Article
Treatment of cardiac arrhythmias in a mouse model of Rett syndrome with Na+-channel-blocking antiepileptic drugs.
Disease models & mechanisms - 1 Apr 2015
Herrera José A, Ward Christopher S, Pitcher Meagan R, Percy Alan K, Skinner Steven, Kaufmann Walter E, Glaze Daniel G, Wehrens Xander H T, Neul Jeffrey L
Abstract excerpt
One quarter of deaths associated with Rett syndrome (RTT), an X-linked neurodevelopmental disorder, are sudden and unexpected. RTT is associated with prolonged QTc interval (LQT), and LQT-associated cardiac arrhythmias are a potential cause of unexpected death. The standard of care for LQT in RTT is treatment with β-adrenergic antagonists; however, recent work indicates that acute treatment of mice with RTT with...
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