PMID- 30689978
OWN - NLM
STAT- MEDLINE
DCOM- 20200709
LR  - 20211204
IS  - 1872-6240 (Electronic)
IS  - 0006-8993 (Linking)
VI  - 1711
DP  - 2019 May 15
TI  - Spared cognitive and behavioral functions prior to epilepsy onset in a rat model 
      of subcortical band heterotopia.
PG  - 146-155
LID - S0006-8993(19)30052-6 [pii]
LID - 10.1016/j.brainres.2019.01.030 [doi]
AB  - 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.
CI  - Copyright (c) 2019 Elsevier B.V. All rights reserved.
FAU - Martineau, Fanny Sandrine
AU  - Martineau FS
AD  - INMED, Aix-Marseille University, INSERM U1249, Marseille 13273 CEDEX 09, France.
FAU - Fournier, Lauriane
AU  - Fournier L
AD  - INMED, Aix-Marseille University, INSERM U1249, Marseille 13273 CEDEX 09, France.
FAU - Buhler, Emmanuelle
AU  - Buhler E
AD  - INMED, Aix-Marseille University, INSERM U1249, Marseille 13273 CEDEX 09, France.
FAU - Watrin, Francoise
AU  - Watrin F
AD  - INMED, Aix-Marseille University, INSERM U1249, Marseille 13273 CEDEX 09, France.
FAU - Sargolini, Francesca
AU  - Sargolini F
AD  - LNC - Federation de recherche 3C, Aix-Marseille University, CNRS UMR7291, 
      Marseille 13331 CEDEX 03, France.
FAU - Manent, Jean-Bernard
AU  - Manent JB
AD  - INMED, Aix-Marseille University, INSERM U1249, Marseille 13273 CEDEX 09, France.
FAU - Poucet, Bruno
AU  - Poucet B
AD  - LNC - Federation de recherche 3C, Aix-Marseille University, CNRS UMR7291, 
      Marseille 13331 CEDEX 03, France.
FAU - Represa, Alfonso
AU  - Represa A
AD  - INMED, Aix-Marseille University, INSERM U1249, Marseille 13273 CEDEX 09, France. 
      Electronic address: alfonso.represa@inserm.fr.
LA  - eng
PT  - Journal Article
PT  - Research Support, Non-U.S. Gov't
DEP - 20190125
PL  - Netherlands
TA  - Brain Res
JT  - Brain research
JID - 0045503
RN  - 0 (DCX protein, human)
RN  - 0 (Dcx protein, rat)
RN  - 0 (Doublecortin Domain Proteins)
RN  - 0 (Doublecortin Protein)
RN  - 0 (Microtubule-Associated Proteins)
RN  - 0 (Neuropeptides)
RN  - 0 (RNA, Small Interfering)
SB  - IM
MH  - Animals
MH  - Anxiety/genetics
MH  - Asymptomatic Diseases
MH  - *Behavior, Animal
MH  - Cell Movement
MH  - Classical Lissencephalies and Subcortical Band 
      Heterotopias/complications/embryology/genetics/*psychology
MH  - *Cognition
MH  - *Disease Models, Animal
MH  - Doublecortin Domain Proteins
MH  - Doublecortin Protein
MH  - Electroporation
MH  - Epilepsy/*genetics
MH  - Exploratory Behavior
MH  - Gray Matter/abnormalities/embryology
MH  - Intellectual Disability/*genetics
MH  - Learning
MH  - Maze Learning
MH  - Memory
MH  - Microtubule-Associated Proteins/deficiency/genetics
MH  - Mosaicism
MH  - Neuropeptides/deficiency/genetics
MH  - RNA, Small Interfering/administration & dosage/toxicity
MH  - Rats
MH  - Rotarod Performance Test
MH  - Sensation
MH  - White Matter/abnormalities/embryology
OTO - NOTNLM
OT  - Cognition
OT  - Cortex development
OT  - Cortical malformation
OT  - Doublecortin
OT  - Memory
OT  - Somatosensory processing
EDAT- 2019/01/29 06:00
MHDA- 2020/07/10 06:00
CRDT- 2019/01/29 06:00
PHST- 2018/10/25 00:00 [received]
PHST- 2019/01/23 00:00 [revised]
PHST- 2019/01/24 00:00 [accepted]
PHST- 2019/01/29 06:00 [pubmed]
PHST- 2020/07/10 06:00 [medline]
PHST- 2019/01/29 06:00 [entrez]
AID - S0006-8993(19)30052-6 [pii]
AID - 10.1016/j.brainres.2019.01.030 [doi]
PST - ppublish
SO  - Brain Res. 2019 May 15;1711:146-155. doi: 10.1016/j.brainres.2019.01.030. Epub 
      2019 Jan 25.