Establishment and pathological analyses of transgenic marmoset models of polyglutamine diseases

• Project/Area Number: 26670446
• Research Category: Grant-in-Aid for Challenging Exploratory Research
• Allocation Type: Multi-year Fund
• Research Field: Neurology
• Research Institution: National Center of Neurology and Psychiatry
• Principal Investigator: NAGAI Yoshitaka 国立研究開発法人国立精神・神経医療研究センター, 神経研究所疾病研究第四部, 客員研究員 (60335354)
• Co-Investigator(Renkei-kenkyūsha): SEKI Kazuhiko 国立研究開発法人国立精神・神経医療研究センター, 神経研究所モデル動物研究部, 部長 (00226630) ; TOMIOKA Ikuo 信州大学, 先鋭領域融合研究群バイオメディカル研究所, 助教 (30528196)
• Project Period (FY): 2014-04-01 – 2016-03-31
• Project Status: Completed (Fiscal Year 2015)
• Budget Amount: ¥3,640,000 (Direct Cost: ¥2,800,000、Indirect Cost: ¥840,000); Fiscal Year 2015: ¥1,820,000 (Direct Cost: ¥1,400,000、Indirect Cost: ¥420,000); Fiscal Year 2014: ¥1,820,000 (Direct Cost: ¥1,400,000、Indirect Cost: ¥420,000)
• Keywords: 神経科学 / 脳神経疾患 / ポリグルタミン病 / 疾患モデル / 霊長類 / マーモセット / 遺伝子 / 神経変性疾患

Outline of Final Research Achievements

In this study, aiming to establish non-human primate models of neurodegenerative diseases, we performed pathological analyses of transgenic marmoset models of Machado-Joseph disease (MJD)/spinocerebellar ataxia type 3, one of the polyglutamine diseases. We found that three of seven SCA3 transgenic marmosets, which expressed the mutant ataxin-3 transgene at higher levels, gradually developed progressive neurological symptoms such as motor impairment and muscle weakness. Neuropathological examinations revealed significant neurodegeneration accompanied with polyglutamine inclusions in their brains. We therefore conclude that our SCA3 transgenic marmosets faithfully recapitulate human SCA3 patients’ features, and are useful models for elucidating the pathophysiological mechanisms of and developing clinically-applicable therapies for neurodegenerative diseases.

Research Products

• [Journal Article] Molecular Basis of Orb2 Amyloidogenesis and Blockade of Memory Consolidation (2016)
  • Author(s): Hervas R, Li L, Majumdar A, Fernandez-Ramirez Mdel C, Unruh JR, Slaughter BD, Galera-Prat A, Santana E, Suzuki M, Nagai Y, Bruix M, Casas-Tinto S, Menendez M, Laurents DV, Si K, Carrio-Vazquez M.
  • Journal Title: PLoS Biol Volume: 26 Issue: 1 Pages: e1002361-e1002361
  • DOI: 10.1371/journal.pbio.1002361
  • Peer Reviewed / Open Access / Int'l Joint Research
• [Journal Article] Guidelines for the use and interpretation of assays for monitoring autophagy (3rd edition) (2016)
  • Author(s): Klionsky DJ., Abdelmohsen K., Abe A., Abedin MJ.,Inomata M., et al.
  • Journal Title: Autophagy Volume: 12(1) Issue: 1 Pages: 1-222
  • DOI: 10.1080/15548627.2015.1100356
  • Peer Reviewed / Open Access / Int'l Joint Research / Acknowledgement Compliant
• [Journal Article] エクソソームによる生体内のタンパク質恒常性の維持 (2016)
  • Author(s): 武内敏秀、永井義隆
  • Journal Title: 細胞 Volume: 48 Pages: 5-8
• [Journal Article] パ－キンソン病の動物モデル－最近の話題 (2016)
  • Author(s): 高橋良輔、佐藤栄人、永井義隆、松井秀彰、望月秀樹
  • Journal Title: Frontiers in Parkinson Disease Volume: 9 Pages: 5-13
• [Journal Article] Synchrotron FTIR Micro-Spectroscopy for Structural Analysis of Lewy Bodies in the Brain of Parkinson’s Disease Patients (2015)
  • Author(s): K. Araki, N. Yagi, Y. Ikemoto, S. Choong, H. Hayakawa, G. Beck, H. Sumi, H. Fujimura, T. Moriwaki, Y. Nagai, Y. Goto and H. Mochizuki
  • Journal Title: Scientific Reports Volume: 5 Issue: 1 Pages: 17625-17625
  • DOI: 10.1038/srep17625
  • NAID: 120006557628
  • Peer Reviewed / Open Access / Int'l Joint Research
• [Journal Article] Abnormalities in synaptic dynamics during development in a mouse model of spinocerebellar ataxia type 1. (2015)
  • Author(s): Hatanaka Y., Watase K., Wada K., Nagai Y.
