Journal
DEVELOPMENTAL BIOLOGY
Volume 334, Issue 1, Pages 59-71Publisher
ACADEMIC PRESS INC ELSEVIER SCIENCE
DOI: 10.1016/j.ydbio.2009.07.014
Keywords
Forebrain; Telencephalon; Basal ganglia; Neurogenesis; GABA; Cell migration; Arx; Lissencephaly; West syndrome; Gene expression; Expression profile; Transcription factor
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Funding
- Italian Telethon Foundation [GGP07181]
- Max-Planck Society
- Alte Leipziger foundation
- Inserm-Avenir Program
- Juvenile Diabetes Research foundation [26-2008-639]
- NIH Beta Cell Biology Consortium [U19 DK 072495-01]
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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. (C) 2009 Elsevier Inc. All rights reserved.
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