Research ArticleCardiology

Identification of a New Modulator of the Intercalated Disc in a Zebrafish Model of Arrhythmogenic Cardiomyopathy

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Science Translational Medicine  11 Jun 2014:
Vol. 6, Issue 240, pp. 240ra74
DOI: 10.1126/scitranslmed.3008008

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Fishing for Heart Healing

When Judy Garland sings “Thump, thump, thump went my heart strings,” she’s celebrating a new love connection. But it remains unclear why patients with arrhythmogenic cardiomyopathy (ACM) suffer frequent heart arrhythmias. Asimaki et al. used a zebrafish model of ACM to illuminate disease mechanisms and discovered a potential drug, SB216763, that suppressed the disease phenotype in the fish model. The authors also observed SB216763-reversible, abnormal cardiac remodeling in cardiac myocytes derived from rats that carried an ACM-related mutation and from induced pluripotent stem cells from two ACM probands. These observations pinpoint aberrant trafficking of cardiac proteins as a central mechanism underlying ACM cardiomyocyte injury and electrical abnormalities.