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Publication : Arx acts as a regional key selector gene in the ventral telencephalon mainly through its transcriptional repression activity.

First Author  Colasante G Year  2009
Journal  Dev Biol Volume  334
Issue  1 Pages  59-71
PubMed ID  19627984 Mgi Jnum  J:153556
Mgi Id  MGI:4365703 Doi  10.1016/j.ydbio.2009.07.014
Citation  Colasante G, et al. (2009) Arx acts as a regional key selector gene in the ventral telencephalon mainly through its transcriptional repression activity. Dev Biol 334(1):59-71
abstractText  The homeobox-containing gene Arx is expressed during ventral telencephalon development and required for correct GABAergic interneuron tangential migration from the ganglionic eminences to the olfactory bulbs, cerebral cortex and striatum. Its human ortholog is associated with a variety of neurological clinical manifestations whose symptoms are compatible with the loss of cortical interneurons and altered basal ganglia-related activities. Herein, we report the identification of a number of genes whose expression is consistently altered in Arx mutant ganglionic eminences. Our analyses revealed a striking ectopic expression in the ganglionic eminences of several of these genes normally at most marginally expressed in the ventral telencephalon. Among them, Ebf3 was functionally analyzed. Thus, its ectopic expression in ventral telencephalon was found to prevent neuronal tangential migration. Further, we showed that Arx is sufficient to repress Ebf3 endogenous expression and that its silencing in Arx mutant tissues partially rescues tangential cell movement. Together, these data provide new insights into the molecular pathways regulated by Arx during telencephalon development.
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