Disturbance of brain environment by impairment of proteolysis leading to neurodegeneration

• Project Area: Brain Environment
• Project/Area Number: 23111002
• Research Category: Grant-in-Aid for Scientific Research on Innovative Areas (Research in a proposed research area)
• Allocation Type: Single-year Grants
• Review Section: Biological Sciences
• Research Institution: Kyoto University
• Principal Investigator: Takahashi Ryosuke 京都大学, 医学(系)研究科(研究院), 教授 (90216771)
• Co-Investigator(Kenkyū-buntansha): Urushitani Makoto 京都大学, 大学院医学研究科, 准教授 (60332326)
• Project Period (FY): 2011-04-01 – 2016-03-31
• Project Status: Completed (Fiscal Year 2015)
• Budget Amount: ¥201,500,000 (Direct Cost: ¥155,000,000、Indirect Cost: ¥46,500,000); Fiscal Year 2015: ¥37,050,000 (Direct Cost: ¥28,500,000、Indirect Cost: ¥8,550,000); Fiscal Year 2014: ¥37,050,000 (Direct Cost: ¥28,500,000、Indirect Cost: ¥8,550,000); Fiscal Year 2013: ¥39,130,000 (Direct Cost: ¥30,100,000、Indirect Cost: ¥9,030,000); Fiscal Year 2012: ¥38,740,000 (Direct Cost: ¥29,800,000、Indirect Cost: ¥8,940,000); Fiscal Year 2011: ¥49,530,000 (Direct Cost: ¥38,100,000、Indirect Cost: ¥11,430,000)
• Keywords: ALS / Proteasome / Autophagy / Medaka / Gaucher disease / GBA / Lysosome / Alpha-synuclein / 脳神経疾患 / 神経科学 / 遺伝子 / 内科 / トランスレーショナルリサーチ / proteasome / micorarray / TDP-43 / Cullin-2 / VHL / 筋萎縮性側索硬化症 / プロテアソーム分解系 / 神経外伝播 / トランスジェニックマウス / Rpt3

Outline of Final Research Achievements

We show that impairment of the ubiquitin-proteasome system, but not the autophagy-lysosome system in motor neurons replicates ALS in mice. Conditional knock-out mice of the proteasome subunit Rpt3 in a motor neuron-specific manner (Rpt3-CKO) showed locomotor dysfunction and typical ALS cytopathology. On the other hand, motor neuron-specific knock-out of Atg7 (Atg7-CKO), only resulted in no TDP-43 or FUS pathologies or motor dysfunction was observed. These results strongly suggest that proteasomes, but not autophagy, fundamentally govern the development of ALS. Recent genetic studies have revealed that GBA mutations confer a strong risk for sporadic Parkinson's disease (PD). To investigate how GBA mutations cause PD, we generated GBA knockout medaka that are deficient in glucocerebrosidase activity. Pathological findings represented lysosomal abnormalities in neurons and alpha-synuclein accumulation in axonal swellings, linking GBA mutation and pathogenesis of PD

Research Products

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• [Journal Article] Different Mode of Afferents Determines the Frequency Range of High Frequency Activities in the Human Brain: Direct Electrocorticographic Comparison between Peripheral Nerve and Direct Cortical Stimulation (2015)
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  • Peer Reviewed
• [Journal Article] Drug screening for ALS using patient-specific induced pluripotent stem cells (2012)
  • Author(s): Egawa, N., Kitaoka, S., Tsukita, K., Naitoh, M., Takahashi, K., Yamamoto, T., Adachi, F., Kondo, T., Okita, K., Asaka, I., Aoi, T., Watanabe, A., Yamada, Y., Morizane, A., Takahashi, J., Ayaki, T., Ito, H., Yoshikawa, K., Yamawaki, S., Suzuki, S., Watanabe, D., Hioki, H., Kaneko, T., Makioka, K., Okamoto, K., Takuma, H., Tamaoka, A., Hasegawa, K., Nonaka, T., Hasegawa, M., Kawata, A., Yoshida, M., Nakahata, T., Takahashi, R., Marchetto, M. C. N., Gage, F. H., Yamanaka, S., and Inoue, H
