Lovastatin suppresses hyperexcitability and seizure in Angelman syndrome model.


Journal Article

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.

Full Text

Cited Authors

  • Chung, L; Bey, AL; Towers, AJ; Cao, X; Kim, IH; Jiang, Y-H

Published Date

  • February 2018

Published In

Volume / Issue

  • 110 /

Start / End Page

  • 12 - 19

PubMed ID

  • 29097328

Pubmed Central ID

  • 29097328

Electronic International Standard Serial Number (EISSN)

  • 1095-953X

International Standard Serial Number (ISSN)

  • 0969-9961

Digital Object Identifier (DOI)

  • 10.1016/j.nbd.2017.10.016


  • eng