Skip Navigation


Cerebral Cortex Advance Access originally published online on June 18, 2008
Cerebral Cortex 2009 19(3):563-575; doi:10.1093/cercor/bhn106
This Article
Right arrow Full Text
Right arrow Full Text (PDF)
Right arrow Supplementary Data
Right arrow All Versions of this Article:
19/3/563    most recent
bhn106v1
Right arrow Alert me when this article is cited
Right arrow Alert me if a correction is posted
Services
Right arrow Email this article to a friend
Right arrow Similar articles in this journal
Right arrow Similar articles in PubMed
Right arrow Alert me to new issues of the journal
Right arrow Add to My Personal Archive
Right arrow Download to citation manager
Right arrowRequest Permissions
Google Scholar
Right arrow Articles by Croquelois, A.
Right arrow Articles by Welker, E.
Right arrow Search for Related Content
PubMed
Right arrow PubMed Citation
Right arrow Articles by Croquelois, A.
Right arrow Articles by Welker, E.
Social Bookmarking
Add to CiteULike   Add to Connotea   Add to Del.icio.us  
What's this?

© The Author 2008. Published by Oxford University Press. All rights reserved. For permissions, please e-mail: journals.permissions@oxfordjournals.org

Characterization of the HeCo Mutant Mouse: A New Model of Subcortical Band Heterotopia Associated with Seizures and Behavioral Deficits

Alexandre Croquelois1,2, Fabienne Giuliani3,4, Christine Savary2, Michel Kielar2, Clotilde Amiot5, Françoise Schenk3,4 and Egbert Welker2

1 Service de Neuropsychologie et de Neuroréhabilitation, Centre Hospitalier Universitaire Vaudois (CHUV), Avenue Pierre Decker 5, 1011 Lausanne, Switzerland, 2 Department de Biologie Cellulaire et de Morphologie, Université de Lausanne, Rue du Bugnon 9, CH-1005 Lausanne, Switzerland, 3 Institut de Physiologie, Centre de Neurosciences Psychiatriques, Site de Cery, CH-1008 Prilly, Switzerland, 4 Institut de Psychologie, Université de Lausanne, Quartier UNIL-Dorigny, Bâtiment Anthropole, 1015 Lausanne, Switzerland, 5 Service de Génétique, Histologie, Biologie du Développement et de la Reproduction (EA 3922), Hôpital Saint-Jacques, Place Saint-Jacques, 25030 Besancon cedex, France

Address correspondence to Alexandre Croquelois, MD, Service de Neuropsychologie et de Neuroréhabilitation, Unité Universitaire de Neuroréhabilitation, Centre Hospitalier Universitaire Vaudois (CHUV), Avenue Pierre Decker 5, CH-1011 Lausanne, Switzerland. Email: alexandre.croquelois{at}chuv.ch.

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.

Key Words: cerebral cortex growth and development • migration disorder • models: animal • neuronal neurobehavioral manifestations


Add to CiteULike CiteULike   Add to Connotea Connotea   Add to Del.icio.us Del.icio.us    What's this?




Disclaimer: Please note that abstracts for content published before 1996 were created through digital scanning and may therefore not exactly replicate the text of the original print issues. All efforts have been made to ensure accuracy, but the Publisher will not be held responsible for any remaining inaccuracies. If you require any further clarification, please contact our Customer Services Department.