  • Journal Title: Sci. Transl. Med. Volume: 4 Issue: 145 Pages: 1-8
  • DOI: 10.1126/scitranslmed.3004052
  • Peer Reviewed
• [Journal Article] "Hot cross bun" sign in multiple system atrophy with predominant cerebellar ataxia: A comparison between proton density-weighted imaging and T2-weighted imaging. (2012)
  • Author(s): Kasahara S, Miki Y, Kanagaki M, Kondo T, Yamamoto A, Morimoto E, Okada T, Ito H, Takahashi R, Togashi K
  • Journal Title: Eur J Radiol Volume: 81(10) Issue: 10 Pages: 2848-52
  • DOI: 10.1016/j.ejrad.2011.12.012
  • NAID: 120004873778
  • Peer Reviewed
• [Journal Article] In vivo imaging of brain ischemia using an oxygen-dependent degradative fusion prote in probe (2012)
  • Author(s): Fujita Y, Kuchimaru T、Kadonosono T, Tanaka S, Hase Y, Tomimoto H, Hiraoka M, Kizaka-Kondoh S, Ihara M, Takahashi R
  • Journal Title: PLoS ONE Volume: 7 Issue: 10 Pages: e48051-e48051
  • DOI: 10.1371/journal.pone.0048051
  • Peer Reviewed
• [Journal Article] Parkinson's disease-associated kinase PINK1 regulates Miro protein level and axonal transport of mitochondria (2012)
  • Author(s): Liu Song
  • Journal Title: PLoS Genet Volume: 8 Issue: 3 Pages: e1002537-e1002537
  • DOI: 10.1371/journal.pgen.1002537
  • Peer Reviewed
• [Journal Article] Clinicopathologic study on an ALS family with a heterozygous E478G optineurin mutation (2011)
  • Author(s): Ito H, Nakamura M, Komure O, Ayaki T, Wate R, Maruyama H, Nakamura Y, FujitaK, Kaneko S, Okamoto Y, Ihara M, Konishi T, Ogasawara K, Hirano A, Kusaka H, KajiR, Takahashi R, Kawakami H
  • Journal Title: Acta Neuropathologica Volume: 122 Issue: 2 Pages: 223-229
  • DOI: 10.1007/s00401-011-0842-y
  • Peer Reviewed
• [Journal Article] Protein misdirection inside and outside motor neurons in ALS : a possible clue for therapeutic strategies (2011)
  • Author(s): Ido A, et al
  • Journal Title: Int J Mol Sci Volume: 12 Issue: 10 Pages: 6980-7003
  • DOI: 10.3390/ijms12106980
  • Peer Reviewed
• [Journal Article] An autopsy case of SOD1-related ALS with TDP-43 positive inclusions (2011)
  • Author(s): Okamoto Y, Ihara M, Urushitani M, Yamashita H, Kondo T, Tanigaki A, Oono M, Kawamata J, Ikemoto A, Kawamoto Y, Takahashi R, Ito H
  • Journal Title: Neurology Volume: 77:1993-1995 Issue: 22 Pages: 1993-1995
  • DOI: 10.1212/wnl.0b013e31823a0cfc
  • Peer Reviewed
• [Journal Article] Co localization of 14-3-3 proteins with SOD1 in Lewy Body-like hyaline inclusions in familial amyotrophic lateral sclerosis cases and the animal model (2011)
  • Author(s): Okamoto Y, Shirakashi Y, Ihara M, Urushitani M, Oono M, Kawamoto Y, Yamashita H, Shimohama S, Kato S, Hirano A, Tomimoto H, Ito H, Takahashi R
  • Journal Title: PLoS One Volume: 6 Issue: 5 Pages: e20427-e20427
  • DOI: 10.1371/journal.pone.0020427
  • Peer Reviewed
• [Journal Article] In vivo detection of amyloid β deposition using 19F magnetic resonance imaging with a 19F-containing curcurain derivative in a mouse model of Alzheimer's disease (2011)
  • Author(s): 柳沢大治郎
  • Journal Title: Neuroscience Volume: 184 Pages: 120-127
  • DOI: 10.1016/j.neuroscience.2011.03.071
  • Peer Reviewed
