Article
Neonatal estradiol stimulation prevents epilepsy in Arx model of X-linked infantile spasms syndrome.
Science translational medicine - 22 Jan 2014
Olivetti Pedro R, Maheshwari Atul, Noebels Jeffrey L
Abstract excerpt
Infantile spasms are a catastrophic form of pediatric epilepsy with inadequate treatment. In patients, mutation of ARX, a transcription factor selectively expressed in neuronal precursors and adult inhibitory interneurons, impairs cell migration and causes a major inherited subtype of the disease X-linked infantile spasms syndrome. Using an animal model, the Arx((GCG)10+7) mouse, we determined that brief...
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