Basic mechanisms of MCD in animal models

Giorgio Battaglia, Albert J. Becker, Joseph LoTurco, Alfonso Represa, Scott C. Baraban, Steven N. Roper, Annamaria Vezzani

Research output: Contribution to journalArticlepeer-review

Abstract

Epilepsy-associated glioneuronal malformations (malformations of cortical development [MCD]) include focal cortical dysplasias (FCD) and highly differentiated glioneuronal tumors, most frequently gangliogliomas. The neuropathological findings are variable but suggest aberrant proliferation, migration, and differentiation of neural precursor cells as essential pathogenetic elements. Recent advances in animal models for MCDs allow new insights in the molecular pathogenesis of these epilepsy-associated lesions. Novel approaches, presented here, comprise RNA interference strategies to generate and study experimental models of subcortical band heterotopia and study functional aspects of aberrantly shaped and positioned neurons. Exciting analyses address impaired NMDA receptor expression in FCD animal models compared to human FCDs and excitatory imbalances in MCD animal models such as lissencephaly gene ablated mice as well as in utero irradiated rats. An improved understanding of relevant pathomechanisms will advance the development of targeted treatment strategies for epilepsy-associated malformations.

Original languageEnglish
Pages (from-to)206-214
Number of pages9
JournalEpileptic Disorders
Volume11
Issue number3
DOIs
Publication statusPublished - Sep 2009

Keywords

  • Animal model
  • Epileptogenesis
  • Glutamate receptors
  • In utero irradiation
  • MAEUK proteins
  • MCD
  • Methylazoxymethanol
  • Pilocarpine

ASJC Scopus subject areas

  • Clinical Neurology
  • Neurology

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