Article
Protein kinase R and the integrated stress response drive immunopathology caused by mutations in the RNA deaminase ADAR1.
Immunity - 14 Sept 2021
Maurano Megan, Snyder Jessica M, Connelly Caitlin, Henao-Mejia Jorge, Sidrauski Carmela, Stetson Daniel B
Abstract excerpt
The RNA deaminase ADAR1 is an essential negative regulator of the RNA sensor MDA5, and loss of ADAR1 function triggers inappropriate activation of MDA5 by self-RNAs. Mutations in ADAR, the gene that encodes ADAR1, cause human immune diseases, including Aicardi-Goutières syndrome (AGS). However, the mechanisms of MDA5-dependent disease pathogenesis in vivo remain unknown. Here we generated mice with a single amino...
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