Article
Arrhythmia Mechanism and Dynamics in a Humanized Mouse Model of Inherited Cardiomyopathy Caused by Phospholamban R14del Mutation.
Circulation - 10 Aug 2021
Raad Nour, Bittihn Philip, Cacheux Marine, Jeong Dongtak, Ilkan Zeki, Ceholski Delaine, Kohlbrenner Erik, Zhang Lu, Cai Chen-Leng, Kranias Evangelia G, Hajjar Roger J, Stillitano Francesca, Akar Fadi G
Abstract excerpt
BACKGROUND: Arginine (Arg) 14 deletion (R14del) in the calcium regulatory protein phospholamban (hPLNR14del) has been identified as a disease-causing mutation in patients with an inherited cardiomyopathy. Mechanisms underlying the early arrhythmogenic phenotype that predisposes carriers of this mutation to sudden death with no apparent structural remodeling remain unclear. METHODS: To address this, we performed...
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