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- W2049342904 endingPage "348" @default.
- W2049342904 startingPage "339" @default.
- W2049342904 abstract "Misfolded TAR DNA binding protein 43 (TDP-43) and Fused-In-Sarcoma (FUS) protein have recently been identified as pathological hallmarks of the neurodegenerative disorders amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration (FTLD) characterized by the presence of ubiquitin-positive inclusions (FTLD-U). Although TDP-43 and FUS are normally located predominantly in the nucleus, pathological TDP-43 and FUS inclusions are mostly found in the cytosol. Cytosolic deposition is paralleled by a striking nuclear depletion of either protein. Based on a number of recent findings, we postulate that defects in nuclear import are an important step towards TDP-43 and FUS dysfunction. Failure of nuclear transport can arise from mutations within a nuclear localization signal or from age-related decline of nuclear import mechanisms. We propose that nuclear import defects in combination with additional hits, for example cellular stress and genetic risk factors, may be a central underlying cause of ALS and FTLD-U pathology." @default.
- W2049342904 created "2016-06-24" @default.
- W2049342904 creator A5065084452 @default.
- W2049342904 creator A5068527702 @default.
- W2049342904 date "2011-07-01" @default.
- W2049342904 modified "2023-09-25" @default.
- W2049342904 title "TDP-43 and FUS: a nuclear affair" @default.
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