ArticleDisease models & mechanisms2015
Treatment of cardiac arrhythmias in a mouse model of Rett syndrome with Na+-channel-blocking antiepileptic drugs.
Article in Disease models & mechanisms, 2015. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 15 papers.
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Who cites it
15 citing papers in PubMed, 18 citations in OpenAlex.
- Rett Syndrome: The Emerging Landscape of Treatment Strategies.CNS drugs · 2024Review
- Rett syndrome: The Natural History Study journey.Annals of the Child Neurology Society · 2024Review
- The Heart of Rett Syndrome: A Quantitative Analysis of Cardiac Repolarization.Cardiology research · 2023Article
- Variant-specific therapy for long QT syndrome type 3.Heart rhythm · 2023Article
- Rett syndrome with atrial tachycardia in a girl.Pediatric investigation · 2022Article
- QTPhysiological reports · 2022Article
- Delayed Ventricular Repolarization and Sodium Channel Current Modification in a Mouse Model of Rett Syndrome.International journal of molecular sciences · 2022Article
- Drug Studies on Rett Syndrome: From Bench to Bedside.Journal of autism and developmental disorders · 2020Review
- Antiproliferative and antimigratory effects of 3-(4-substituted benzyl)-5- isopropyl-5-phenylhydantoin derivatives in human breast cancer cells.Saudi pharmaceutical journal : SPJ : the official publication of the Saudi Pharmaceutical Society · 2020Article
- Potent hERG channel inhibition by sarizotan, an investigative treatment for Rett Syndrome.Journal of molecular and cellular cardiology · 2019Article
- Evaluation of QTc in Rett syndrome: Correlation with age, severity, and genotype.American journal of medical genetics. Part A · 2017Article
- Paradoxical physiological responses to propranolol in a Rett syndrome patient: a case report.BMC pediatrics · 2016Article
- Methyl-CpG binding-protein 2 function in cholinergic neurons mediates cardiac arrhythmogenesis.Human molecular genetics · 2016Article
- Progressive Changes in a Distributed Neural Circuit Underlie Breathing Abnormalities in Mice Lacking MeCP2.The Journal of neuroscience : the official journal of the Society for Neuroscience · 2016Article
- From bench to patient: model systems in drug discovery.Disease models & mechanisms · 2015Article
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Authors and funding
9 authors at 5 institutions in 1 country.
Funding
Abstract
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 a β-antagonist, propranolol, does not prevent lethal arrhythmias. In contrast, acute treatment with the Na(+) channel blocker phenytoin prevented arrhythmias. Chronic dosing of propranolol may be required for efficacy; therefore, we tested the efficacy of chronic treatment with either propranolol or phenytoin on RTT mice. Phenytoin completely abolished arrhythmias, whereas propranolol showed no benefit. Surprisingly, phenytoin also normalized weight and activity, but worsened breathing patterns. To explore the role of Na(+) channel blockers on QT in people with RTT, we performed a retrospective analysis of QT status before and after Na(+) channel blocker antiepileptic therapies. Individuals with RTT and LQT significantly improved their QT interval status after being started on Na(+) channel blocker antiepileptic therapies. Thus, Na(+) channel blockers should be considered for the clinical management of LQT in individuals with RTT.
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