Article
Molecular disturbance underlies to arrhythmogenic cardiomyopathy induced by transgene content, age and exercise in a truncated PKP2 mouse model.
Human molecular genetics - 1 Sept 2016
Moncayo-Arlandi Javier, Guasch Eduard, Sanz-de la Garza Maria, Casado Marta, Garcia Nahuel Aquiles, Mont Lluis, Sitges Marta, Knöll Ralph, Buyandelger Byambajav, Campuzano Oscar, Diez-Juan Antonio, Brugada Ramon
Abstract excerpt
Arrhythmogenic cardiomyopathy (ACM) is a disorder characterized by a progressive ventricular myocardial replacement by fat and fibrosis, which lead to ventricular arrhythmias and sudden cardiac death. Mutations in the desmosomal gene Plakophilin-2 (PKP2) accounts for >40% of all known mutations, generally causing a truncated protein. In a PKP2-truncated mouse model, we hypothesize that content of transgene,...
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