Article
Loss of Nuclear Envelope Integrity and Increased Oxidant Production Cause DNA Damage in Adult Hearts Deficient in PKP2: A Molecular Substrate of ARVC.
Circulation - 13 Sept 2022
Pérez-Hernández Marta, van Opbergen Chantal J M, Bagwan Navratan, Vissing Christoffer Rasmus, Marrón-Liñares Grecia M, Zhang Mingliang, Torres Vega Estefania, Sorrentino Andrea, Drici Lylia, Sulek Karolina, Zhai Ruxu, Hansen Finn B, Christensen Alex H, Boesgaard Søren, Gustafsson Finn, Rossing Kasper, Small Eric M, Davies Michael J, Rothenberg Eli, Sato Priscila Y, Cerrone Marina, Jensen Thomas Hartvig Lindkær, Qvortrup Klaus, Bundgaard Henning, Delmar Mario, Lundby Alicia
Abstract excerpt
BACKGROUND: Arrhythmogenic right ventricular cardiomyopathy (ARVC) is characterized by high propensity to life-threatening arrhythmias and progressive loss of heart muscle. More than 40% of reported genetic variants linked to ARVC reside in the PKP2 gene, which encodes the PKP2 protein (plakophilin-2). METHODS: We describe a comprehensive characterization of the ARVC molecular landscape as determined by...
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