Rudy Celeghin, Giovanni Risato, Giorgia Beffagna, Marco Cason, Maria Bueno Marinas, Mila Della Barbera, Nicola Facchinello, Alice Giuliodori, Raquel Brañas Casas, Micol Caichiolo, Andrea Vettori, Enrico Grisan, Stefania Rizzo, Luisa Dalla Valle, Francesco Argenton, Gaetano Thiene, Natascia Tiso, Kalliopi Pilichou, Cristina Basso
Arrhythmogenic cardiomyopathy (AC) is an inherited disorder characterized by progressive loss of the ventricular myocardium causing life-threatening ventricular arrhythmias, syncope and sudden cardiac death in young and athletes. About 40% of AC cases carry one or more mutations in genes encoding for desmosomal proteins, including Desmoplakin (Dsp). We present here the first stable Dsp knock-out (KO) zebrafish line able to model cardiac alterations and cell signalling dysregulation, characteristic of the AC disease, on which environmental factors and candidate drugs can be tested...
December 6, 2023: Cell Death Discovery