  • Journal Title: Sci. Rep. Volume: 5 Issue: 1 Pages: 16102-16102
  • DOI: 10.1038/srep16102
  • Peer Reviewed / Open Access
• [Journal Article] Glucocerebrosidase deficiency accelerates the accumulation of proteinase K-resistant α-synuclein and aggravates neurodegeneration in a Drosophila model of Parkinson's disease. (2015)
  • Author(s): Suzuki M, Fujikake N, Takeuchi T, Kohyama-Koganeya A, Nakajima K, Hirabayashi Y, Wada K, Nagai Y.
  • Journal Title: Hum Mol Genet. 2015 Dec 1;24(23):6675-86 Volume: 24 Issue: 23 Pages: 6675-6686
  • DOI: 10.1093/hmg/ddv372
  • Peer Reviewed
• [Journal Article] Normalization of overexpressed alpha-synuclein causing Parkinson’s disease by a moderate gene silencing with RNA interference. (2015)
  • Author(s): Takahashi M., Suzuki M., Fukuoka M., Fujikake N., Watanabe S., Murata M., Wada K., Nagai Y., and *Hohjoh H.
  • Journal Title: Mol Ther Nucleic Acids Volume: 4 Pages: e241-e241
  • DOI: 10.1038/mtna.2015.14
  • Peer Reviewed / Open Access / Acknowledgement Compliant
• [Journal Article] Intercellular chaperone transmission via exosomes contributes to maintenance of protein homeostasis at the organismal level. (2015)
  • Author(s): Takeuchi T, Suzuki M, Fujikake N, Popiel HA, Kikuchi H, Futaki S, Wada K, Nagai Y
  • Journal Title: Proc Natl Acad Sci USA Volume: in press Issue: 19
  • DOI: 10.1073/pnas.1412651112
  • Peer Reviewed / Acknowledgement Compliant
• [Journal Article] Normalization of overexpressed alpha-synuclein causing Parkinson's disease by a moderate gene silencing with RNA interference (2015)
  • Author(s): Takahashi M, Suzuki M, Fukuoka M, Fujikake N, Watanabe S, Murata M, Wada K, Nagai Y, Hohjoh H
  • Journal Title: Mol Ther Nucleic Acids Volume: in press
  • Peer Reviewed
• [Journal Article] p62 plays a protective role in the autophagic degradation of polyglutamine protein oligomers in polyglutamine disease model flies. (2015)
  • Author(s): Saitoh Y, Fujikake N, Okamoto Y, Popiel HA, Hatanaka Y, Ueyama M, Suzuki M, Gaumer S, Murata M, Wada K, Nagai Y.
  • Journal Title: J Biol Chem Volume: 290 Issue: 3 Pages: 1442-53
  • DOI: 10.1074/jbc.m114.590281
  • Peer Reviewed / Open Access
• [Journal Article] その他のFTLD―異常蛋白の視点から (2015)
  • Author(s): 上山盛夫、藤掛伸宏、永井義隆
  • Journal Title: Clinical Neuroscience Volume: 33 Pages: 312-314
• [Journal Article] VPS35 dysfunction impairs lysosomal degradation of α-synuclein and exacerbates neurotoxicity in a Drosophila model of Parkinson's disease. (2014)
  • Author(s): Miura E, Hasegawa T, Konno M, Suzuki M, Sugeno N, Fujikake N, Geisler S, Tabuchi M, Oshima R, Kikuchi A, Baba T, Wada K, Nagai Y, Takeda A, Aoki M.
