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Disrupted migration of GABAergic interneurons lead to the epileptogenesis: Interneuron pathology associated with Arx mutation

      Aim: X-linked lissencephaly with abnormal genitalia (XLAG), showing severe neonatal seizure and developmental delay, is a rare disorder caused by mutations in the aristaless-related homeobox (ARX) gene, located in Xp22.13. Arx-null mice for human XLAG model showed loss of tangential migration of GABAergic interneurons. However, GABAergic interneuron distribution of XLAG brain has never been reported. In the present study, we investigated subpopulation of GABAergic interneurons in the brain of an infant with XLAG, who had a nonsense mutation of the ARX gene, compared with those of age-matched normal control and Miller–Dieker syndrome.
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