The RNA-Editing Enzyme ADAR1 Controls Innate Immune Responses to RNA
Institute of Genetics and Cancer · University of Edinburgh · +7 more institutions
Abstract
The ADAR RNA-editing enzymes deaminate adenosine bases to inosines in cellular RNAs. Aberrant interferon expression occurs in patients in whom ADAR1 mutations cause Aicardi-Goutières syndrome (AGS) or dystonia arising from striatal neurodegeneration. Adar1 mutant mouse embryos show aberrant interferon induction and die by embryonic day E12.5. We demonstrate that Adar1 embryonic lethality is rescued to live birth in Adar1; Mavs double mutants in which the antiviral interferon induction response to cytoplasmic double-stranded RNA (dsRNA) is prevented. Aberrant immune responses in Adar1 mutant mouse embryo fibroblasts are dramatically reduced by restoring the expression of editing-active cytoplasmic ADARs. We…
Citation impact
- FWCI
- 14.80
- Percentile
- 100%
- References
- 40
Authors
17- NMNiamh MannionCorresponding
Institute of Genetics and Cancer, University of Edinburgh
- SMSam M. Greenwood
University of Edinburgh, Institute of Genetics and Cancer
- RSRobert S. Young
Institute of Genetics and Cancer, University of Edinburgh
- SLSarah L. Cox
University of Edinburgh, Institute of Genetics and Cancer
- JBJames Brindle
Institute of Genetics and Cancer, University of Edinburgh
Topics & keywords
- Innate immune system
- RNA
- RNA editing
- Inosine
- Biology
- RIG-I
- Interferon
- Enzyme
- Good health and well-being