Aicardi-Goutières syndrome-associated mutation at ADAR1 gene locus activates innate immune response in mouse brain

Aicardi-Goutières syndrome-associated mutation at ADAR1 gene locus activates innate immune response in mouse brain

Abstract Background Aicardi-Goutières syndrome (AGS) is a severe infant or juvenile-onset autoimmune disease characterized by inflammatory encephalopathy with an elevated type 1 interferon-stimulated gene (ISG) expression signature in the brain. Mutations in seven different protein-coding genes, all...

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Bibliographic Details
Main Authors: Xinfeng Guo, Clayton A. Wiley, Richard A. Steinman, Yi Sheng, Beihong Ji, Junmei Wang, Liyong Zhang, Tony Wang, Mazen Zenatai, Timothy R. Billiar, Qingde Wang
Format: Article
Language:English
Published: BMC 2021-07-01
Series:Journal of Neuroinflammation
Subjects:
Aicardi-Goutières syndrome (AGS)
Animal model
RNA editing
Adenosine Deaminase Acting on RNA 1 (ADAR1)
Interferonopathy
In situ hybridization
Online Access:https://doi.org/10.1186/s12974-021-02217-9

Internet

https://doi.org/10.1186/s12974-021-02217-9

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