• [Journal Article] 筋萎縮性側索硬化症の免疫療法 (2011)
  • Author(s): 漆谷真
  • Journal Title: 炎症と免疫 Volume: 119 Pages: 557-563
• [Journal Article] 神経変性疾患に対する免疫療法の現状 (2011)
  • Author(s): 大野美樹、漆谷真
  • Journal Title: 細胞工学 Volume: 30 Pages: 1054-1059
• [Presentation] Fish model of Parkinson’s disease. (2016)
  • Author(s): Takahashi R
  • Organizer: Keio Longevity Seminar Series “Resilience in Brain Agin –From Model Systems of Neurodegenerastion to Supercentenarian Study” Keio Longevity Initiative Seminar Series,
  • Place of Presentation: 東京
  • Year and Date: 2016-02-18
  • Invited
• [Presentation] The Mechanism of Motor Neuron Degeneration in Ubiquitin Proteasome System Dysfunction (2015)
  • Author(s): 星野 友則、Yamashita H, Tashiro Y, Misawa H, Makoto U, Takahashi R
  • Organizer: BMB2015
  • Place of Presentation: 神戸
  • Year and Date: 2015-12-02
• [Presentation] 孤発性パーキンソン病の遺伝的リスク因子を利用した新しいパーキンソン病モデルマウス作製の試み (2015)
  • Author(s): 生野真嗣、浅野剛史、山門穂高、高橋良輔
  • Organizer: 第9回パーキンソン病・運動障害疾患コングレス
  • Place of Presentation: 東京
  • Year and Date: 2015-10-16
• [Presentation] Medaka fish model of Parkinson’s disease (2015)
  • Author(s): Norihito Uemura, Ryosuke Takahashi
  • Organizer: 10th GEO-PD
  • Place of Presentation: 東京
  • Year and Date: 2015-10-02
• [Presentation] Gaucher disease model in medaka displays axonal accumulation of alpha-synuclein (2015)
  • Author(s): Norihito Uemura
  • Organizer: 第58回日本神経化学会大会
  • Place of Presentation: さいたま
  • Year and Date: 2015-09-12
• [Presentation] 神経型ゴーシェ病メダカは軸索にアルファシヌクレイン蓄積を示す (2015)
  • Author(s): 上村紀仁
  • Organizer: BMB2015
  • Place of Presentation: 神戸
  • Year and Date: 2015-09-12
• [Presentation] Current Perspectives and Future Directions (2015)
  • Author(s): Ryosuke Takahashi
  • Organizer: ASEANNeuroscience 2015
  • Place of Presentation: シンガポール
  • Year and Date: 2015-07-31
• [Presentation] Parkinson Disease Treatment: Current Perspective and Future Directions.(Plenary Lecture) (2015)
  • Author(s): Takahashi R
  • Organizer: ASEAN 2015 Neuroscience
  • Place of Presentation: Singapore
  • Year and Date: 2015-07-30
  • Invited
• [Presentation] Pathological role of GBA2 in GBA1-deficient neuronopathic Gaucher’s disease model of medaka (2015)
  • Author(s): Yosuke Taruno, Hideaki Matsui, Norihito Uemura, Hodaka Yamakado, Ryoruke Takahashi
  • Organizer: 第38回日本神経科学大会
  • Place of Presentation: 神戸
  • Year and Date: 2015-07-30
• [Presentation] FUS遺伝子変異を伴う筋萎縮性側索硬化症の臨床像：若年発症例を含む4症例の検討. (2015)
  • Author(s): 引網亮太,, 端祐一郎, 山門穂高, 辰己 新水, 山下博史, 澤本伸克, 辻輝之, 川本未知, 幸原伸夫, 井上治久, 漆谷真, 高橋良輔.
  • Organizer: 第56回日本神経学会 学術集会
  • Place of Presentation: 新潟
  • Year and Date: 2015-05-23
• [Presentation] Creating mice models for Parkinson's disease based on its genetic risk factors (2015)
  • Author(s): 生野 真嗣、浅野剛史、山門穂高、高橋良輔
  • Organizer: 第56回日本神経学会 学術集会
  • Place of Presentation: 新潟
  • Year and Date: 2015-05-23
• [Presentation] VHL とCUL2; TDP-43 の凝集を促進するVHL と分解を促進するCUL2. TDP-43 proteinopathy におけるVHL の2 面性. (2015)
  • Author(s): 内田 司, 玉木良高, 小代明美, 綾木 孝, 守村敏史, 辰巳新水, 伊東秀文, 坂下 直実, 高橋良輔, 漆谷真.
  • Organizer: 第56回日本神経学会 学術集会
  • Place of Presentation: 新潟
  • Year and Date: 2015-05-22
• [Presentation] Immunohistochemical localization of HAX-1 and PALR in Lewy bodies in PD and DLB. (2015)
  • Author(s): 河本恭裕、辰己新水、漆谷 真、伊東秀文、髙橋良輔.
