2013 Fiscal Year Final Research Report
Polyglutamine oligomers in human brain play a crucial role in the pathogenesis of polyglutamine diseases.
Project/Area Number |
23591235
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Research Category |
Grant-in-Aid for Scientific Research (C)
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Allocation Type | Multi-year Fund |
Section | 一般 |
Research Field |
Neurology
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Research Institution | Niigata University |
Principal Investigator |
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Co-Investigator(Renkei-kenkyūsha) |
TADA Masayoshi 新潟大学, 脳研究所, 助教 (10467079)
KATADA Shinnichi 新潟大学, 医歯学総合病院, 特任助教 (70599167)
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Project Period (FY) |
2011 – 2013
|
Keywords | polyglutamine / oligomer / DRPLA / C.elegans / SDD-AGE |
Research Abstract |
Expanded polyglutamine proteins cause neurodegenerative disorders. Although polyglutamine oligomers have been proposed as a cytotoxic structure, the cytotoxicity of polyglutamine oligomers, not inclusion bodies, has not been proven in human brain tissue. To clarify the cytotoxicity of polyglutamine oligomers, we carried out Semi-Denaturing Detergent-Agarose Gel Electrophoresis (SDD-AGE) and detected oligomers in autopsy brain tissue in DRPLA patients. Quantitative analysis indicates that accumulation of polyglutamine oligomers correlate with the severity of neuronal loss in human brain tissue. These results show that oligomers in human brain play a crucial role in the pathogenesis of polyglutamine diseases.
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