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- W2922136998 abstract "Abstract Mutations of Fused in sarcoma (FUS), a ribonucleoprotein involved in RNA metabolism, have been found associated with both familial and sporadic cases of amyotrophic lateral sclerosis (ALS). Notably, besides mutations in the coding sequence, also mutations into the 3′ untranslated region, leading to increased levels of the wild-type protein, have been associated with neuronal death and ALS pathology, in ALS models and patients. The mechanistic link between altered FUS levels and ALS-related neurodegeneration is far to be elucidated, as well as the consequences of elevated FUS levels in the modulation of the inflammatory response sustained by glial cells, a well-recognized player in ALS progression. Here, we studied the effect of wild-type FUS overexpression on the responsiveness of mouse and human neural progenitor-derived astrocytes to a pro-inflammatory stimulus (IL1β) used to mimic an inflammatory environment. We found that astrocytes with increased FUS levels were more sensitive to IL1β, as shown by their enhanced expression of inflammatory genes, compared with control astrocytes. Moreover, astrocytes overexpressing FUS promoted neuronal cell death and pro-inflammatory microglia activation. We conclude that overexpression of wild-type FUS intrinsically affects astrocyte reactivity and drives their properties toward pro-inflammatory and neurotoxic functions, suggesting that a non-cell autonomous mechanism can support neurodegeneration in FUS-mutated animals and patients." @default.
- W2922136998 created "2019-03-22" @default.
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- W2922136998 date "2019-03-14" @default.
- W2922136998 modified "2023-09-30" @default.
- W2922136998 title "Increased FUS levels in astrocytes leads to astrocyte and microglia activation and neuronal death" @default.
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- W2922136998 doi "https://doi.org/10.1038/s41598-019-41040-4" @default.
- W2922136998 hasPubMedCentralId "https://www.ncbi.nlm.nih.gov/pmc/articles/6418113" @default.
- W2922136998 hasPubMedId "https://pubmed.ncbi.nlm.nih.gov/30872738" @default.
- W2922136998 hasPublicationYear "2019" @default.
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