  • Organizer: 第56回日本神経学会 学術集会
  • Place of Presentation: 新潟
  • Year and Date: 2015-05-22
• [Presentation] Analysis of the ATP13A2mutant (PARK9) medaka (Oryziaslatipes) (2015)
  • Author(s): 樽野 陽亮、松井秀明 上村紀仁 山門穂高 髙橋良輔
  • Organizer: 第56回日本神経学会 学術集会
  • Place of Presentation: 新潟
  • Year and Date: 2015-05-22
• [Presentation] 多発性硬化症患者におけるTLR/CD40刺激によるB細胞サイトカインの解析 (2015)
  • Author(s): 岡田洋一郎、藤井ちひろ、端祐一郎、越智博文、中川正法、水野敏樹、松本禎之、漆谷 真、髙橋良輔、近藤誉之.
  • Organizer: 第56回日本神経学会 学術集会
  • Place of Presentation: 新潟
  • Year and Date: 2015-05-21
• [Presentation] Clinicopathologic study of autopsied familial ALS cases with optineurin mutation. (2015)
  • Author(s): 綾木 孝, 伊東秀文、小牟禮修、辰己新水、鎌田正紀、漆谷 真、川上秀史、髙橋良輔.
  • Organizer: 第56回日本神経学会 学術集会
  • Place of Presentation: 新潟
  • Year and Date: 2015-05-21
• [Presentation] ApoE Isoforms Differentially Affect Amyloid beta-Induced Synaptic Toxicity (2015)
  • Author(s): 上村 麻衣子、植村健吾、木下彩栄、高橋良輔
  • Organizer: 第56回日本神経学会 学術集会
  • Place of Presentation: 新潟
  • Year and Date: 2015-05-21
• [Presentation] Hereditary Diffuse Leukoencephalopathy with Spheroids: A Hidden Culprit. (2015)
  • Author(s): 梶 誠兒、沖亮祐、宮本亮介、瓦井俊孝、和泉唯信、梶龍兒、高橋良輔
  • Organizer: 第56回日本神経学会 学術集会
  • Place of Presentation: 新潟
  • Year and Date: 2015-05-21
• [Presentation] Microarray analysis in Motor Neuron-Specific 26S Proteasome Conditional KO Mice (2015)
  • Author(s): 星野 友則、Yamashita H, Tashiro Y, Misawa H, Takahashi R
  • Organizer: 第56回日本神経学会 学術集会
  • Place of Presentation: 新潟
  • Year and Date: 2015-05-21
• [Presentation] Gaucher disease model in medaka displays axonal accumulation of alpha-synuclein (2015)
  • Author(s): 上村紀仁
  • Organizer: 第56回日本神経学会 学術集会
  • Place of Presentation: 新潟
  • Year and Date: 2015-05-20
• [Presentation] Molecular epitope identifi cation for misfolded/unfolded TDP-43 in ALS (2014)
  • Author(s): Makoto Urushitani
  • Organizer: 10th Annual Symposium of ALS of the Foundation Andre-Delambre.
  • Place of Presentation: カナダ
  • Year and Date: 2014-09-19
  • Int'l Joint Research / Invited
• [Presentation] Alpha-synuclein accumulation in Gaucher disease model of medaka does not contribute to neurodegeneration (2014)
  • Author(s): Norihito Uemura, Tomoko Fujiwara-Ishikawa, Masato Kinoshita, Masato Koike, Hideaki Matsui, Hodaka Yamakado, Yasuo Uchiyama, Takeshi Todo, Shun-ichi Takeda, Ryosuke Takahashi
  • Organizer: 第37回日本神経科学大会
  • Place of Presentation: 横浜
  • Year and Date: 2014-09-12
• [Presentation] The role of conformation of RNA recognition motifs of TDP-43 in the protein quality control system in ALS (2014)
  • Author(s): Makoto Urushitani
