2013 Fiscal Year Final Research Report
Therapeutic strategy for the polyglutamine diseases by selective degradation of expanded polyglutamine proteins using polyglutamine-binding pepetides
| Project/Area Number |
23390237
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| Research Category |
Grant-in-Aid for Scientific Research (B)
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| Allocation Type | Single-year Grants |
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| Section | 一般 |
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| Research Field |
Neurology
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| Research Institution | National Center of Neurology and Psychiatry |
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Principal Investigator |
NAGAI Yoshitaka 独立行政法人国立精神・神経医療研究センター, 神経研究所疾病研究第四部, 室長 (60335354)
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| Co-Investigator(Renkei-kenkyūsha) |
KOMATSU Masaaki (財)東京都臨床医学総合研究所, プロジェクトリーダー (90356254)
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| Project Period (FY) |
2011-04-01 – 2014-03-31
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| Keywords | 神経科学 / 蛋白質 / 脳神経疾患 / 治療 / 蛋白質分解 / 神経変性疾患 / ポリグルタミン病 / 分子デザイン |
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| Research Abstract |
Toward developing a therapy for the polyglutamine (polyQ) neurodegenerative diseases by specific degradation of expanded polyQ proteins, 1) we showed that p62 plays an important role in the autophagic clearance of polyQ protein, especially its oligomers, resulting in protection against polyQ-induced neurodegeneration in Drosophila. 2) We further designed the p62-QBP1 chimeric protein, which consists of p62, an adaptor protein for selective autophagy and the expanded polyQ-specific binding peptide QBP1, and demonstrated its therapeutic potential for the polyQ diseases by selective degradation of expanded polyQ proteins.
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[Journal Article] Identification of ter94, Drosophila VCP, as a strong modulator of motor neuron degeneration induced by knockdown of Caz, Drosophila FUS2014
Author(s)
Azuma Y., Tokuda T., Shimamura M., Kyotani A., Sasayama H., Yoshida T., Mizuta I., Mizuno T., Nakagawa M., Fujikake N., Ueyama M., Nagai Y., Yamaguchi M
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Journal Title
Hum. Mol. Genet
Volume: 23(13)
Pages: 3467-3480
DOI
Peer Reviewed
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[Journal Article] Genetic and clinical analysis in a Chinese parkinsonism-predominant spinocerebellar ataxia type 2 family2011
Author(s)
Sun H, Satake W, Zhang C, Nagai Y, Tian Y, Fu S, Yu J, Qian Y, Qian Y, Chu J, Toda T
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Journal Title
J Hum Genet
Volume: 56 (4)
Pages: 330-334
DOI
Peer Reviewed
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