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

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 G...

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Bibliographic Details
Main Authors: Meagan S. Siehr, Cory A. Massey, Jeffrey L. Noebels
Format: Article
Language:English
Published: The Company of Biologists 2020-03-01
Series:Disease Models & Mechanisms
Subjects:
estradiol
acth
interneuronopathy
inflammation
epileptic encephalopathy
Online Access:http://dmm.biologists.org/content/13/3/dmm042515

Internet

http://dmm.biologists.org/content/13/3/dmm042515

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