title: A fishing trip to cure arrhythmogenic cardiomyopathy?
abstract: The paper entitled "Identification of a New Modulator of the Intercalated Disc in a Zebrafish Model of Arrhythmogenic Cardiomyopathy", as published in 2014 in Science Translational Medicine, examined the effects of the newly discovered drug SB216763 (SB21) on arrhythmogenic cardiomyopathy (ACM). In this paper, the authors focused on mechanisms underlying ACM and the accompanying molecular and cellular alterations. Most importantly they showed that SB21 was able to rescue and partly reverse the ACM phenotype in three different experimental models: (I) a zebrafish model of Naxos disease induced by the overexpression of the 2057del2 mutation in plakoglobin (PKG); (II) neonatal rat cardiomyocytes overexpressing the same mutation in PKG; (III) cardiomyocytes derived from induced pluripotent stem cells expressing two different forms of mutations in plakophilin-2. This editorial will focus on the potency and possible restrictions concerning SB21 treatment as a potential intervention for ACM and the usefulness of the applied zebrafish models in general.
authors.affiliation: Department of Medical Physiology, Division of Heart & Lungs, University Medical Center Utrecht Utrecht, The Netherlands.
authors.forename: Elise L
authors.initials: EL
authors.lastname: Kessler
authors.affiliation: Department of Medical Physiology, Division of Heart & Lungs, University Medical Center Utrecht Utrecht, The Netherlands.
authors.forename: Toon A B
authors.initials: TA
authors.lastname: van Veen
keywords: Arrhythmogenic cardiomyopathy (ACM); GSK-3β; SB216763 (SB21); therapy; zebrafish