Article
Abnormal network activity in a targeted genetic model of human double cortex.
The Journal of neuroscience : the official journal of the Society for Neuroscience - 14 Jan 2009
Ackman James B, Aniksztejn Laurent, Crépel Valérie, Becq Hélène, Pellegrino Christophe, Cardoso Carlos, Ben-Ari Yehezkel, Represa Alfonso
Abstract excerpt
In human patients, cortical dysplasia produced by Doublecortin (DCX) mutations lead to mental retardation and intractable infantile epilepsies, but the underlying mechanisms are not known. DCX(-/-) mice have been generated to investigate this issue. However, they display no neocortical abnormality, lessening their impact on the field. In contrast, in utero knockdown of DCX RNA produces a morphologically relevant...
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