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- W2898173676 endingPage "495" @default.
- W2898173676 startingPage "469" @default.
- W2898173676 abstract "Frontotemporal dementia is a group of early onset dementia syndromes linked to underlying frontotemporal lobar degeneration (FTLD) pathology that can be classified based on the formation of abnormal protein aggregates involving tau and two RNA binding proteins, TDP-43 and FUS. Although elucidation of the mechanisms leading to FTLD pathology is in progress, recent advances in genetics and neuropathology indicate that a majority of FTLD cases with proteinopathy involving RNA binding proteins show highly congruent genotype–phenotype correlations. Specifically, recent studies have uncovered the unique properties of the low-complexity domains in RNA binding proteins that can facilitate liquid–liquid phase separation in the formation of membraneless organelles. Furthermore, there is compelling evidence that mutations in FTLD genes lead to dysfunction in diverse cellular pathways that converge on the endolysosomal pathway, autophagy, and neuroinflammation. Together, these results provide key mechanistic insights into the pathogenesis and potential therapeutic targets of FTLD." @default.
- W2898173676 created "2018-11-02" @default.
- W2898173676 creator A5036894949 @default.
- W2898173676 creator A5074694043 @default.
- W2898173676 creator A5080428514 @default.
- W2898173676 date "2019-01-24" @default.
- W2898173676 modified "2023-10-16" @default.
- W2898173676 title "RNA Binding Proteins and the Pathogenesis of Frontotemporal Lobar Degeneration" @default.
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