Matches in SemOpenAlex for { <https://semopenalex.org/work/W2960641374> ?p ?o ?g. }
- W2960641374 endingPage "1105" @default.
- W2960641374 startingPage "1097" @default.
- W2960641374 abstract "Spinocerebellar ataxia type 17 (SCA17) is caused by polyglutamine (polyQ) expansion in the TATA box-binding protein (TBP), which functions as a general transcription factor. Like other polyQ expansion-mediated diseases, SCA17 is characterized by late-onset and selective neurodegeneration, despite the disease protein being ubiquitously expressed in the body. To date, the pathogenesis of polyQ diseases is not fully understood, and there are no effective treatments for these devastating disorders. The well-characterized function of TBP and typical neurodegeneration in SCA17 give us opportunities to understand how polyQ expansion causes selective neurodegeneration and to develop effective therapeutics. In this review, we discuss the molecular mechanisms behind SCA17, focusing on transcriptional dysregulation as its major cause. Mounting evidence suggests that reversing transcriptional alterations induced by mutant TBP and reducing the expression of mutant TBP are promising strategies to treat SCA17." @default.
- W2960641374 created "2019-07-23" @default.
- W2960641374 creator A5012301761 @default.
- W2960641374 creator A5058026632 @default.
- W2960641374 creator A5072263969 @default.
- W2960641374 date "2019-07-17" @default.
- W2960641374 modified "2023-09-30" @default.
- W2960641374 title "Molecular Mechanisms and Therapeutics for SCA17" @default.
- W2960641374 cites W1484316196 @default.
- W2960641374 cites W1910082823 @default.
- W2960641374 cites W1968190810 @default.
- W2960641374 cites W1982826808 @default.
- W2960641374 cites W1986022352 @default.
- W2960641374 cites W1988723452 @default.
- W2960641374 cites W1988762046 @default.
- W2960641374 cites W1989669244 @default.
- W2960641374 cites W1991848900 @default.
- W2960641374 cites W1996346895 @default.
- W2960641374 cites W2004659216 @default.
- W2960641374 cites W2017309058 @default.
- W2960641374 cites W2017518842 @default.
- W2960641374 cites W2021549542 @default.
- W2960641374 cites W2022440848 @default.
- W2960641374 cites W2023496461 @default.
- W2960641374 cites W2027659013 @default.
- W2960641374 cites W2028033234 @default.
- W2960641374 cites W2038895605 @default.
- W2960641374 cites W2042166639 @default.
- W2960641374 cites W2042425655 @default.
- W2960641374 cites W2050679356 @default.
- W2960641374 cites W2051130119 @default.
- W2960641374 cites W2052836084 @default.
- W2960641374 cites W2055111891 @default.
- W2960641374 cites W2058349538 @default.
- W2960641374 cites W2063482551 @default.
- W2960641374 cites W2068677696 @default.
- W2960641374 cites W2069339262 @default.
- W2960641374 cites W2080828566 @default.
- W2960641374 cites W2086315977 @default.
- W2960641374 cites W2090210400 @default.
- W2960641374 cites W2098919421 @default.
- W2960641374 cites W2106200048 @default.
- W2960641374 cites W2107639627 @default.
- W2960641374 cites W2108570063 @default.
- W2960641374 cites W2114364233 @default.
- W2960641374 cites W2118264257 @default.
- W2960641374 cites W2124141726 @default.
- W2960641374 cites W2128989600 @default.
- W2960641374 cites W2129194953 @default.
- W2960641374 cites W2136584707 @default.
- W2960641374 cites W2148014901 @default.
- W2960641374 cites W2157513658 @default.
- W2960641374 cites W2162695817 @default.
- W2960641374 cites W2163331386 @default.
- W2960641374 cites W2167400349 @default.
- W2960641374 cites W2169482727 @default.
- W2960641374 cites W2178300138 @default.
- W2960641374 cites W2221506660 @default.
- W2960641374 cites W2315039893 @default.
- W2960641374 cites W2464754433 @default.
- W2960641374 cites W2626238587 @default.
- W2960641374 cites W2749196262 @default.
- W2960641374 cites W2760870410 @default.
- W2960641374 cites W2792048849 @default.
- W2960641374 cites W2794874880 @default.
- W2960641374 cites W2799425982 @default.
- W2960641374 cites W2811286526 @default.
- W2960641374 cites W2909139375 @default.
- W2960641374 cites W2912977831 @default.
- W2960641374 cites W4237255420 @default.
- W2960641374 doi "https://doi.org/10.1007/s13311-019-00762-z" @default.
- W2960641374 hasPubMedCentralId "https://www.ncbi.nlm.nih.gov/pmc/articles/6985407" @default.
- W2960641374 hasPubMedId "https://pubmed.ncbi.nlm.nih.gov/31317427" @default.
- W2960641374 hasPublicationYear "2019" @default.
- W2960641374 type Work @default.
- W2960641374 sameAs 2960641374 @default.
- W2960641374 citedByCount "14" @default.
- W2960641374 countsByYear W29606413742019 @default.
- W2960641374 countsByYear W29606413742020 @default.
- W2960641374 countsByYear W29606413742021 @default.
- W2960641374 countsByYear W29606413742022 @default.
- W2960641374 countsByYear W29606413742023 @default.
- W2960641374 crossrefType "journal-article" @default.
- W2960641374 hasAuthorship W2960641374A5012301761 @default.
- W2960641374 hasAuthorship W2960641374A5058026632 @default.
- W2960641374 hasAuthorship W2960641374A5072263969 @default.
- W2960641374 hasBestOaLocation W29606413741 @default.
- W2960641374 hasConcept C104317684 @default.
- W2960641374 hasConcept C142724271 @default.
- W2960641374 hasConcept C166252455 @default.
- W2960641374 hasConcept C169760540 @default.
- W2960641374 hasConcept C180754005 @default.
- W2960641374 hasConcept C2776925932 @default.
- W2960641374 hasConcept C2779134260 @default.
- W2960641374 hasConcept C2779500118 @default.
- W2960641374 hasConcept C2780055153 @default.
- W2960641374 hasConcept C2780647506 @default.
- W2960641374 hasConcept C2780906641 @default.