  • Journal Title: Neurobio Dis Volume: 71 Pages: 1-13
  • DOI: 10.1016/j.nbd.2014.07.014
  • Peer Reviewed
• [Journal Article] Identification of ter94, Drosophila VCP, as a strong modulator of motor neuron degeneration induced by knockdown of Caz, Drosophila FUS (2014)
  • 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
  • Journal Title: Hum. Mol. Genet Volume: 23(13) Issue: 13 Pages: 3467-3480
  • DOI: 10.1093/hmg/ddu055
  • Peer Reviewed
• [Journal Article] Peptide-based therapeutic approaches for treatment of the polyglutamine diseases (2014)
  • Author(s): Takeuchi T., Popiel H.A., Futaki S., Wada K., Nagai Y
  • Journal Title: Curr. Med. Chem Volume: (in press) Issue: 23 Pages: 2575-2582
  • DOI: 10.2174/0929867321666140217124038
  • Peer Reviewed
• [Journal Article] ポリグルタミン病における神経変性 (2014)
  • Author(s): 永井義隆
  • Journal Title: BRAIN MEDICAL Volume: 26 Pages: 225-229
• [Presentation] 神経変性疾患におけるRNA chaperoneの働き：RNA quality controlの観点から (2015)
  • Author(s): 石黒太郎、横田隆徳、永井義隆、石川欽也
  • Organizer: 第10回 臨床ストレス応答学会
  • Place of Presentation: 東京
  • Year and Date: 2015-11-06
  • Invited
• [Presentation] 分子シャペロンのエクソソームを介した細胞間伝播による細胞非自律的なタンパク質の恒常性維持機構 (2015)
  • Author(s): 武内敏秀、鈴木マリ、藤掛伸宏、ポピエル明子、菊地寿枝、二木史朗、和田圭司、永井義隆
  • Organizer: 第10回 臨床ストレス応答学会
  • Place of Presentation: 東京
  • Year and Date: 2015-11-06
• [Presentation] Establishment of a novel animal model of ALS/FTD expressing GGGGCC repeat RNA in Drosophila (2015)
  • Author(s): Ueyama M., Ishiguro T., Fujikake N., Konno T., Koyama A., Onodera O., Ishikawa K., Wada K., Nagai Y.
  • Organizer: 10th Brain Research Conference
  • Place of Presentation: Chicago, IL, USA
  • Year and Date: 2015-10-15
  • Int'l Joint Research
• [Presentation] The SCA31-linked UGGAAexp repeat forms RNA foci and is translated into aggregating pentapeptide-repeat proteins in the SCA31 patient brain and Drosophila model. (2015)
  • Author(s): Ishiguro T., Sato N., Fujikake N., Ueyama M., Yokota T., Mizusawa H., Wada K., Nagai Y., Ishikawa K.
