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Title: L1 knockout mice show dilated ventricles, vermis hypoplasia and impaired exploration patterns
Authors: Fransen, E. ×
D'Hooge, Rudi
Van Camp, G.
Verhoye, M.
Sijbers, J.
Reniers, E.
Soriano, P.
Kamiguchi, H.
Willemsen, R.
Koekkoek, S.K.E.
De Zeeuw, C.I.
Lemmon, V.
De Deyn, P.P.
Van der Linden, A.M.
Kooy, R.F.
Willems, P.J. #
Issue Date: 1998
Publisher: IRL Press
Series Title: Human Molecular Genetics vol:7 issue:6 pages:999-1009
Abstract: L1 is a neural cell adhesion molecule mainly involved in axon guidance and neuronal migration during brain development. Mutations in the human L1 gene give rise to a complex clinical picture, with mental retardation, neurologic abnormalities and a variable degree of hydrocephalus. Recently, a transgenic mouse model with a targeted null mutation in the L1 gene was generated. These knockout (KO) mice show hypoplasia of the corticospinal tract. Here we have performed further studies of these KO mice including magnetic resonance imaging of the brain, neuropathological analysis and behavioral testing. The ventricular system was shown to be abnormal with dilatation of the lateral ventricles and the 4th ventricle, and an altered shape of the Sylvius aqueduct. Additionally, the cerebellar vermis of the KO mice is hypoplastic. Their exploratory behavior is characterized by stereotype peripheral circling reminiscent of that of rodents with induced cerebellar lesions
ISSN: 0964-6906
Publication status: published
KU Leuven publication type: IT
Appears in Collections:Biological Psychology
× corresponding author
# (joint) last author

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