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

Fanny Sandrine Martineau 1 Lauriane Fournier Emmanuelle Buhler 2 Françoise Watrin 3 Francesca Sargolini 4 Jean-Bernard Manent 5 Bruno Poucet 4 Alfonso Represa 2
1 AMU - Aix Marseille Université
2 INMED - Institut de neurobiologie de la Méditerranée
3 IBDM - Institut de Biologie du Développement de Marseille
4 LNC - Laboratoire de Neurosciences Cognitives [Marseille]
5 Epilepsie et ischémie cérébrale
Abstract : 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
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Fanny Sandrine Martineau, Lauriane Fournier, Emmanuelle Buhler, Françoise Watrin, Francesca Sargolini, et al.. Spared cognitive and behavioral functions prior to epilepsy onset in a rat model of 2 subcortical band heteropia. Brain Research, Elsevier, 2019, 1711, pp.146-155. ⟨10.1016/j.brainres.2019.01.030⟩. ⟨inserm-02001716⟩

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