Conditional Loss of Arx From the Developing Dorsal Telencephalon Results in Behavioral Phenotypes Resembling Mild Human ARX Mutations.

@article{Simonet2015ConditionalLO,
  title={Conditional Loss of Arx From the Developing Dorsal Telencephalon Results in Behavioral Phenotypes Resembling Mild Human ARX Mutations.},
  author={Jacqueline C. Simonet and C Nicole Sunnen and Jue Wu and Jeffrey A Golden and Eric D. Marsh},
  journal={Cerebral cortex},
  year={2015},
  volume={25 9},
  pages={
          2939-50
        }
}
Mutations in the Aristaless-Related Homeobox (ARX) gene cause structural anomalies of the brain, epilepsy, and neurocognitive deficits in children. During forebrain development, Arx is expressed in both pallial and subpallial progenitor cells. We previously demonstrated that elimination of Arx from subpallial-derived cortical interneurons generates an epilepsy phenotype with features overlapping those seen in patients with ARX mutations. In this report, we have selectively removed Arx from… CONTINUE READING

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