First Author | Chung L | Year | 2018 |
Journal | Neurobiol Dis | Volume | 110 |
Pages | 12-19 | PubMed ID | 29097328 |
Mgi Jnum | J:268451 | Mgi Id | MGI:6267117 |
Doi | 10.1016/j.nbd.2017.10.016 | Citation | Chung L, et al. (2018) Lovastatin suppresses hyperexcitability and seizure in Angelman syndrome model. Neurobiol Dis 110:12-19 |
abstractText | Epilepsy is prevalent and often medically intractable in Angelman syndrome (AS). AS mouse model (Ube3a(m-/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 Ube3a(m-/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 Ube3a(m-/p+). The in vitro model of Ube3a(m-/p+) is valuable for dissection of neural mechanism and epilepsy drug screening in vivo. |