Matches in SemOpenAlex for { <https://semopenalex.org/work/W2794395939> ?p ?o ?g. }
- W2794395939 abstract "SUMMARY Innate immune signaling activation and DNA damage are pathological hallmarks of aging that may herald multiple adult-onset neurodegenerative diseases. Here, we report that both cell autonomous and non-autonomous neuronal death are triggered by the production of cytoplasmic double-stranded RNA (cdsRNA) from a regulated, disarticulated transgene in the setting of type I interferon (IFN-I) signaling. CdsRNA is a pathogen associated molecular pattern that induces IFN-I in many cell types. Transfection of a dsRNA mimetic into cultured human neurons also induces IFN-I signaling and cell death in a dose-dependent manner. Direct relevance to human disease is found in neurons of ALS-FTD patients carrying C9ORF72 intronic hexanucleotide expansions; cdsRNA isolated from these tissues is comprised of repeat sequences. Together, these findings implicate cdsRNA generated from genomic sequences in neurons as a trigger for sterile, viral-mimetic IFN-I induction and propagated neuronal death within in a neural circuit in the aging nervous system." @default.
- W2794395939 created "2018-03-29" @default.
- W2794395939 creator A5002949304 @default.
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- W2794395939 creator A5084252241 @default.
- W2794395939 date "2018-01-19" @default.
- W2794395939 modified "2023-10-06" @default.
- W2794395939 title "Genome-Encoded Cytoplasmic Double-Stranded RNAs, Found in C9ORF72 ALS-FTD Brain, Provoke Propagated Neuronal Death" @default.
- W2794395939 cites W1610732998 @default.
- W2794395939 cites W1797572428 @default.
- W2794395939 cites W1837455343 @default.
- W2794395939 cites W1971254602 @default.
- W2794395939 cites W1973435863 @default.
- W2794395939 cites W1974297564 @default.
- W2794395939 cites W1974961961 @default.
- W2794395939 cites W1979369962 @default.
- W2794395939 cites W1980894900 @default.
- W2794395939 cites W1984538960 @default.
- W2794395939 cites W1986730863 @default.
- W2794395939 cites W1987183038 @default.
- W2794395939 cites W1989032379 @default.
- W2794395939 cites W1994150511 @default.
- W2794395939 cites W1995222212 @default.
- W2794395939 cites W2006600110 @default.
- W2794395939 cites W2006764478 @default.
- W2794395939 cites W2007743488 @default.
- W2794395939 cites W2008334829 @default.
- W2794395939 cites W2013488820 @default.
- W2794395939 cites W2016609825 @default.
- W2794395939 cites W2018775541 @default.
- W2794395939 cites W2023453519 @default.
- W2794395939 cites W2027177575 @default.
- W2794395939 cites W2030641075 @default.
- W2794395939 cites W2042433863 @default.
- W2794395939 cites W2053476949 @default.
- W2794395939 cites W2054068057 @default.
- W2794395939 cites W2054542675 @default.
- W2794395939 cites W2056079743 @default.
- W2794395939 cites W2060122782 @default.
- W2794395939 cites W2061769617 @default.
- W2794395939 cites W2063703350 @default.
- W2794395939 cites W2065219073 @default.
- W2794395939 cites W2069171272 @default.
- W2794395939 cites W2070050178 @default.
- W2794395939 cites W2073518622 @default.
- W2794395939 cites W2073606662 @default.
- W2794395939 cites W2075383643 @default.
- W2794395939 cites W2076163730 @default.
- W2794395939 cites W2076305899 @default.
- W2794395939 cites W2078819049 @default.
- W2794395939 cites W2080053685 @default.
- W2794395939 cites W2085454814 @default.
- W2794395939 cites W2090092360 @default.
- W2794395939 cites W2092145129 @default.
- W2794395939 cites W2092288031 @default.
- W2794395939 cites W2093896551 @default.
- W2794395939 cites W2095331989 @default.
- W2794395939 cites W2095798723 @default.
- W2794395939 cites W2099003198 @default.
- W2794395939 cites W2101042581 @default.
- W2794395939 cites W2102053742 @default.
- W2794395939 cites W2102717949 @default.
- W2794395939 cites W2103279555 @default.
- W2794395939 cites W2103350275 @default.
- W2794395939 cites W2104770286 @default.
- W2794395939 cites W2107458751 @default.
- W2794395939 cites W2110308292 @default.
- W2794395939 cites W2116710635 @default.
- W2794395939 cites W2119708131 @default.
- W2794395939 cites W2121484182 @default.
- W2794395939 cites W2122550126 @default.
- W2794395939 cites W2126231259 @default.
- W2794395939 cites W2126525177 @default.
- W2794395939 cites W2128739333 @default.
- W2794395939 cites W2136482257 @default.
- W2794395939 cites W2141760092 @default.
- W2794395939 cites W2145045929 @default.
- W2794395939 cites W2146608733 @default.
- W2794395939 cites W2149561999 @default.
- W2794395939 cites W2153816436 @default.
- W2794395939 cites W2153825225 @default.
- W2794395939 cites W2154520196 @default.
- W2794395939 cites W2164104132 @default.
- W2794395939 cites W2168804671 @default.
- W2794395939 cites W2170327627 @default.
- W2794395939 cites W2260519762 @default.