Spared cognitive and behavioral functions prior to epilepsy onset in a rat model of 2 subcortical band heteropia

  • Martineau Fanny Sandrine
  • Fournier Lauriane
  • Buhler Emmanuelle
  • Watrin Françoise
  • Sargolini Francesca
  • Manent Jean-Bernard
  • Poucet Bruno
  • Represa Alfonso

ART

13 Subcortical band heterotopia (SBH), also known as doublecortex syndrome, is a 14 malformation of cortical development resulting from mutations in the doublecortin gene 15 (DCX). It is characterized by a lack of migration of cortical neurons that accumulate in the 16 white matter forming a heterotopic band. Patients with SBH may present mild to moderate 17 intellectual disability as well as epilepsy. The SBH condition can be modeled in rats by in 18 utero knockdown (KD) of Dcx. The affected cells form an SBH reminiscent of that observed in 19 human patients and the animals develop a chronic epileptic condition in adulthood. Here, 20 we investigated if the presence of an SBH is sufficient to induce cognitive impairment in 21