  • Organizer: 6th Japan-Canada joint symposium. 37th Annual Meeting of Japan Neuroscience Society
  • Place of Presentation: 横浜
  • Year and Date: 2014-09-12
  • Invited
• [Presentation] ALSのTDP-43のinclusion形成におけるVon Hippel-Lindau proteinの役割 (2014)
  • Author(s): 内田 司，小代 明美，守村 敏史，辰巳 新水，高橋 良輔，漆谷 真
  • Organizer: 第37回日本神経科学大会
  • Place of Presentation: 横浜
  • Year and Date: 2014-09-11
• [Presentation] Optineurin suppression causes neuronal cell death via NF-κB pathway (2014)
  • Author(s): Mayumi Akizuki, Hirofumi Yamashita, Kengo Uemura, Hirofumi Maruyama, Hideshi Kawakami, Hidefumi Ito, Ryosuke Takahashi
  • Organizer: 14th asian ＆ oceanian congress of Neurology
  • Place of Presentation: マカオ
• [Presentation] PINK1とParkinは脊椎動物において相補的にミトコンドリアを保護する (2013)
  • Author(s): 浅野 剛史、松井 秀彰、 Robert Gavinio、上村紀人、伊東秀文、谷口善仁、Jie Shen、武田 俊一、植村 健吾、山門 穂高、髙橋 良輔
  • Organizer: 第３６回 日本分子生物学会
  • Place of Presentation: 神戸 国際会議場
• [Presentation] Neurodegeneration, molecular mechanism, Parkinson's diease, animal model, medaka, mouse. (2013)
  • Author(s): Ryosuke Takahashi
  • Organizer: Swiss-Kyoto Symposium
  • Place of Presentation: 京都 京都大学
• [Presentation] Viable Gaucher's disease model of medaka fish completely deficienct in glucocerebrosidase activity developed alpha-synuclein aggregation in the brain (2013)
  • Author(s): Norihito Uemura, Tomoko Fujiwara-Ishikawa, Masato Kinoshita, Masato Koike, Hideaki Matsui, Hodaka Yamakado, Kengo Uemura, Yasuo Uchiyama, Takeshi Todo, Shun-ichi Takeda and Ryosuke Takahashi
  • Organizer: Neuroscience 2013
  • Place of Presentation: SanDiego
• [Presentation] ATP13A2変異メダカの解析 (2013)
  • Author(s): 樽野陽亮、谷口善仁、松井秀彰、上村紀仁、山門穂高、高橋良輔
  • Organizer: 第７回パーキンソン病・運動障害疾患コングレス
  • Place of Presentation: 東京 京王プラザホテル
• [Presentation] マウスを用いたゾニサミドの作用機序の解析 (2013)
  • Author(s): 浅野剛史、山門穂高、高橋良輔
  • Organizer: 第７回 パーキンソン病・運動障害疾患コングレス
  • Place of Presentation: 東京 京王プラザホテル
• [Presentation] 疾患特異的iPS細胞モデルによるLRRK2変異パーキンソン病の研究 (2013)
  • Author(s): 小芝泰、森実飛鳥、山門穂高、陣上直人、菊地哲広、土井大輔、皆川栄子、江川斉宏、井上治久、髙橋淳、髙橋良輔
  • Organizer: 第７回 パーキンソン病・運動障害疾患コングレス
  • Place of Presentation: 東京 京王プラザホテル
• [Presentation] NF-κB経路を介したOptineurinのノックダウンによる神経細胞死 (2013)
  • Author(s): 秋月真由美、山下博史、植村健吾、丸山博文、川上秀史、伊東秀文、高橋良輔
  • Organizer: 第22回 cell death学会
  • Place of Presentation: 京都 京都大学
• [Presentation] GBAノックアウトメダカは脳にアルファシヌクレイン凝集体を形成する (2013)
  • Author(s): 上村紀仁、石川智子、木下政人、小池正人、松井秀彰、山門穂高、植村健吾、内山安男、藤堂剛、武田俊一、高橋良輔
  • Organizer: 第22回 日本Cell death 学会
  • Place of Presentation: 京都 京都大学
• [Presentation] PINK1とParkinは脊椎動物において相補的にミトコンドリアを保護する (2013)
  • Author(s): 浅野 剛史、松井 秀彰、 Robert Gavinio、上村 紀人、伊東 秀文、谷口 善仁、Jie Shen、武田 俊一、植村 健吾、山門 穂高、髙橋 良輔
  • Organizer: 第22回 日本Cell death 学会