  • Organizer: 10th Brain Research Conference
  • Place of Presentation: Chicago, IL, USA
  • Year and Date: 2015-10-15
  • Int'l Joint Research
• [Presentation] 分子シャペロンはエクソソーム細胞間伝播により細胞非自律的な治療効果を発揮する (2015)
  • Author(s): 武内敏秀、永井義隆
  • Organizer: 第34回 日本認知症学会学術集会
  • Place of Presentation: 青森
  • Year and Date: 2015-10-02
  • Invited
• [Presentation] 神経変性疾患モデルショウジョウバエの神経変性は過栄養摂取により増悪する (2015)
  • Author(s): 永井義隆、鈴木マリ、Anne-Marie Neumann、藤掛伸宏、和田圭司
  • Organizer: 第34回 日本認知症学会学術集会
  • Place of Presentation: 青森
  • Year and Date: 2015-10-02
• [Presentation] G4C2リピートRNAを発現する新規ALS/FTDモデルショウジョウバエの樹立 (2015)
  • Author(s): 上山盛夫、石黒太郎、藤掛伸宏、今野卓哉、小山哲秀、小野寺理、石川欽也、和田圭司、永井義隆
  • Organizer: 第87回 日本遺伝学会大会
  • Place of Presentation: 仙台
  • Year and Date: 2015-09-24
  • Invited
• [Presentation] 分子シャペロンはエクソソームを介した細胞間伝播により個体レベルのプロテオスターシス維持に寄与している (2015)
  • Author(s): 武内敏秀、鈴木マリ、藤掛伸宏、ポピエル明子、菊地寿枝、二木史朗、和田圭司、永井義隆
  • Organizer: 第7回 日本RNAi研究会/第2回 日本細胞外小胞学会
  • Place of Presentation: 広島
  • Year and Date: 2015-08-26
• [Presentation] 異常伸長UGGAA リピートRNAはショウジョウバエにおいて神経毒性を引き起こす (2015)
  • Author(s): 石黒太郎、藤掛伸宏、佐藤望、上山盛夫、横田隆徳、和田圭司、水澤英洋、永井義隆、石川 欽也
  • Organizer: 第38回 日本神経科学会
  • Place of Presentation: 神戸
  • Year and Date: 2015-07-28
• [Presentation] Establishment of a novel animal model of ALS and FTD expressing GGGGCC repeat RNA in Drosophila (2015)
  • Author(s): 上山盛夫、石黒太郎、藤掛伸宏、今野卓哉、小山哲秀、小野寺理、石川欽也、和田圭司、永井義隆
  • Organizer: 第38回 日本神経科学会
  • Place of Presentation: 神戸
  • Year and Date: 2015-07-28
• [Presentation] Intercellular chaperone transmission via exosomes contributes to non-cell autonomous therapeutic effects on polyglutamine disease models (2015)
  • Author(s): 武内敏秀、鈴木マリ、藤掛伸宏、ポピエル明子、菊地寿枝、二木史朗、和田圭司、永井義隆
  • Organizer: 第38回 日本神経科学会
  • Place of Presentation: 神戸
  • Year and Date: 2015-07-28
• [Presentation] ポリグルタミン病トランスジェニックマーモセットの樹立 (2015)
  • Author(s): 永井義隆、富岡郁夫、石橋英俊、本橋秀之、高山修、齊藤祐子、皆川栄子、ポピエル明子、プエンテスサンドラ、山本和弘、野口悟、西野一三、和田圭司、高坂新一、関和彦
  • Organizer: 第56回 日本神経学会学術大会
  • Place of Presentation: 新潟
  • Year and Date: 2015-05-20
• [Presentation] Effect of dietary conditions on Drosophila models of neurodegenerative diseases (2015)
  • Author(s): 鈴木マリ、Anne-Marie Neumann、斉藤勇二、藤掛伸宏、和田圭司、永井義隆
  • Organizer: 第56回 日本神経学会学術大会
  • Place of Presentation: 新潟
  • Year and Date: 2015-05-20
• [Presentation] p62 plays a protective role in neurodegenerative diseases model flies (2015)
  • Author(s): 斉藤勇二、藤掛伸宏、岡本佑馬、和田圭司、永井義隆
  • Organizer: 第56回 日本神経学会学術大会
  • Place of Presentation: 新潟
  • Year and Date: 2015-05-20
• [Presentation] SCA31-linked UGGAAexp RNA causes progressive neurodegeneration in Drosophila. (2015)
  • Author(s): 石黒太郎、藤掛伸宏、佐藤望、上山盛夫、横田隆徳、和田圭司、水澤英洋、永井義隆、石川欽也
  • Organizer: 第56回 日本神経学会学術大会
  • Place of Presentation: 新潟
  • Year and Date: 2015-05-20
• [Presentation] Identification of genes modulating TDP-43 by Drosophila ALS model (2015)
  • Author(s): 東裕美子、徳田隆彦、山本格、京谷茜、櫛村由紀恵、吉田誠克、水田依久子、水野俊樹、中川正法、藤掛伸宏、上山盛夫、永井義隆、吉田英樹、山口政光
  • Organizer: 第56回 日本神経学会学術大会
  • Place of Presentation: 新潟
  • Year and Date: 2015-05-20
• [Presentation] Exome association study and 2nd SNP-GWAS of Parkinson's disease (2015)
  • Author(s): 佐竹渉、安藤祐子、鈴木マリ、富山弘幸、永井義隆、村山繁雄、望月秀樹、中島健二、小幡文弥、長谷川一子、武田篤、和田圭司、辻省次、山本光利、村田美穂、服部信孝、戸田達史
  • Organizer: 第56回 日本神経学会学術大会
  • Place of Presentation: 新潟
  • Year and Date: 2015-05-20
• [Presentation] Toxic protein conformational transition and aggregation as a therapeutic target for the polyglutamine neurodegenerative diseases. (2015)
  • Author(s): Nagai Y.
