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Mutations in the adenosine deaminase ADAR1 that prevent endogenous Z-RNA binding induce Aicardi-Goutières-syndrome-like encephalopathy

Immunity. 2021-09; 
Taisuke Nakahama, Yuki Kato, Toshiharu Shibuya, Maal Inoue, Jung In Kim, Tuangtong Vongpipatana, Hiroyuki Todo, Yanfang Xing, Yukio Kawahara
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Stable Cell Lines … both Adar1 and Adar2 genes by genome editing using a CRISPR-Cas9 system to exclude … effect of Zα domain mutation on editing activity using this cell line without the secondary effect … ) were obtained from GenScript. These plasmids were then transfected into Raw 264.7 cells … Get A Quote

摘要

Mutations in the adenosine-to-inosine RNA-editing enzyme ADAR1 p150, including point mutations in the Z-RNA recognition domain Zα, are associated with Aicardi-Goutières syndrome (AGS). Here, we examined the in vivo relevance of ADAR1 binding of Z-RNA. Mutation of W197 in Zα, which abolished Z-RNA binding, reduced RNA editing. Adar1 mice displayed severe growth retardation after birth, broad expression of interferon-stimulated genes (ISGs), and abnormal development of multiple organs. Notably, malformation of the brain was accompanied by white matter vacuolation and gliosis, reminiscent of AGS-associated encephalopathy. Concurrent deletion of the double-stranded RNA sensor MDA5 ameliorated these abnormalitie... More

关键词

ADAR1, AGS, ISG, MDA5, RNA editing, Z-RNA, encephalopathy, hematopoiesis, inosine