2007 Fiscal Year Final Research Report Summary
Neuropathological studies on Parkinson's disease and amyotrophic lateral sclerosis
| Project/Area Number |
18590926
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| Research Category |
Grant-in-Aid for Scientific Research (C)
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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 | Gunma University |
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Principal Investigator |
OKAMOTO Koichi Gunma University, Department of Neurology, Graduate School of Medicine, Professor (00124652)
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| Co-Investigator(Kenkyū-buntansha) |
MIZUNO Yuji Gunma University, School of Medicine, Department of Neurology, Assistant Professor (20282395)
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| Project Period (FY) |
2006 – 2007
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| Keywords | Parkinson's disease / amyotrophic lateral sclerosis / Golgi apparatus / TDP-43 / Lewy body |
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| Research Abstract |
We examined whether the Golgi apparatus (GA) is fragmented in nigral neurons in Parkinson's disease (PD). We did not observe fragmented GA in nigral neurons in contoro cases by immunocytochemistry with an anti-TGN46 antibody. In PD, the GA was fragmented in 3% of the nigral neurons without inclusions, and in 5% of the neurons with Lewy bodies. In contrast, fragmented GA was noted in 19% of the neurons containing pale bodies. Since pale bodies represent early stages in the development of brainstem Lewy bodies, our results suggest that the cytotoxicity of alpha-synuclein-positive aggregates is reduced in the process of Lewy body formation. Transferrin, an iron-binding protein, plays an important role in the transport and delivery of circulating ferric iron to the tissues. We demonstrated that transferrin localized in Bunina bodies and some of the basophilic inclusions in ALS cases. In contrast, skein-like inclusions and Lewy body-like inclusions or round inclusions did not show obviously detectable transferrin immunoreactivities. Recently, TDP-43 was identified as a major component of ubiquitinated neuronal cytoplasmic inclusions observed in lower motor neurons in ALS and frontotemporal lobar degeneration with ubiquitinated inclusions. Almost all of the anterior horn cells with abnormal TDP-43 immunoreactivities showed GA fragmentation. These results suggest that neurons with abnormal TDP-43 immunoreactivities are associated with dysfunction of the secretory pathway in motor neurons in ALS.
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Research Products
(36 results)
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[Journal Article] Neuropathological diagnostic and nosologic criteria for frontotemporal lobar degeneration: consensus of Consortium for Frontotemporal Lobar Degeneration2007
Author(s)
Cairns NJ, Bigio EH, Mackenzie IRA, Neumann M, Lee VMY, Hatanpaa K, White III CL, Schneider JA, Grinberg LT, Halliday G, Duyckaerts C, Low JS, Holm IE, Tolnay M, Okamoto K, Yokoo H, Murayama S, Woulfe J, Munoz DG, Dickson DW, Ince PG, Trojanowski JQ, Mann
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Journal Title
Acta Neuropathol 114(1)
Pages: 5-20
Description
「研究成果報告書概要(和文)」より
Peer Reviewed
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[Journal Article] Familial amyloid polyneuropathy (Finnish type) in a Japanese famly: Clinical features and immunocytochemical studies2007
Author(s)
Ikeda M, Mizushima K, Fujita Y, Watanabe M, Sasaki A, Makioka K, Enoki M, Nakamura M, Otani T, Takatama M, Okamoto K
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Journal Title
J Neurol Sci 252(1)
Pages: 4-8
Description
「研究成果報告書概要(和文)」より
Peer Reviewed
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[Journal Article] Resistance to experimental autoimmune encephalomyelitis and impaired T cell priming by dendritic cells in src homology 2 domain-containing protein tyrosine phosphatase substrate-1 mutant mice2007
Author(s)
Tomizawa T, Kaneko Y, Kaneko Y, Saito Y, Ohnishi H, Okajo J, Okuzawa C, Ishikawa-Sekigami T, Murata Y, Okazwa H, Okamoto K, Nojima Y, Matozaki T
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Journal Title
J Immunol 179(2)
Pages: 869-877
Description
「研究成果報告書概要(和文)」より
Peer Reviewed
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[Journal Article] Neuropathological diagnostic and nosologic criteria for frontotemporal lobar degeneration: consensus of Consortium for Frontotemporal Lobar Degeneration2007
Author(s)
Cairns NJ, Bigio EH, Mackenzie IRA, Neumann M, Lee VMY, Hatanpaa K, White III CL, Schneider JA, Grinberg LT, Halliday G, Duyckaerts C, Low JS, Holm IE, Tolnay M, Okamoto K, Yokoo H, Murayama S, Woulfe J, Munoz DG, Dickson DW, Ince PG, Trojanowski JQ, Mann DMA
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Journal Title
Acta Neuropathol 114(1)
Pages: 5-20
Description
「研究成果報告書概要(欧文)」より
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[Journal Article] Familial amyloid polyneuropathy (Finnish type) in a Japanese family: Clinical features and immunocytochemical studies2007
Author(s)
Ikeda M, Mizushima K, Fujita Y, Watanabe M, Sasaki A, Makioka K, Enoki M, Nakamura M, Otani T, Takatama M, Okamoto K
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Journal Title
J Neurol Sci 252(1)
Pages: 4-8
Description
「研究成果報告書概要(欧文)」より
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[Presentation] PS1 M233L and H146R commonly presenting severe dementia, psychiatric symptoms and motor deficits but diverse progression of deterioration2007
Author(s)
Ikeda M, Yonemura K, Yoshida J, Hashimoto Y, Matsubara E, Tsukie T, Ishiguro K, Oriuchi N, Kuwano R, Harigaya Y, Okamoto K
Organizer
第23回国際脳循環代謝学会総会
Place of Presentation
大阪
Year and Date
20070520-24
Description
「研究成果報告書概要(和文)」より
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[Presentation] Protein synthesizing system in the motor neurons in the spinal cord in amyotrophic lateral sclerosis: pursuing the beginning of the alterations.2006
Author(s)
Oyanagi K, Nagasao J, Yamazaki M, Okamoto K, Aoki M, Watabe K, Wada M, Morita T, Takahashi H, Mizutani T, Hayashi H.
Organizer
17 th International Symposium of ALS/MND
Place of Presentation
横浜
Year and Date
20061130-1202
Description
「研究成果報告書概要(和文)」より
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[Presentation] Protein synthesizing system in the motor neurons in the spinal cord in amyotrophic lateral sclerosis pursuing the beginning of the alterations2006
Author(s)
Oyanagi K, Nagasao J, Yamazaki M, Okamoto K, Aoki M, Watabe K, Wada M, Morita T, Takahashi H, Mizutani T, Hayashi H
Organizer
17th International Symposium of ALSIMND
Place of Presentation
Yokohama
Year and Date
20061130-1202
Description
「研究成果報告書概要(欧文)」より
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