Inflammatory cytokine production in a mouse model of Aicardi-Goutieres syndrome and neuroinflammation

Most neurological diseases are associated with a tissue injury that is detected by the innate immune response(IIR),leading to an inflammatory component.The IIR is activated through conserved Pattern Recognition Receptors,including membrane bound Toll like receptors(TLRs),intracellular nucleotide-binding oligomerization domain like receptors and the receptors for advanced glycation end-products(Amor et al.,2010;Heppnerrt et al.,2015).These receptors detect highly conserved structural motifs of damaged or stressed tissues(danger-associated molecular patterns(DAMP)).Cytosolic receptors of nucleic acids,such as cyclic guanosine monophosphate-adenosine monophosphate synthase(cGAS)and melanoma differentiation associated gene 5(MDA-5)can also trigger IIR activation leading to interferon(IFN)and IFN stimulated gene(ISG)expression through DNA/RNA sensing signaling pathways(Yang and Li,2020).