Whereas, inter-observer agreement was 100.0 ± 0.0% for abnormal junctional plectin localization (diagnostic scores of 0–1) in myocardium for ARVC patients carrying an additional PLEC variant (Fig 1C, S4 Table), indicating that the observation of abnormal localization is not due to variation between the observers. Here, PLEC is linked to Arrhythmogenic right ventricular dysplasia.