| Item type |
デフォルトアイテムタイプ_（フル）(1) |
| 公開日 |
2023-03-18 |
| タイトル |
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|
タイトル |
Effect of Trehalose on the Properties of Mutant γPKC, Which Causes Spinocerebellar Ataxia Type 14, in Neuronal Cell Lines and Cultured Purkinje Cells |
|
言語 |
en |
| 作成者 |
Seki, Takahiro
Abe-Seki, Nana
Kikawada, Takahiro
Takahashi, Hideyuki
Yamamoto, Kazuhiro
Adachi, Naoko
Tanaka, Shigeru
Hide, Izumi
Saito, Naoaki
Sakai, Norio
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| アクセス権 |
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|
アクセス権 |
open access |
|
アクセス権URI |
http://purl.org/coar/access_right/c_abf2 |
| 権利情報 |
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|
権利情報 |
Copyright (c) 2010 by The American Society for Biochemistry and Molecular Biology, Inc. |
| 主題 |
|
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主題Scheme |
NDC |
|
主題 |
490 |
| 内容記述 |
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|
内容記述 |
Several missense mutations in the protein kinase C gamma (gamma PKC) gene have been found to cause spinocerebellar ataxia type 14 (SCA14), an autosomal dominant neurodegenerative disease. We previously demonstrated that the mutant gamma PKC found in SCA14 is susceptible to aggregation, which induces apoptotic cell death. The disaccharide trehalose has been reported to inhibit aggregate formation and to alleviate symptoms in cellular and animal models of Huntington disease, Alzheimer disease, and prion disease. Here, we show that trehalose can be incorporated into SH-SY5Y cells and reduces the aggregation of mutant gamma PKC-GFP, thereby inhibiting apoptotic cell death in SH-SY5Y cells and primary cultured Purkinje cells (PCs). Trehalose acts by directly stabilizing the conformation of mutant gamma PKC without affecting protein turnover. Trehalose was also found to alleviate the improper development of dendrites in PCs expressing mutant gamma PKC-GFP without aggregates but not in PCs with aggregates. In PCs without aggregates, trehalose improves the mobility and translocation of mutant gamma PKC-GFP, probably by inhibiting oligomerization and thereby alleviating the improper development of dendrites. These results suggest that trehalose counteracts various cellular dysfunctions that are triggered by mutant gamma PKC in both neuronal cell lines and primary cultured PCs by inhibiting oligomerization and aggregation of mutant gamma PKC. |
|
言語 |
en |
| 出版者 |
|
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出版者 |
American Society for Biochemistry and Molecular Biology Inc |
| 言語 |
|
|
言語 |
eng |
| 資源タイプ |
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|
資源タイプ識別子 |
http://purl.org/coar/resource_type/c_6501 |
|
資源タイプ |
journal article |
| 出版タイプ |
|
|
出版タイプ |
AO |
|
出版タイプResource |
http://purl.org/coar/version/c_b1a7d7d4d402bcce |
| 関連情報 |
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|
識別子タイプ |
DOI |
|
|
関連識別子 |
10.1074/jbc.M110.146704 |
| 関連情報 |
|
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|
識別子タイプ |
DOI |
|
|
関連識別子 |
http://dx.doi.org/10.1074/jbc.M110.146704 |
| 収録物識別子 |
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|
収録物識別子タイプ |
ISSN |
|
収録物識別子 |
0021-9258 |
| 収録物識別子 |
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|
収録物識別子タイプ |
NCID |
|
収録物識別子 |
AA00251083 |
| 開始ページ |
|
|
開始ページ |
33252 |
| 書誌情報 |
The Journal of Biological Chemistry
The Journal of Biological Chemistry
巻 285,
号 43,
p. 33252-33264,
発行日 2010-08-12
|
| 旧ID |
30787 |
JBC_285_33252.pdf (6.1 MB)
