PMID- 18562329
OWN - NLM
STAT- MEDLINE
DCOM- 20091217
LR  - 20090206
IS  - 1460-2199 (Electronic)
IS  - 1047-3211 (Linking)
VI  - 19
IP  - 3
DP  - 2009 Mar
TI  - Characterization of the HeCo mutant mouse: a new model of subcortical band 
      heterotopia associated with seizures and behavioral deficits.
PG  - 563-75
LID - 10.1093/cercor/bhn106 [doi]
AB  - 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.
FAU - Croquelois, Alexandre
AU  - Croquelois A
AD  - Service de Neuropsychologie et de Neurorehabilitation, Centre Hospitalier 
      Universitaire Vaudois (CHUV), Lausanne, Switzerland. alexandre.croquelois@chuv.ch
FAU - Giuliani, Fabienne
AU  - Giuliani F
FAU - Savary, Christine
AU  - Savary C
FAU - Kielar, Michel
AU  - Kielar M
FAU - Amiot, Clotilde
AU  - Amiot C
FAU - Schenk, Francoise
AU  - Schenk F
FAU - Welker, Egbert
AU  - Welker E
LA  - eng
PT  - Comparative Study
PT  - Journal Article
PT  - Research Support, Non-U.S. Gov't
DEP - 20080618
PL  - United States
TA  - Cereb Cortex
JT  - Cerebral cortex (New York, N.Y. : 1991)
JID - 9110718
SB  - IM
MH  - Animals
MH  - Animals, Newborn
MH  - *Cerebral Cortex
MH  - Choristoma/genetics/*pathology
MH  - Classical Lissencephalies and Subcortical Band Heterotopias/genetics/*pathology
MH  - Cognition Disorders/genetics/*pathology
MH  - Crosses, Genetic
MH  - *Disease Models, Animal
MH  - Female
MH  - Male
MH  - Maze Learning
MH  - Mice
MH  - Mice, Inbred C57BL
MH  - Mice, Mutant Strains
MH  - Pregnancy
MH  - Seizures/genetics/*pathology
EDAT- 2008/06/20 09:00
MHDA- 2009/12/18 06:00
CRDT- 2008/06/20 09:00
PHST- 2008/06/20 09:00 [pubmed]
PHST- 2009/12/18 06:00 [medline]
PHST- 2008/06/20 09:00 [entrez]
AID - bhn106 [pii]
AID - 10.1093/cercor/bhn106 [doi]
PST - ppublish
SO  - Cereb Cortex. 2009 Mar;19(3):563-75. doi: 10.1093/cercor/bhn106. Epub 2008 Jun 
      18.