Lovastatin suppresses hyperexcitability and seizure in Angelman syndrome model

Leeyup Chung, Alexandra L. Bey, Aaron J. Towers, Xinyu Cao, Il Hwan Kim, Yong hui Jiang

Research output: Contribution to journalArticle

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Abstract

Epilepsy is prevalent and often medically intractable in Angelman syndrome (AS). AS mouse model (Ube3am −/p +) shows reduced excitatory neurotransmission but lower seizure threshold. The neural mechanism linking the synaptic dysfunction to the seizure remains elusive. We show that the local circuits of Ube3am −/p + in vitro are hyperexcitable and display a unique epileptiform activity, a phenomenon that is reminiscent of the finding in fragile X syndrome (FXS) mouse model. Similar to the FXS model, lovastatin suppressed the epileptiform activity and audiogenic seizures in Ube3am −/p +. The in vitro model of Ube3am −/p + is valuable for dissection of neural mechanism and epilepsy drug screening in vivo.

Original languageEnglish (US)
Pages (from-to)12-19
Number of pages8
JournalNeurobiology of Disease
Volume110
DOIs
StatePublished - Feb 1 2018

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Angelman Syndrome
Lovastatin
Fragile X Syndrome
Seizures
Epilepsy
Preclinical Drug Evaluations
Synaptic Transmission
Dissection
In Vitro Techniques

All Science Journal Classification (ASJC) codes

  • Neurology

Cite this

Lovastatin suppresses hyperexcitability and seizure in Angelman syndrome model. / Chung, Leeyup; Bey, Alexandra L.; Towers, Aaron J.; Cao, Xinyu; Kim, Il Hwan; Jiang, Yong hui.

In: Neurobiology of Disease, Vol. 110, 01.02.2018, p. 12-19.

Research output: Contribution to journalArticle

Chung, Leeyup ; Bey, Alexandra L. ; Towers, Aaron J. ; Cao, Xinyu ; Kim, Il Hwan ; Jiang, Yong hui. / Lovastatin suppresses hyperexcitability and seizure in Angelman syndrome model. In: Neurobiology of Disease. 2018 ; Vol. 110. pp. 12-19.
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