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Rodriguez, Steven; Sahin, Asli; Schrank, Benjamin R; Al-Lawati, Hawra; Costantino, Isabel; Benz, Eric; Fard, Darian; Albers, Alefiya D; Cao, Luxiang; Gomez, Alexis C; Evans, Kyle; Ratti, Elena; Cudkowicz, Merit; Frosch, Matthew P; Talkowski, Michael; Sorger, Peter K; Hyman, Bradley T; Albers, Mark W
Science translational medicine, 07/2021, Letnik: 13, Številka: 601Journal Article
Triggers of innate immune signaling in the CNS of patients with amyotrophic lateral sclerosis and frontotemporal degeneration (ALS/FTD) remain elusive. We report the presence of cytoplasmic double-stranded RNA (cdsRNA), an established trigger of innate immunity, in ALS-FTD brains carrying intronic hexanucleotide expansions that included genomically encoded expansions of the G C repeat sequences. The presence of cdsRNA in human brains was coincident with cytoplasmic TAR DNA binding protein 43 (TDP-43) inclusions, a pathologic hallmark of ALS/FTD. Introducing cdsRNA into cultured human neural cells induced type I interferon (IFN-I) signaling and death that was rescued by FDA-approved JAK inhibitors. In mice, genomically encoded dsRNAs expressed exclusively in a neuronal class induced IFN-I and death in connected neurons non-cell-autonomously. Our findings establish that genomically encoded cdsRNAs trigger sterile, viral-mimetic IFN-I induction and propagated death within neural circuits and may drive neuroinflammation and neurodegeneration in patients with ALS/FTD.
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in: SICRIS
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