Article
Pathogenesis of lethal cardiac arrhythmias in Mecp2 mutant mice: implication for therapy in Rett syndrome.
Science translational medicine - 14 Dec 2011
McCauley Mark D, Wang Tiannan, Mike Elise, Herrera Jose, Beavers David L, Huang Teng-Wei, Ward Christopher S, Skinner Steven, Percy Alan K, Glaze Daniel G, Wehrens Xander H T, Neul Jeffrey L
Abstract excerpt
Rett syndrome is a neurodevelopmental disorder typically caused by mutations in methyl-CpG-binding protein 2 (MECP2) in which 26% of deaths are sudden and of unknown cause. To explore the hypothesis that these deaths may be due to cardiac dysfunction, we characterized the electrocardiograms in 379 people with Rett syndrome and found that 18.5% show prolongation of the corrected QT interval (QTc), an indication of...
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