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- W4313385689 abstract "Abstract Mutations in the ADAR gene that encodes the ADAR1 RNA editing enzyme cause Aicardi-Goutières Syndrome (AGS), a severe autoimmune disease associated with an aberrant type I interferon response. How ADAR1 prevents autoimmunity remains incompletely defined. Here, we demonstrate that ADAR1 is a specific and essential negative regulator of the MDA5-MAVS RNA sensing pathway. Moreover, we uncovered a MDA5-MAVS-independent function for ADAR1 in the development of multiple organs. We showed that the p150 isoform of ADAR1 uniquely regulated the MDA5 pathway, whereas both the p150 and p110 isoforms contributed to development. Abrupt deletion of ADAR1 in adult mice revealed that both of these functions were required throughout life. Our findings delineate genetically distinct roles for both ADAR1 isoforms in vivo, with implications for the human diseases caused by ADAR mutations." @default.
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- W4313385689 date "2016-05-01" @default.
- W4313385689 modified "2023-10-18" @default.
- W4313385689 title "Isoforms of ADAR1 independently control MDA5-driven autoimmunity and multi-organ development" @default.
- W4313385689 doi "https://doi.org/10.4049/jimmunol.196.supp.203.6" @default.
- W4313385689 hasPublicationYear "2016" @default.
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