Arx expansion mutation perturbs cortical development by augmenting apoptosis without activating innate immunity in a mouse model of X-linked infantile spasms syndrome

X-linked infantile spasms syndrome (ISSX) is a clinically devastating developmental epileptic encephalopathy with life-long impact. Arx((GCG)10+7), a mouse model of the most common triplet-repeat expansion mutation of ARX, exhibits neonatal spasms, electrographic phenotypes and abnormal migration of...

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Detalles Bibliográficos
Autores principales: Siehr, Meagan S., Massey, Cory A., Noebels, Jeffrey L.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: The Company of Biologists Ltd 2020
Materias:
Research Article
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7132796/
https://www.ncbi.nlm.nih.gov/pubmed/32033960
http://dx.doi.org/10.1242/dmm.042515

Internet

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7132796/
https://www.ncbi.nlm.nih.gov/pubmed/32033960
http://dx.doi.org/10.1242/dmm.042515

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