  • Place of Presentation: 京都 京都大学
• [Presentation] Viable Gaucher's disease model of medaka fish completely deficienct in glucocerebrosidase activity developed alpha-synuclein aggregation in the brain (2013)
  • Author(s): Norihito Uemura1, Tomoko Fujiwara-Ishikawa2, Masato Kinoshita3, Masato Koike6, Hideaki Matsui, Hodaka Yamakado, Kengo Uemura, Yasuo Uchiyama, Takeshi Todo, Shun-ichi Takeda and Ryosuke Takahashi
  • Organizer: 日本神経科学学会
  • Place of Presentation: 京都 国際会議場
• [Presentation] ATP13A2変異メダカの解析 (2013)
  • Author(s): 樽野陽亮、谷口善仁、松井秀彰、上村紀仁、山門穂高、高橋良輔
  • Organizer: 日本神経科学学会
  • Place of Presentation: 京都 国際会議場
• [Presentation] Viable Gaucher's disease model of medaka fish completely deficienct in glucocerebrosidase activity developed alpha-synuclein aggregation in the brain (2013)
  • Author(s): Norihito Uemura, Tomoko Fujiwara-Ishikawa, Masato Kinoshita, Masato Koike, Hideaki Matsui, Hodaka Yamakado, Kengo Uemura, Yasuo Uchiyama, Takeshi Todo, Shun-ichi Takeda and Ryosuke Takahashi
  • Organizer: 17th international congress of Parkinson's disease and movement disorders
  • Place of Presentation: Sydney
• [Presentation] NF-κB経路を介したOptineurinのノックダウンによる神経細胞死 (2013)
  • Author(s): 秋月真由美、山下博文、植村健吾、丸山博文、川上秀史、伊東秀文、高橋良輔
  • Organizer: Neuro2013
  • Place of Presentation: 京都 国際会議場
• [Presentation] α-Synucleinの発現量の調節を標的とするパーキンソン病治療薬剤のスクリーニング (2013)
  • Author(s): 浅野剛史、山門穂高、高橋良輔
  • Organizer: Neuro2013
  • Place of Presentation: 京都 国際会議場
• [Presentation] 疾患特異的iPS細胞モデルによる遺伝性パーキンソン病の研究 (2013)
  • Author(s): 小芝泰、森実飛鳥、山門穂高、菊地哲広、土井大輔、西村周泰、皆川栄子、江川斉宏、近藤孝之、井上治久、髙橋淳、髙橋良輔
  • Organizer: Neuro2013
  • Place of Presentation: 京都 国際会議場
• [Presentation] Therapeutic approach to Parkinson's disease by modifying α-synuclein expression. (2013)
  • Author(s): Yamakado H, Asano T, Takahashi, R
  • Organizer: The MDS 17th International Congress of Parkinson's Disease and Movement Disorders
  • Place of Presentation: Sydney
• [Presentation] メダカを用いたGBA変異とパーキンソン病の関連性の解析 (2013)
  • Author(s): 上村紀仁、石川智子、木下政人、小池正人、松井秀彰、山門穂高、植村健吾、内山安男、藤堂剛、武田俊一、高橋良輔
  • Organizer: 日本神経学会
  • Place of Presentation: 東京 東京国際フォーラム
• [Presentation] ATP13A2変異メダカの解析 (2013)
  • Author(s): 樽野陽亮、谷口善仁、松井秀彰、上村紀仁、山門穂高、高橋良輔
  • Organizer: 第５４回日本神経学会学術大会
  • Place of Presentation: 東京 東京国際フォーラム
• [Presentation] 疾患特異的iPS細胞モデルによる遺伝性パーキンソン病の研究 (2013)
  • Author(s): 小芝泰、森実飛鳥、山門穂高、菊地哲広、皆川栄子、土井大輔、西村周泰、江川斉宏、井上治久、髙橋淳、髙橋良輔
  • Organizer: 第54回日本神経学会学術総会
  • Place of Presentation: 東京 東京国際フォーラム
• [Presentation] Regulation of the PINK1 signaling by a mitochondrial protein PGAM5. (2012)
  • Author(s): Sawada T
  • Organizer: Neuroscience 2012
  • Place of Presentation: New Orleans, U.S.A.
