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- W2017001665 abstract "In Huntington's disease and other polyglutamine (polyQ) disorders, mutant proteins containing a long polyQ stretch are well documented as the trigger of numerous aberrant cellular processes that primarily lead to degeneration and, ultimately, the death of neuronal cells. However, mutant transcripts containing expanded CAG repeats may also be toxic and contribute to cellular dysfunction. The exact nature and importance of RNA toxicity in polyQ diseases are only beginning to be recognized, and the first insights have mainly resulted from studies using simple model systems. In this review, we briefly present the basic mechanisms of protein toxicity in polyQ disorders and RNA toxicity in myotonic dystrophy type 1 and discuss recent results suggesting that the pathogenesis of polyQ diseases may also be mediated by mutant transcripts. This review is focused on the experimental systems used thus far to demonstrate RNA toxicity in polyQ disorders and the design of new systems that will be more relevant to the human disease situation and capable of separating RNA toxicity from protein toxicity." @default.
- W2017001665 created "2016-06-24" @default.
- W2017001665 creator A5039950251 @default.
- W2017001665 creator A5075484720 @default.
- W2017001665 date "2013-03-20" @default.
- W2017001665 modified "2023-09-30" @default.
- W2017001665 title "RNA toxicity in polyglutamine disorders: concepts, models, and progress of research" @default.
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- W2017001665 cites W1497938915 @default.
- W2017001665 cites W1555409852 @default.
- W2017001665 cites W1557634022 @default.
- W2017001665 cites W1963989418 @default.
- W2017001665 cites W1964365272 @default.
- W2017001665 cites W1965341424 @default.
- W2017001665 cites W1965568102 @default.
- W2017001665 cites W1969813445 @default.
- W2017001665 cites W1972792035 @default.
- W2017001665 cites W1976828379 @default.
- W2017001665 cites W1980624807 @default.
- W2017001665 cites W1982138681 @default.
- W2017001665 cites W1985474993 @default.
- W2017001665 cites W1989619236 @default.
- W2017001665 cites W1995306841 @default.
- W2017001665 cites W1997555550 @default.
- W2017001665 cites W1999617545 @default.
- W2017001665 cites W2003576322 @default.
- W2017001665 cites W2008526580 @default.
- W2017001665 cites W2009372610 @default.
- W2017001665 cites W2012024892 @default.
- W2017001665 cites W2012145036 @default.
- W2017001665 cites W2012768611 @default.
- W2017001665 cites W2013444252 @default.
- W2017001665 cites W2013820019 @default.
- W2017001665 cites W2014643086 @default.
- W2017001665 cites W2016281944 @default.
- W2017001665 cites W2020334159 @default.
- W2017001665 cites W2029122003 @default.
- W2017001665 cites W2029440153 @default.
- W2017001665 cites W2034644012 @default.
- W2017001665 cites W2042427251 @default.
- W2017001665 cites W2055197343 @default.
- W2017001665 cites W2061184966 @default.
- W2017001665 cites W2062181873 @default.
- W2017001665 cites W2062682951 @default.
- W2017001665 cites W2067551263 @default.
- W2017001665 cites W2068477500 @default.
- W2017001665 cites W2070224195 @default.
- W2017001665 cites W2073012734 @default.
- W2017001665 cites W2074172901 @default.
- W2017001665 cites W2076791245 @default.
- W2017001665 cites W2078217107 @default.
- W2017001665 cites W2081280332 @default.
- W2017001665 cites W2081635886 @default.
- W2017001665 cites W2082759452 @default.
- W2017001665 cites W2083076780 @default.
- W2017001665 cites W2083824522 @default.
- W2017001665 cites W2099643329 @default.
- W2017001665 cites W2099963610 @default.
- W2017001665 cites W2101747555 @default.
- W2017001665 cites W2102145465 @default.
- W2017001665 cites W2103116226 @default.
- W2017001665 cites W2104895670 @default.
- W2017001665 cites W2105291133 @default.
- W2017001665 cites W2108092527 @default.
- W2017001665 cites W2108570063 @default.
- W2017001665 cites W2110640321 @default.
- W2017001665 cites W2118546680 @default.
- W2017001665 cites W2121642311 @default.
- W2017001665 cites W2123193865 @default.
- W2017001665 cites W2126877059 @default.
- W2017001665 cites W2127313039 @default.
- W2017001665 cites W2131828888 @default.
- W2017001665 cites W2134802476 @default.
- W2017001665 cites W2144717644 @default.
- W2017001665 cites W2144927513 @default.
- W2017001665 cites W2146308704 @default.
- W2017001665 cites W2153992384 @default.
- W2017001665 cites W2158110928 @default.
- W2017001665 cites W2159854077 @default.
- W2017001665 cites W2162747288 @default.
- W2017001665 cites W2166145393 @default.
- W2017001665 cites W2167616277 @default.
- W2017001665 cites W2171165237 @default.
- W2017001665 cites W2171439103 @default.
- W2017001665 cites W2255469197 @default.
- W2017001665 cites W4236100085 @default.
- W2017001665 doi "https://doi.org/10.1007/s00109-013-1016-2" @default.
- W2017001665 hasPubMedCentralId "https://www.ncbi.nlm.nih.gov/pmc/articles/3659269" @default.
- W2017001665 hasPubMedId "https://pubmed.ncbi.nlm.nih.gov/23512265" @default.
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