2014 Fiscal Year Final Research Report
Proteome analysis for spinocerebellar ataxia type 31
| Project/Area Number |
24591255
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| Research Category |
Grant-in-Aid for Scientific Research (C)
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| Allocation Type | Multi-year Fund |
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| Section | 一般 |
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| Research Field |
Neurology
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| Research Institution | Shinshu University |
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Principal Investigator |
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| Co-Investigator(Kenkyū-buntansha) |
OYANAGI Kiyomitsu 信州大学, 医学部, 特任教授 (00134958)
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| Project Period (FY) |
2012-04-01 – 2015-03-31
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| Keywords | 脊髄小脳失調症31型 / プルキンエ細胞 / Golgi装置断片化 / RNA結合蛋白 |
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| Outline of Final Research Achievements |
We performed neuropathological examinations on two autopsied brains from patients with spinocerebellar ataxia type 31 (SCA31). We found there were two degenerating pathways of Purkinje cells with or without halo-like structures. In Purkinje cells with halo-like structures, the nuclear deformity and fragmentation of the Golgi apparatus were more frequently observed than in those without halo-like structures.
Using biotin-labeled (UGGAA)n probe, we tried to identify proteins that bound to pre-messenger RNA with abnormally expanded (UGGAA) repeat in SCA31 brains. As a result, a protein identified in our experiments was a mitochondrial inner membrane protein. This might be false positive, but based on neuropathological observations, it is possible that abnormal pre-messenger RNA could pass through fragile nuclear membrane and interact with proteins involving cytoplasmic organelles in SCA31.
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| Free Research Field |
脊髄小脳変性症
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