOIs	https://doi.org/10.1016/j.ymthe.2019.09.012
State	Published - 8 Jan 2020
Externally published	Yes

Keywords

Ca release
cardiac arrhythmia
gene therapy
genetic disease
sarcoplasmic reticulum

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10.1016/j.ymthe.2019.09.012

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title = "Interplay between Triadin and Calsequestrin in the Pathogenesis of CPVT in the Mouse",

abstract = "Catecholaminergic polymorphic ventricular tachycardia (CPVT) is induced by mutations in triadin or calsequestrin. Deletion/mutation of triadin is associated with reduction in calsequestrin and leads to CPVT. Cacheux and colleagues show that partial re-expression of triadin and calsequestrin through gene or pharmacological therapy reverses this cardiac arrhythmia.",

keywords = "Ca release, cardiac arrhythmia, gene therapy, genetic disease, sarcoplasmic reticulum",

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AU - Cacheux, Marine

AU - Fauconnier, Jérémy

AU - Thireau, Jérôme

AU - Osseni, Alexis

AU - Brocard, Jacques

AU - Roux-Buisson, Nathalie

AU - Brocard, Julie

AU - Fauré, Julien

AU - Lacampagne, Alain

AU - Marty, Isabelle

PY - 2020/1/8

Y1 - 2020/1/8

N2 - Catecholaminergic polymorphic ventricular tachycardia (CPVT) is induced by mutations in triadin or calsequestrin. Deletion/mutation of triadin is associated with reduction in calsequestrin and leads to CPVT. Cacheux and colleagues show that partial re-expression of triadin and calsequestrin through gene or pharmacological therapy reverses this cardiac arrhythmia.

AB - Catecholaminergic polymorphic ventricular tachycardia (CPVT) is induced by mutations in triadin or calsequestrin. Deletion/mutation of triadin is associated with reduction in calsequestrin and leads to CPVT. Cacheux and colleagues show that partial re-expression of triadin and calsequestrin through gene or pharmacological therapy reverses this cardiac arrhythmia.

KW - Ca release

KW - cardiac arrhythmia

KW - gene therapy

KW - genetic disease

KW - sarcoplasmic reticulum

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JO - Molecular Therapy

JF - Molecular Therapy

IS - 1

ER -

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

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Keywords

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