2013 Fiscal Year Final Research Report
Comprehensive study on dystonia pathology
| Project/Area Number |
23500428
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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 |
Nerve anatomy/Neuropathology
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| Research Institution | The University of Tokushima |
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Principal Investigator |
GOTO Satoshi 徳島大学, ヘルスバイオサイエンス研究部, 特任教授 (50240916)
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| Co-Investigator(Kenkyū-buntansha) |
KAJI Ryuji 徳島大学, 大学院・ヘルスバイオサイエンス研究部, 教授 (00214304)
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| Project Period (FY) |
2011 – 2013
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| Keywords | ジストニア / 神経病理 / 機能解剖 / 線条体 |
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| Research Abstract |
In the mammalian striatum, there exist two functional subdivisions termed as striosome and matrix compartments. We have proposed a hypothesis that an imbalanced activities between the striosome and matrix compartments may cause movement disorders that include dystonia. During the last 3 years, we found a selective loss of dopamine D1 receptor D1R and Golf expression in the striosomes in a transgenic mouse model with dystonia, and suggest that a loss of D1Rmediated signaling in the striosomes may cause dystonia symptoms. Moreover, our pathological study on the autopsied brains showed the defects in the neuropeptide NPY system in the striatum of the patients with DYT3 dystonia. Since NPY has a protective role against glutamate receptor-mediated neurotoxicity and promotes striatal neurogenesis, this finding suggests that defects in the NPY system may underlie the genesis of progressive striatal generation that primarily occurs in the striosome compartment.
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[Journal Article] Depression in X-linked dystonia-parkinsonism : a case-control study2013
Author(s)
R. Morigaki, Nakataki, T. Kawarai, L.V . Lee, R.A. Teleg, M.D. Tabuena, H. Mure, W. Sako, P.M. Pasco, S. Nagahiro, J. Iga, T. Ohmori, S. Goto & R. Kaji
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Journal Title
Parkinsonism Relat Disord
Volume: 19
Pages: 844-846
DOI
Peer Reviewed
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[Journal Article] Application of long-range polymerase chain reaction in the diagnosis of X-linked dystonia-parkinsonism2013
Author(s)
T. Kawarai, P .M. Pasco, R.A. Teleg, M. Kamada, W. Sako, K. Shimozono, M. Mizuguchi, D. Tabuena, A. Orlacchio, Y. Izumi, S. Goto, L.V. Lee & R. Kaji
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Journal Title
Neurogenetics
Volume: 14
Pages: 167-169
DOI
Peer Reviewed
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[Journal Article] Parkin interacts with Klokin1 for mitochondrial import and maintenance of membrane potential2012
Author(s)
Y. Kuroda, W. Sako, S. Goto, T. Sawada, D. Uchida, Y. Izumi, T. Takahashi, N. Kagawa, M. Matsumoto, M. Matsumoto, R. Takahashi, R. Kaji & T. Mitsui
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Journal Title
Hum Mol Genet
Volume: 21
Pages: 991-1003
DOI
Peer Reviewed
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[Journal Article] The TRK-fused gene is mutated in hereditary motor and sensory neuropathy with proximal dominant involvement2012
Author(s)
H. Ishiura, W. Sako, M. Yoshida, T. Kawarai, O. Tanabe, J. Goto, Y. Takahashi, H. Date, J. Mitsui, B. Ahsan, Y. Ichikawa, A. Iwata, H. Yoshino, Y. Izumi, K. Fujita, K. Maeda, S. Goto, R. Kaji & S. Tsuji (他9名)
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Journal Title
Am J Hum Genet
Volume: 91
Pages: 320-329
DOI
Peer Reviewed
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[Journal Article] Generalized dystonia in a patient with a novel mutation in the GLUD1 gene2012
Author(s)
R. Miyamoto, S. Goto, W. Sako, A. Miyashiro, I. Kim, F . Escande, M. Harada, R. Morigaki, K. Asanuma, Y. Mizobuchi, S. Nagahiro, Y. Izumi & R. Kaji
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Journal Title
Mov Disord
Volume: 27
Pages: 1198-1199
DOI
Peer Reviewed
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