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

Gaia Colasante, Alessandro Sessa, Stefania Crispi, Raffaele Calogero, Ahmed Mansouri, Patrick Collombat, Vania Broccoli

Research output: Contribution to journalArticle

Abstract

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.

Original languageEnglish
Pages (from-to)59-71
Number of pages13
JournalDevelopmental Biology
Volume334
Issue number1
DOIs
Publication statusPublished - Oct 1 2009

Keywords

  • Arx
  • Basal ganglia
  • Cell migration
  • Expression profile
  • Forebrain
  • GABA
  • Gene expression
  • Lissencephaly
  • Neurogenesis
  • Telencephalon
  • Transcription factor
  • West syndrome

ASJC Scopus subject areas

  • Cell Biology
  • Developmental Biology
  • Molecular Biology

Fingerprint Dive into the research topics of 'Arx acts as a regional key selector gene in the ventral telencephalon mainly through its transcriptional repression activity'. Together they form a unique fingerprint.

  • Cite this