Matches in SemOpenAlex for { <https://semopenalex.org/work/W2051130119> ?p ?o ?g. }
- W2051130119 endingPage "e1003781" @default.
- W2051130119 startingPage "e1003781" @default.
- W2051130119 abstract "There are no effective therapeutics that antagonize or reverse the protein-misfolding events underpinning polyglutamine (PolyQ) disorders, including Spinocerebellar Ataxia Type-3 (SCA3). Here, we augment the proteostasis network of Drosophila SCA3 models with Hsp104, a powerful protein disaggregase from yeast, which is bafflingly absent from metazoa. Hsp104 suppressed eye degeneration caused by a C-terminal ataxin-3 (MJD) fragment containing the pathogenic expanded PolyQ tract, but unexpectedly enhanced aggregation and toxicity of full-length pathogenic MJD. Hsp104 suppressed toxicity of MJD variants lacking a portion of the N-terminal deubiquitylase domain and full-length MJD variants unable to engage polyubiquitin, indicating that MJD-ubiquitin interactions hinder protective Hsp104 modalities. Importantly, in staging experiments, Hsp104 suppressed toxicity of a C-terminal MJD fragment when expressed after the onset of PolyQ-induced degeneration, whereas Hsp70 was ineffective. Thus, we establish the first disaggregase or chaperone treatment administered after the onset of pathogenic protein-induced degeneration that mitigates disease progression." @default.
- W2051130119 created "2016-06-24" @default.
- W2051130119 creator A5038969227 @default.
- W2051130119 creator A5047629225 @default.
- W2051130119 creator A5084836766 @default.
- W2051130119 date "2013-09-05" @default.
- W2051130119 modified "2023-10-11" @default.
- W2051130119 title "Hsp104 Suppresses Polyglutamine-Induced Degeneration Post Onset in a Drosophila MJD/SCA3 Model" @default.
- W2051130119 cites W1519439387 @default.
- W2051130119 cites W1541950149 @default.
- W2051130119 cites W1963866642 @default.
- W2051130119 cites W1965584354 @default.
- W2051130119 cites W1966923623 @default.
- W2051130119 cites W1969055742 @default.
- W2051130119 cites W1969725515 @default.
- W2051130119 cites W1973897761 @default.
- W2051130119 cites W1977241652 @default.
- W2051130119 cites W1979964642 @default.
- W2051130119 cites W1980431043 @default.
- W2051130119 cites W1984777146 @default.
- W2051130119 cites W1985113471 @default.
- W2051130119 cites W1985755878 @default.
- W2051130119 cites W1985772487 @default.
- W2051130119 cites W1988110501 @default.
- W2051130119 cites W1992341804 @default.
- W2051130119 cites W1998934132 @default.
- W2051130119 cites W2000706612 @default.
- W2051130119 cites W2001624926 @default.
- W2051130119 cites W2004879226 @default.
- W2051130119 cites W2005508231 @default.
- W2051130119 cites W2013029120 @default.
- W2051130119 cites W2013112316 @default.
- W2051130119 cites W2019129236 @default.
- W2051130119 cites W2024695270 @default.
- W2051130119 cites W2025269346 @default.
- W2051130119 cites W2029471292 @default.
- W2051130119 cites W2030985020 @default.
- W2051130119 cites W2031155677 @default.
- W2051130119 cites W2032459218 @default.
- W2051130119 cites W2032625890 @default.
- W2051130119 cites W2033500343 @default.
- W2051130119 cites W2035989121 @default.
- W2051130119 cites W2036563840 @default.
- W2051130119 cites W2037089507 @default.
- W2051130119 cites W2037262355 @default.
- W2051130119 cites W2038299405 @default.
- W2051130119 cites W2042716629 @default.
- W2051130119 cites W2042731166 @default.
- W2051130119 cites W2042811939 @default.
- W2051130119 cites W2047000182 @default.
- W2051130119 cites W2047304481 @default.
- W2051130119 cites W2051939475 @default.
- W2051130119 cites W2052098570 @default.
- W2051130119 cites W2056558785 @default.
- W2051130119 cites W2056911532 @default.
- W2051130119 cites W2061874150 @default.
- W2051130119 cites W2062203120 @default.
- W2051130119 cites W2064700750 @default.
- W2051130119 cites W2071012611 @default.
- W2051130119 cites W2072545392 @default.
- W2051130119 cites W2073449985 @default.
- W2051130119 cites W2073687064 @default.
- W2051130119 cites W2079470515 @default.
- W2051130119 cites W2082762584 @default.
- W2051130119 cites W2089629318 @default.
- W2051130119 cites W2110643873 @default.
- W2051130119 cites W2111700402 @default.
- W2051130119 cites W2112335210 @default.
- W2051130119 cites W2119651404 @default.
- W2051130119 cites W2125465720 @default.
- W2051130119 cites W2128033750 @default.
- W2051130119 cites W2131926693 @default.
- W2051130119 cites W2134124061 @default.
- W2051130119 cites W2134802476 @default.
- W2051130119 cites W2135124405 @default.
- W2051130119 cites W2135718381 @default.
- W2051130119 cites W2135732941 @default.
- W2051130119 cites W2136560136 @default.
- W2051130119 cites W2137222303 @default.
- W2051130119 cites W2137546687 @default.
- W2051130119 cites W2139587743 @default.
- W2051130119 cites W2141566674 @default.
- W2051130119 cites W2143014850 @default.
- W2051130119 cites W2143306707 @default.
- W2051130119 cites W2146671222 @default.
- W2051130119 cites W2151809142 @default.
- W2051130119 cites W2153123993 @default.
- W2051130119 cites W2153490747 @default.
- W2051130119 cites W2155976721 @default.
- W2051130119 cites W2157355956 @default.
- W2051130119 cites W2158073956 @default.
- W2051130119 cites W2158936307 @default.
- W2051130119 cites W2162930176 @default.
- W2051130119 cites W2163930643 @default.
- W2051130119 cites W2165186407 @default.
- W2051130119 cites W2169579807 @default.
- W2051130119 cites W2170594193 @default.
- W2051130119 cites W2171049750 @default.