4.5 Article

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

期刊

BRAIN RESEARCH
卷 1711, 期 -, 页码 146-155

出版社

ELSEVIER SCIENCE BV
DOI: 10.1016/j.brainres.2019.01.030

关键词

Cortical malformation; Cortex development; Doublecortin; Cognition; Memory; Somatosensory processing

资金

  1. French National Agency for Research [19012012 SAMENTA]
  2. European Community 7th Framework programs [Health-F2-602531-2013]
  3. La Fondation pour la Recherche Medicale, France [FDT20160435216]

向作者/读者索取更多资源

Subcortical band heterotopia (SBH), also known as doublecortex syndrome, is a malformation of cortical development resulting from mutations in the doublecortin gene (DCX). It is characterized by a lack of migration of cortical neurons that accumulate in the white matter forming a heterotopic band. Patients with SBH may present mild to moderate intellectual disability as well as epilepsy. The SBH condition can be modeled in rats by in utero knockdown (KD) of Dcx. The affected cells form an SBH reminiscent of that observed in human patients and the animals develop a chronic epileptic condition in adulthood. Here, we investigated if the presence of a SBH is sufficient to induce cognitive impairment in juvenile Dcx-KD rats, before the onset of epilepsy. Using a wide range of behavioral tests, we found that the presence of SBH did not appear to affect motor control or somatosensory processing. In addition, cognitive abilities such as learning, short-term and long-term memory, were normal in pre-epileptic Dcx-KD rats. We suggest that the SBH presence is not sufficient to impair these behavioral functions.

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