  • Year and Date: 2012-10-15
• [Presentation] BACテクノロジーを用いたパーキンソン病の治療薬候補の探索 (2012)
  • Author(s): 浅野剛史
  • Organizer: 第6回パーキンソン病・運動障害疾患コングレス
  • Place of Presentation: 京都ホテルオークラ
  • Year and Date: 2012-10-13
• [Presentation] トランスグルタミナーゼ２によるSOD1オリゴマー形成を標的にしたALSモデルマウスの治療解析 (2012)
  • Author(s): 大野美樹
  • Organizer: 第35回日本神経科学大会
  • Place of Presentation: 名古屋国際会議場
  • Year and Date: 2012-09-18
• [Presentation] 培養細胞モデルを用いたALSにおけるTDP-43の分子的機構の解析 (2012)
  • Author(s): 内田 司
  • Organizer: 第35回日本神経科学大会
  • Place of Presentation: 名古屋国際会議場
  • Year and Date: 2012-09-18
• [Presentation] α-synuclein BAC transgenic mice as a model for Parkinson’s disease manifested decreased anxiety-like behavior. (2012)
  • Author(s): Yamakado H,
  • Organizer: The MDS 17th International Congress of Parkinson's Disease and Movement Disorders
  • Place of Presentation: Dablin, Ireland
  • Year and Date: 2012-06-21
• [Presentation] ALS病態におけるtransglutaminase2を介したSOD1オリゴマー形成の関与 (2012)
  • Author(s): 大野美樹
  • Organizer: 第53回日本神経学会学術大会
  • Place of Presentation: 東京国際フォーラム
  • Year and Date: 2012-05-22
• [Presentation] テトラサイクリン誘導性にTDP-43を発現するヒト神経由来細胞の樹立とその応用 (2012)
  • Author(s): 内田 司
  • Organizer: 第53回日本神経学会学術大会
  • Place of Presentation: 東京国際フォーラム
  • Year and Date: 2012-05-22
• [Presentation] CONGNITIVE AND PHYCHIATRIC FEATURES IN ANIMAL PD MODELS. (2012)
  • Author(s): Takahashi R
  • Organizer: 8th International Congress On Mental Dysfunction Other Non-Motor Features In Parkinson’s Diease and Related Disorders
  • Place of Presentation: Berlin, Germany
  • Year and Date: 2012-05-04
  • Invited
• [Presentation] PINK1 and Parkin regulate the mitochondrial transport machinery (2011)
  • Author(s): 澤田知世
  • Organizer: 第34回日本分子生物学会
  • Place of Presentation: パシフィコ横浜
  • Year and Date: 2011-12-14
• [Presentation] spinal motor neuron specific 2Bs proteasome subunit conditional knockout mice offer cell death of motor neurons with similar pathological ALS peculiarities in human patients (2011)
  • Author(s): 田代善崇, ら
  • Organizer: 第34回日本分子生物学会年会
  • Place of Presentation: パシフィコ横浜
  • Year and Date: 2011-12-13
• [Presentation] Variant forms of human chromogranin B fail to interact with mutant SOD1 (2011)
  • Author(s): 太田康之, ら
  • Organizer: Annual Meeting of Society for Neuroscience 2011
  • Place of Presentation: ワシントンDC(米国)
  • Year and Date: 2011-11-12
• [Presentation] HECT型ユビキチンリガーゼHuwel/MuleはPlNK1安定性を制御する (2011)
  • Author(s): 澤田知世
  • Organizer: Neuroscience2011
  • Place of Presentation: パシフィコ横浜
  • Year and Date: 2011-09-17
• [Presentation] アルファ・シヌクレインBACトランスジェニックマウスにおける不安の低下とパーキンソン病非運動症状について (2011)
  • Author(s): 山門穂高
  • Organizer: Neuroscience2011
  • Place of Presentation: パシフィコ横浜
  • Year and Date: 2011-09-15
• [Presentation] ALSモデルマウスにおけるトランスグルタミナーゼ2の誘導 (2011)
  • Author(s): 大野美樹, ら
  • Organizer: 第34回日本神経科学大会
  • Place of Presentation: パシフィコ横浜
  • Year and Date: 2011-09-15
• [Presentation] 神経変性疾患モデル作製のための26Sプロテアソームコンディショナルノックアウトマウスの解析 (2011)
  • Author(s): 田代善崇, ら
  • Organizer: 第34回日本神経科学大会
  • Place of Presentation: パシフィコ横浜
  • Year and Date: 2011-09-07
• [Presentation] Molecular mechanisms underlying Parkin/PINK1-related Parkinson's disease (2011)
