학술논문
Spared cognitive and behavioral functions prior to epilepsy onset in a rat model of subcortical band heterotopia.
Document Type
Academic Journal
Author
Martineau FS; INMED, Aix-Marseille University, INSERM U1249, Marseille 13273 CEDEX 09, France.; Fournier L; INMED, Aix-Marseille University, INSERM U1249, Marseille 13273 CEDEX 09, France.; Buhler E; INMED, Aix-Marseille University, INSERM U1249, Marseille 13273 CEDEX 09, France.; Watrin F; INMED, Aix-Marseille University, INSERM U1249, Marseille 13273 CEDEX 09, France.; Sargolini F; LNC - Fédération de recherche 3C, Aix-Marseille University, CNRS UMR7291, Marseille 13331 CEDEX 03, France.; Manent JB; INMED, Aix-Marseille University, INSERM U1249, Marseille 13273 CEDEX 09, France.; Poucet B; LNC - Fédération de recherche 3C, Aix-Marseille University, CNRS UMR7291, Marseille 13331 CEDEX 03, France.; Represa A; INMED, Aix-Marseille University, INSERM U1249, Marseille 13273 CEDEX 09, France. Electronic address: alfonso.represa@inserm.fr.
Source
Publisher: Elsevier/North-Holland Biomedical Press Country of Publication: Netherlands NLM ID: 0045503 Publication Model: Print-Electronic Cited Medium: Internet ISSN: 1872-6240 (Electronic) Linking ISSN: 00068993 NLM ISO Abbreviation: Brain Res Subsets: MEDLINE
Subject
Language
English
Abstract
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.
(Copyright © 2019 Elsevier B.V. All rights reserved.)
(Copyright © 2019 Elsevier B.V. All rights reserved.)