Engineered Heart Tissues Expressing Mutant Desmoplakin Exhibit Altered Twitch Kinetics

Arrhythmogenic cardiomyopathy (AC) is a disease characterized by electrical abnormalities and fibro-fatty infiltration of the myocardium. It is often linked to mutations in desmosomal genes, but molecular mechanisms whereby desmosomal mutations give rise to end-stage AC remain to be fully established. In order to probe phenotypic consequences of an AC-linked desmosomal mutation, we identified a candidate family with a history of sudden cardiac...