Article
ADAR1 Zα domain P195A mutation activates the MDA5-dependent RNA-sensing signaling pathway in brain without decreasing overall RNA editing.
Cell reports - 25 Jul 2023
Guo Xinfeng, Liu Silvia, Sheng Yi, Zenati Mazen, Billiar Timothy, Herbert Alan, Wang Qingde
Abstract excerpt
Variants of the RNA-editing enzyme ADAR1 cause Aicardi-Goutières syndrome (AGS), in which severe inflammation occurs in the brain due to innate immune activation. Here, we analyze the RNA-editing status and innate immune activation in an AGS mouse model that carries the Adar P195A mutation in the N terminus of the ADAR1 p150 isoform, the equivalent of the P193A human Zα variant causal for disease. This mutation...
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