  • Author(s): Ryosuke Takahashi
  • Organizer: International Symposium "Parkinson Disease and Mitophagy"
  • Place of Presentation: 東京歯科大学講堂(招待講演)
  • Year and Date: 2011-06-11
• [Presentation] 神経疾患に対する細胞治療の開発-現状と展望 (2011)
  • Author(s): 高橋良輔
  • Organizer: 第52回日本神経学会学術大会
  • Place of Presentation: 名古屋国際会議場(招待講演)
  • Year and Date: 2011-05-20
• [Presentation] HECT型ユビキチンリガーゼMule/HuwelによるPINK1安定性の調節 (2011)
  • Author(s): 澤田知世
  • Organizer: 第52回日本神経学会学術大会
  • Place of Presentation: 名古屋国際会議場
  • Year and Date: 2011-05-20
• [Presentation] 神経変性疾患における抗体療法「筋萎縮性側索硬化症の抗体療法」 (2011)
  • Author(s): 漆谷真
  • Organizer: 第52回日本神経学会学術集会
  • Place of Presentation: 名古屋国際会議場
  • Year and Date: 2011-05-20
• [Presentation] 神経変性疾患モデル作製のための26Sプロテアソームコンディショナルノックアウトマウスの確立と解析 (2011)
  • Author(s): 田代善崇, ら
  • Organizer: 第52回日本神経学会学術集会
  • Place of Presentation: 名古屋国際会議場
  • Year and Date: 2011-05-19
• [Presentation] Molecular targeting strategies against misfolded proteins in ALS (2011)
  • Author(s): 漆谷真
  • Organizer: 7th ALS Research Forum, ALS Society of Canada
  • Place of Presentation: トロント(カナダ)
  • Year and Date: 2011-05-01
• [Book] MOOK別冊「細胞死研究の今－疾患との関わり、創薬に向けてのアプローチ」 (2013)
  • Author(s): 守村 敏史，高橋 良輔，漆谷 真
  • Total Pages: 44
  • Publisher: メディカルドゥ
• [Book] Medical Science Digest 39巻 (2013)
  • Author(s): 高橋 良輔
  • Total Pages: 100
  • Publisher: ニューサイエンス社
• [Book] 脳21 16巻（１） (2013)
  • Author(s): 澤田知世、今居 譲、高橋良輔
  • Total Pages: 6
  • Publisher: 金芳堂
• [Book] Annual Review神経 2013 (2013)
  • Author(s): 糸数 隆秀、高橋 良輔、山下 俊英
  • Total Pages: 278
  • Publisher: 中外医学社
• [Book] すべてがわかる筋萎縮性側索硬化症・運動ニューロン疾患 (2013)
  • Author(s): 漆谷 真
  • Total Pages: 384
  • Publisher: 中山書店
• [Book] 臨床神経学 (2013)
  • Author(s): Takahashi R.
  • Total Pages: 500
  • Publisher: JSN treatment guideline for Parkinson's disease: its editorial policy and problems to be addressed.
• [Book] Nihon Naika Gakkai Zasshi. (2013)
  • Author(s): Kondo T, Inoue H, Takahashi R.
  • Total Pages: 300
  • Publisher: New and future treatments for neurological disorders--knowledge essential to daily clinics and future prospects. Topics: 15. Therapeutic application of iPS cells.
• [Book] 医学のあゆみ (2013)
  • Author(s): 小芝泰、髙橋良輔
  • Total Pages: 100
  • Publisher: 孤発性パーキンソン病におけるiPS細胞研究
• [Book] 脳神経科学イラストレイテッド 改訂第3版 「ALSなど運動ニューロン病」の章 (2013)
  • Author(s): 山下博史、高橋良輔
  • Total Pages: 395
  • Publisher: 羊土社
• [Book] Amyotrophic Lateral Sclerosis: Symptoms, Treatment and Prognosis. (2013)
  • Author(s): Urushitani M and Morimura T
  • Total Pages: 23
  • Publisher: Nova Science Publishers, Inc.
• [Remarks] 文部省 科学j研究費補助金 新学術領域研究「脳内環境」
  • URL: http://www.neurol.med.kyoto-u.ac.jp/brainenvironment/
• [Remarks] 脳内環境
  • URL: http://www.neurol.med.kyoto-u.ac.jp/brainenvironment/J/index.html
• [Remarks] 滋賀医科大学 神経難病研究機構 分子神経科学研究センター 神経難病治療学分野
  • URL: http://www.shiga-med.ac.jp/~uru/index.html
• [Remarks] 脳内環境
  • URL: http://www.neurol.med.kyoto-u.ac.jp/brainenvironment/#
• [Patent(Industrial Property Rights)] TDP-43の凝集体が蓄積する疾患を治療 (2013)
  • Inventor(s): 漆谷真、藤原範子、北原 亮、伊東秀文
  • Industrial Property Rights Holder: 滋賀医科大学、兵庫医科大学、立命館大学
  • Industrial Property Rights Type: 特許
  • Filing Date: 2013-03-08