ZBP1 induces immunopathology caused by loss of ADAR1-mediated RNA editing

Mutations of ADAR, which encodes the RNA-editing enzyme deaminase acting on RNA 1 (ADAR1), result in autoinflammatory diseases such as Aicardi-Goutières syndrome in humans and mice. Although ADAR1-mediated adenosine to inosine (A-to-I) editing is known to limit the accumulation of immunostimulatory endogenous double-stranded RNA (dsRNA) that signals through MDA5–MAVS, the downstream pathways responsible for autoinflammation in the context of ADAR mutation have not been fully resolved. Three studies in Nature identify ZBP1 as a key effector of both cell death and inflammatory transcription downstream of ADAR mutation, through the recognition of dsRNA from endogenous Alu elements.

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