  • Organizer: Cajal Institute seminar
  • Place of Presentation: Madrid, Spain
  • Year and Date: 2015-04-20
  • Int'l Joint Research / Invited
• [Presentation] Non-cell autonomous therapeutic effects of molecular chaperones on polyglutamine disease models via its exosome-mediated intercellular transmission. (2015)
  • Author(s): Nagai Y., Takeuchi T., Wada K.
  • Organizer: IWBBIO 2015
  • Place of Presentation: Granada, Spain
  • Year and Date: 2015-04-15
  • Int'l Joint Research / Invited
• [Presentation] Dysfunction of microtubule- dependent transport triggers oligomerization and cytoplasmic accumulation of TDP-43, leading to neurodegeneration. (2014)
  • Author(s): Nagai Y, Fujikake N, Kimura N, Saitoh Y, Hatanaka Y, Onodera O, Wada K
  • Organizer: Cold Spring Harbor Laboratory 2014 Neurodegenerative Diseases meeting: Biology & Therapeutics
  • Place of Presentation: Cold Spring Harbor, NY, USA
  • Year and Date: 2014-12-03 – 2014-12-06
• [Presentation] p62 plays a protective role in the autophagic degradation of polyglutamine protein oligomers in polyglutamine disease model flies. (2014)
  • Author(s): Saitoh Y, Fujikake N, Okamoto Y, Wada K, Nagai Y
  • Organizer: Cold Spring Harbor Laboratory 2014 Neurodegenerative Diseases meeting: Biology & Therapeutics
  • Place of Presentation: Cold Spring Harbor, NY, USA
  • Year and Date: 2014-12-03 – 2014-12-06
• [Presentation] Expanded UGGAA repeat RNA associated with SCA31 causes progressive neurodegeneration in Drosophila. (2014)
  • Author(s): Ishiguro T, Fujikake N, Sato N, Ueyama, M, Mizusawa H, Wada K, Nagai Y, Ishikawa K
  • Organizer: Cold Spring Harbor Laboratory 2014 Neurodegenerative Diseases meeting: Biology & Therapeutics
  • Place of Presentation: Cold Spring Harbor, NY, USA
  • Year and Date: 2014-12-03 – 2014-12-06
• [Presentation] 微小管依存的TDP-43輸送の障害はオリゴマー形成を促進し、神経変性を惹き起こす (2014)
  • Author(s): 永井義隆、藤掛伸宏、木村展之、長野清一、斉藤勇二、小野寺理、和田圭司
  • Organizer: 第33回日本認知症学会学術集会
  • Place of Presentation: 横浜
  • Year and Date: 2014-11-29 – 2014-12-01
• [Presentation] Misfolding and aggregation of the polyglutamine protein and its suppression by intercellular transmission of molecular chaperone. (2014)
  • Author(s): Nagai Y
  • Organizer: Hungary-Japanese Symp on Mechanism and regulation of aberrant protein aggregation
  • Place of Presentation: Osaka, Japan
  • Year and Date: 2014-11-17 – 2014-11-21
