Matches in SemOpenAlex for { <https://semopenalex.org/work/W2034963123> ?p ?o ?g. }
- W2034963123 endingPage "1197" @default.
- W2034963123 startingPage "1186" @default.
- W2034963123 abstract "Huntington's disease (HD) and spinocerebellar ataxias (SCAs) are autosomal-dominant neurodegenerative disorders. HD is caused by polyglutamine (polyQ) expansion in the amino-terminal region of a protein huntingtin (Htt) and primarily affects medium spiny striatal neurons (MSN). Many SCAs are caused by polyQ-expansion in ataxin proteins and primarily affect cerebellar Purkinje cells. The reasons for neuronal dysfunction and death in HD and SCAs remain poorly understood and no cure is available for the patients. Our laboratory discovered that mutant huntingtin, ataxin-2 and ataxin-3 proteins specifically bind to the carboxy-terminal region of the type 1 inositol 1,4,5-trisphosphate receptor (IP(3)R1), an intracellular Ca(2+) release channel. Moreover, we found that association of mutant huntingtin or ataxins with IP(3)R1 causes sensitization of IP(3)R1 to activation by IP(3) in planar lipid bilayers and in neuronal cells. These results suggested that deranged neuronal Ca(2+) signaling might play an important role in pathogenesis of HD, SCA2 and SCA3. In support of this idea, we demonstrated a connection between abnormal Ca(2+) signaling and neuronal cell death in experiments with HD, SCA2 and SCA3 transgenic mouse models. Additional data in the literature indicate that abnormal neuronal Ca(2+) signaling may also play an important role in pathogenesis of SCAl, SCA5, SCA6, SCA14 and SCA15/16. Based on these results I propose that IP(3)R and other Ca(2+) signaling proteins should be considered as potential therapeutic targets for treatment of HD and SCAs." @default.
- W2034963123 created "2016-06-24" @default.
- W2034963123 creator A5074668880 @default.
- W2034963123 date "2011-01-06" @default.
- W2034963123 modified "2023-10-14" @default.
- W2034963123 title "Role of Inositol 1,4,5-Trishosphate Receptors in Pathogenesis of Huntington’s Disease and Spinocerebellar Ataxias" @default.
- W2034963123 cites W1484597533 @default.
- W2034963123 cites W1496748157 @default.
- W2034963123 cites W1507443798 @default.
- W2034963123 cites W1510439895 @default.
- W2034963123 cites W1541950149 @default.
- W2034963123 cites W1569018103 @default.
- W2034963123 cites W1606950487 @default.
- W2034963123 cites W1895861480 @default.
- W2034963123 cites W1964776354 @default.
- W2034963123 cites W1965271417 @default.
- W2034963123 cites W1965797477 @default.
- W2034963123 cites W1968905480 @default.
- W2034963123 cites W1971047357 @default.
- W2034963123 cites W1974888639 @default.
- W2034963123 cites W1975238147 @default.
- W2034963123 cites W1975711970 @default.
- W2034963123 cites W1977733590 @default.
- W2034963123 cites W1978021058 @default.
- W2034963123 cites W1981645566 @default.
- W2034963123 cites W1982910974 @default.
- W2034963123 cites W1983435592 @default.
- W2034963123 cites W1984393517 @default.
- W2034963123 cites W1989507218 @default.
- W2034963123 cites W1989619236 @default.
- W2034963123 cites W1993470377 @default.
- W2034963123 cites W1995847434 @default.
- W2034963123 cites W1996592124 @default.
- W2034963123 cites W1998116358 @default.
- W2034963123 cites W2000370819 @default.
- W2034963123 cites W2001014014 @default.
- W2034963123 cites W2002644780 @default.
- W2034963123 cites W2002763309 @default.
- W2034963123 cites W2004483055 @default.
- W2034963123 cites W2005895876 @default.
- W2034963123 cites W2006155616 @default.
- W2034963123 cites W2006426969 @default.
- W2034963123 cites W2006569015 @default.
- W2034963123 cites W2007578481 @default.
- W2034963123 cites W2007719003 @default.
- W2034963123 cites W2012360559 @default.
- W2034963123 cites W2014486882 @default.
- W2034963123 cites W2016742032 @default.
- W2034963123 cites W2018185396 @default.
- W2034963123 cites W2021549542 @default.
- W2034963123 cites W2021729913 @default.
- W2034963123 cites W2022446197 @default.
- W2034963123 cites W2022486367 @default.
- W2034963123 cites W2024150064 @default.
- W2034963123 cites W2025578163 @default.
- W2034963123 cites W2025789853 @default.
- W2034963123 cites W2027275070 @default.
- W2034963123 cites W2028937313 @default.
- W2034963123 cites W2029956630 @default.
- W2034963123 cites W2029964084 @default.
- W2034963123 cites W2030410903 @default.
- W2034963123 cites W2031155677 @default.
- W2034963123 cites W2032459218 @default.
- W2034963123 cites W2032770343 @default.
- W2034963123 cites W2033080896 @default.
- W2034963123 cites W2034537895 @default.
- W2034963123 cites W2034823585 @default.
- W2034963123 cites W2040308828 @default.
- W2034963123 cites W2040683034 @default.
- W2034963123 cites W2041371057 @default.
- W2034963123 cites W2042739691 @default.
- W2034963123 cites W2042811939 @default.
- W2034963123 cites W2042861001 @default.
- W2034963123 cites W2043888873 @default.
- W2034963123 cites W2044356662 @default.
- W2034963123 cites W2046132401 @default.
- W2034963123 cites W2049611687 @default.
- W2034963123 cites W2054286526 @default.
- W2034963123 cites W2056462154 @default.
- W2034963123 cites W2057095204 @default.
- W2034963123 cites W2057647416 @default.
- W2034963123 cites W2057814921 @default.
- W2034963123 cites W2062712292 @default.
- W2034963123 cites W2063690295 @default.
- W2034963123 cites W2065169750 @default.
- W2034963123 cites W2065848862 @default.
- W2034963123 cites W2066261176 @default.
- W2034963123 cites W2066610953 @default.
- W2034963123 cites W2068845666 @default.
- W2034963123 cites W2070053976 @default.
- W2034963123 cites W2071580220 @default.
- W2034963123 cites W2072287157 @default.
- W2034963123 cites W2073297263 @default.
- W2034963123 cites W2074796477 @default.
- W2034963123 cites W2077369445 @default.
- W2034963123 cites W2081070804 @default.
- W2034963123 cites W2081802283 @default.
- W2034963123 cites W2082056883 @default.