A pathogenesis study of Perry syndrome
Project/Area Number |
26860678
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Research Category |
Grant-in-Aid for Young Scientists (B)
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Allocation Type | Multi-year Fund |
Research Field |
Neurology
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Research Institution | Fukuoka University |
Principal Investigator |
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Project Period (FY) |
2014-04-01 – 2018-03-31
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Project Status |
Completed (Fiscal Year 2017)
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Budget Amount *help |
¥3,640,000 (Direct Cost: ¥2,800,000、Indirect Cost: ¥840,000)
Fiscal Year 2016: ¥910,000 (Direct Cost: ¥700,000、Indirect Cost: ¥210,000)
Fiscal Year 2015: ¥910,000 (Direct Cost: ¥700,000、Indirect Cost: ¥210,000)
Fiscal Year 2014: ¥1,820,000 (Direct Cost: ¥1,400,000、Indirect Cost: ¥420,000)
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Keywords | Perry症候群 / DCTN1 / TDP-43 / モデルマウス / iPS細胞 / 診断基準 / トランスジェニックマウス / ダイナクチン / 家族性パーキンソン病 / パーキンソン病 |
Outline of Final Research Achievements |
【Clinical study】We reported clinical features of patients with Perry syndrome. We established international diagnostic criteria for Perry syndrome. 【Basic study】We showed that Perry syndrome is a distinctive type of TDP-43 proteinopathy. We generated induced pluripotent stem cells (iPSCs) from a Perry syndrome patient and differentiated iPSCs into tyrosine hydroxylase (TH)-positive neurons. TH-positive neurons from a patient with Perry syndrome had dynactin aggregations in cytoplasm. Patient TH-positive neurons recapitulated an aspect of the disease phenotype of Perry syndrome. We generated DCTN1 G71A transgenic mice. These mice showed decreased exploratory activity and impaired motor coordination. These behavioral defects paralleled apathy-like symptoms and parkinsonism encountered in Perry syndrome.
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Report
(5 results)
Research Products
(18 results)
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[Journal Article] Establishing diagnostic criteria for Perry syndrome2018
Author(s)
Mishima T, Fujioka S, Tomiyama H, Yabe I, Kurisaki R, Fujii N, Neshige R, Ross OA, Farrer MJ, Dickson DW, Wszolek ZK, Hattori N, Tsuboi Y
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Journal Title
J Neurol Neurosurg Psychiatry
Volume: 89
Issue: 5
Pages: 482-487
DOI
Related Report
Peer Reviewed / Open Access / Int'l Joint Research
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[Journal Article] Behavioral defects in a DCTN1 G71A transgenic mouse model of Perry syndrome2018
Author(s)
Mishima T, Deshimaru M, Watanabe T, Kubota K, Kinoshita-Kawada M, Yuasa-Kawada J, Takasaki K, Uehara Y, Jinno S, Iwasaki K, Tsuboi Y
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Journal Title
Neurosci Lett
Volume: 666
Pages: 98-103
Related Report
Peer Reviewed
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[Journal Article] Cytoplasmic aggregates of dynactin in iPSC-derived tyrosine hydroxylase-positive neurons from a patient with Perry syndrome2016
Author(s)
Mishima T, Ishikawa T, Imamura K, Kondo T, Koshiba Y, Takahashi R, Takahashi J, Watanabe A, Fujii N, Tsuboi Y, Inoue H
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Journal Title
Parkinsonism & Related Disorders
Volume: 30
Pages: 67-72
DOI
Related Report
Peer Reviewed / Acknowledgement Compliant
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[Presentation] Human iPSC disease modeling of Perry syndrome2017
Author(s)
Mishima T, Ishikawa T, Imamura K, Kondo T, Koshiba Y, Takahashi R, Takahashi J, Watanabe A, Fujii N, Tsuboi Y, Inoue H
Organizer
XXIII World Congress of Neurology
Related Report
Int'l Joint Research
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[Presentation] Disease modeling for Perry syndrome using patient induced pluripotent stem cells2017
Author(s)
Mishima T, Ishikawa T, Imamura K, Kondo T, Koshiba Y, Takahashi R, Takahashi J, Watanabe A, Fujii N, Tsuboi Y, Inoue H
Organizer
21th International Congress of Parkinson's Disease and Movement Disorders
Related Report
Int'l Joint Research
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[Presentation] A cellular model for Perry syndrome using patient iPSCs2016
Author(s)
Mishima T, Ishikawa T, Imamura K, Kondo T, Koshiba Y, Takahashi R, Takahashi J, Watanabe A, Fujii N, Tsuboi Y, Inoue H
Organizer
第57回日本神経学会学術大会
Place of Presentation
神戸コンベンションセンター(兵庫・神戸市)
Year and Date
2016-05-18
Related Report
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