  • Invited
• [Presentation] 分子シャペロンによるポリグルタミンタンパク質の品質管理機構 (2014)
  • Author(s): 永井義隆
  • Organizer: 奈良先端大学院大学セミナー
  • Place of Presentation: 奈良
  • Year and Date: 2014-11-12
  • Invited
• [Presentation] In vivoイメージングによって見えてきた神経変性疾患の発達障害としての側面 (2014)
  • Author(s): 畑中悠佑、渡瀬啓、和田圭司、永井義隆
  • Organizer: 第12回神経科学研究会
  • Place of Presentation: 東京
  • Year and Date: 2014-10-25
• [Presentation] TDP-43のオリゴマー化・神経変性メカニズムの解明 (2014)
  • Author(s): 永井義隆
  • Organizer: 平成26年度厚労省「筋萎縮性側索硬化症の新規治療法・病態解明研究班」ワークショップ
  • Place of Presentation: 東京
  • Year and Date: 2014-09-26
  • Invited
• [Presentation] GGGGCCリピートRNAを発現する新規ALSモデルショウジョウバエの樹立と病態解析 (2014)
  • Author(s): 上山盛夫、石黒太郎、藤掛伸宏、今野卓哉、小山哲秀、小野寺理、和田圭司、永井義隆
  • Organizer: 第86回日本遺伝学会大会
  • Place of Presentation: 長浜
  • Year and Date: 2014-09-17 – 2014-09-19
• [Presentation] 神経変性疾患モデルショウジョウバエの神経変性は過栄養摂取により増悪する (2014)
  • Author(s): 鈴木マリ、Anne-Marie Neumann、斉藤勇二、藤掛伸宏、和田圭司、永井義隆
  • Organizer: 第37回日本神経科学会
  • Place of Presentation: 横浜
  • Year and Date: 2014-09-11 – 2014-09-13
• [Presentation] p62/SQSTM1はポリグルタミン病モデルショウジョウバエにおいて、ポリグルタミン蛋白質凝集体をオートファジー分解系で除去することで保護的役割を果たす (2014)
  • Author(s): 斉藤勇二、藤掛伸宏、岡本佑馬、和田圭司、永井義隆
  • Organizer: 第37回日本神経科学会
  • Place of Presentation: 横浜
  • Year and Date: 2014-09-11 – 2014-09-13
• [Presentation] Investigating the Role of Ubiquilin 2 (UBQLN2) in the Pathomechanism of ALS/FTD. (2014)
  • Author(s): Minakawa EN, Sharkey LM, Chen K-C, Thayer M, Lyons J, Ivanova M, Wada K, Nagai Y, Paulson HL
  • Organizer: 第37回日本神経科学会
  • Place of Presentation: 横浜
  • Year and Date: 2014-09-11 – 2014-09-13
• [Presentation] 異常伸長UGGAAリピートRNAはショウジョウバエにおいて神経毒性を引き起こす (2014)
  • Author(s): 石黒太郎、藤掛伸宏、佐藤望、水澤英洋、和田圭司、永井義隆、石川欽也
  • Organizer: 第37回日本神経科学会
  • Place of Presentation: 横浜
  • Year and Date: 2014-09-11 – 2014-09-13
• [Presentation] ショウジョウバエモデルを用いた神経変性疾患の病態解明・治療法開発～ALSモデルを中心に～ (2014)
  • Author(s): 永井義隆
  • Organizer: 第32回 Neuroscience Seminar Tokushima
  • Place of Presentation: 徳島
  • Year and Date: 2014-09-01
  • Invited
• [Presentation] リグルタミン蛋白質の凝集・細胞毒性獲得メカニズム (2014)
  • Author(s): 永井義隆
  • Organizer: タウ研究ミーティング
  • Place of Presentation: 東京
  • Year and Date: 2014-08-01
  • Invited
• [Presentation] Investigating the Role of Ubiquilin 2 (UBQLN2) in the Pathomechanism of ALS/FTD. (2014)
  • Author(s): Minakawa EN, Sharkey LM, Chen K-C, Thayer M, Lyons J, Ivanova M, Wada K, Nagai Y, Paulson HL
