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Cerebral Cortex Advance Access published online on June 18, 2008
Cerebral Cortex, doi:10.1093/cercor/bhn106
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© 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


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