Innate immune activation without immune cell infiltration in brains of murine models of Aicardi-Goutieres Syndrome.

ABSTRACT: Chronic inflammation is frequently invoked as a mechanism of neurodegeneration and yet inflammatory cell infiltrates are seldom seen in brains of these disorders. Different disciplines utilize different technologies and methodologies to describe what is immunologically defined as the innate immune response (IIR). We examined murine models of the human neurodegenerative disease Aicardi-Goutières Syndrome, where an IIR is initiated by aberrant RNA metabolism secondary to a mutation in adenosine deaminase acting on RNA gene (ADAR1). We previously showed that these mice demonstrated a deficit in RNA editing that lead to MDA-5 mediated RNA sensing pathway activation of the IIR with massive interferon stimulated gene transcription and translation. As early as 2 weeks of age, in situ hybridizatio

SUBMITTER: Wiley CA 

PROVIDER: S-EPMC10154360 | biostudies-literature | 2023 May

REPOSITORIES: biostudies-literature