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Updated: Jul 14, 2026

Modeling Myotonic Dystrophy 1 in C2C12 Myoblast Cells
Published on: July 29, 2016
Sudden cardiac death in myotonic dystrophy type 2
B G H Schoser1, K Ricker, C Schneider-Gold
1Friedrich Baur Institute, Department of Neurology, Ludwig Maximilians University Munich, Ziemssenstr. 1a, 80336 Munich, Germany. bschoser@med.uni-muenchen.de
Abstract:
Medical records and follow-up data were reviewed in 297 genetically proven myotonic dystrophy type 2 (DM2) patients. Patients were selected by the criteria of cardiac sudden death before age 45. Sudden death occurred in four patients, three of whom were cardiological asymptomatic, and one with a history of heart failure. Cardiac histopathology showed dilated cardiomyopathy in all, and conduction system fibrosis in two patients. Pathogenetic CCUG ribonuclear inclusions were demonstrable in cardiomyocytes.
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