Characterization of the HeCo mutant mouse: a new model of subcortical band heterotopia associated with seizures and behavioral deficits.

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Serval ID
serval:BIB_A4416F0B5FFE
Type
Article: article from journal or magazin.
Collection
Publications
Institution
Title
Characterization of the HeCo mutant mouse: a new model of subcortical band heterotopia associated with seizures and behavioral deficits.
Journal
Cerebral Cortex
Author(s)
Croquelois A., Giuliani F., Savary C., Kielar M., Amiot C., Schenk F., Welker E.
ISSN
1460-2199
Publication state
Published
Issued date
2009
Peer-reviewed
Oui
Volume
19
Number
3
Pages
563-575
Language
english
Notes
Publication types: Journal Article ; Research Support, Non-U.S. Gov't - Publication Status: ppublish
Abstract
In human, neuronal migration disorders are commonly associated with developmental delay, mental retardation, and epilepsy. We describe here a new mouse mutant that develops a heterotopic cortex (HeCo) lying in the dorsolateral hemispheric region, between the homotopic cortex (HoCo) and subcortical white matter. Cross-breeding demonstrated an autosomal recessive transmission. Birthdating studies and immunochemistry for layer-specific markers revealed that HeCo formation was due to a transit problem in the intermediate zone affecting both radially and tangentially migrating neurons. The scaffold of radial glial fibers, as well as the expression of doublecortin is not altered in the mutant. Neurons within the HeCo are generated at a late embryonic age (E18) and the superficial layers of the HoCo have a correspondingly lower cell density and layer thickness. Parvalbumin immunohistochemistry showed the presence of gamma-aminobutyric acidergic cells in the HeCo and the mutant mice have a lowered threshold for the induction of epileptic seizures. The mutant showed a developmental delay but, in contrast, memory function was relatively spared. Therefore, this unique mouse model resembles subcortical band heterotopia observed in human. This model represents a new and rare tool to better understand cortical development and to investigate future therapeutic strategies for refractory epilepsy.
Keywords
cerebral cortex growth and development, migration disorder, models: animal, neuronal neurobehavioral manifestations
Pubmed
Web of science
Open Access
Yes
Create date
30/01/2009 10:13
Last modification date
25/09/2019 6:10
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