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- W4311632380 abstract "Abstract Amyotrophic lateral sclerosis type 6 (ALS6) is a familial subtype of ALS linked to Fused in Sarcoma (FUS) gene mutation. FUS mutations lead to decreased global protein synthesis, but the mechanism that drives this has not been established. Here, we used ALS6 patient-derived induced pluripotent stem cells (hIPSCs) to study the effect of the ALS6 FUS R521H mutation on the translation machinery in motor neurons (MNs). We find, in agreement with findings of others, that protein synthesis is decreased in ALS6 MNs. Furthermore, ALS6 MNs are more sensitive to oxidative stress and display reduced expression of TGF-β and mTORC gene pathways when stressed. Finally, we show that IFN γ treatment reduces apoptosis of ALS6 MNs exposed to oxidative stress and partially restores the translation rates in ALS6 MNs. Overall, these findings suggest that a functional IFN γ response is important for FUS-mediated protein synthesis, possibly by FUS nuclear translocation in ALS6. Highlights and eTOC blurb ALS6 patient-derived motor neurons show decreased viability and reduced production of innate immune cytokines following oxidative stress FUS cytoplasmic localization coincides with decreased protein synthesis rates IFN γ treatment of ALS6 patient-derived motor neurons reduces apoptosis and ameliorates translation rates resulting from oxidative stress" @default.
- W4311632380 created "2022-12-27" @default.
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- W4311632380 date "2022-12-05" @default.
- W4311632380 modified "2023-09-27" @default.
- W4311632380 title "IFNγ protects motor neurons from oxidative stress via enhanced global protein synthesis in FUS-associated Amyotrophic Lateral Sclerosis" @default.
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- W4311632380 doi "https://doi.org/10.1101/2022.12.05.519150" @default.
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