  • Organizer: Gordon Research Conference on Neurobiology of Brain Disorders
  • Place of Presentation: Girona, Spain
  • Year and Date: 2014-07-27 – 2014-08-01
• [Presentation] 神経変性疾患に対する蛋白質ミスフォールディング・凝集を標的とした治療戦略－ポリグルタミン病をモデルとして (2014)
  • Author(s): 永井義隆
  • Organizer: 第9回青森神経科学談話会
  • Place of Presentation: 青森
  • Year and Date: 2014-06-14
  • Invited
• [Presentation] 蛋白質のミスフォールディング・凝集を標的とした神経変性疾患の治療戦略－ポリグルタミン病をモデルとして (2014)
  • Author(s): 永井義隆
  • Organizer: 京都大学薬学系研究科大学院講義
  • Place of Presentation: 京都
  • Year and Date: 2014-05-27
  • Invited
• [Presentation] ２光子in vivoイメージング解析によるSCA1マウスにおけるシナプス異常の解明 (2014)
  • Author(s): 永井義隆、畑中悠佑、和田圭司
  • Organizer: 第55回日本神経学会学術大会
  • Place of Presentation: 福岡
  • Year and Date: 2014-05-21 – 2014-05-24
• [Presentation] DCTN1依存的輸送の障害はTDP-43のオリゴマー形成を促進する (2014)
  • Author(s): 藤掛伸宏、木村展之、長野清一、斉藤勇二、横関明男、小野寺理、和田圭司、永井義隆
  • Organizer: 第55回日本神経学会学術大会
  • Place of Presentation: 福岡
  • Year and Date: 2014-05-21 – 2014-05-24
• [Presentation] ポリグルタミン病モデルショウジョウバエの神経変性は過栄養摂取により増悪する (2014)
  • Author(s): 鈴木マリ、藤掛伸宏、和田圭司、永井義隆
  • Organizer: 第55回日本神経学会学術大会
  • Place of Presentation: 福岡
  • Year and Date: 2014-05-21 – 2014-05-24
• [Presentation] ポリグルタミン病モデルにおいてp62はオートファジー分解系を介して保護的に作用する (2014)
  • Author(s): 斉藤勇二、藤掛伸宏、岡本佑馬、和田圭司、永井義隆
  • Organizer: 第55回日本神経学会学術大会
  • Place of Presentation: 福岡
  • Year and Date: 2014-05-21 – 2014-05-24
• [Presentation] Investigating the Role of Ubiquilin 2 (UBQLN2) in the Pathomechanism of ALS/FTD. (2014)
  • Author(s): Minakawa EN, Sharkey LM, Chen K-C, Thayer M, Lyons J, Ivanova M, Wada K, Nagai Y, Paulson HL
  • Organizer: 第55回日本神経学会学術大会
  • Place of Presentation: 福岡
  • Year and Date: 2014-05-21 – 2014-05-24
• [Presentation] FUS-ALSモデルショウジョウバエの表現型を修飾する因子の探索 (2014)
  • Author(s): 東裕美子、徳田隆彦、京谷茜、吉田誠克、水田依久子、水野俊樹、中川正法、藤掛伸宏、上山盛夫、永井義隆、山口政光
  • Organizer: 第55回日本神経学会学術大会
  • Place of Presentation: 福岡
  • Year and Date: 2014-05-21 – 2014-05-24
• [Presentation] VPS35障害はカテプシンD活性低下を介しαシヌクレイン蓄積・神経変性を惹起する (2014)
  • Author(s): 三浦永美子、長谷川隆文、今野昌俊、菅野直人、大嶋龍児、菊池昭夫、鈴木マリ、永井義隆、武田篤、青木正志
  • Organizer: 第55回日本神経学会学術大会
  • Place of Presentation: 福岡
  • Year and Date: 2014-05-21 – 2014-05-24
• [Presentation] SCA31 (UGGAA)nリピートはショウジョウバエで進行性神経障害を引き起こす (2014)
  • Author(s): 石黒太郎、石川欽也、藤掛伸宏、上山盛夫、永井義隆、和田圭司、水澤英洋
  • Organizer: 第55回日本神経学会学術大会
  • Place of Presentation: 福岡
  • Year and Date: 2014-05-21 – 2014-05-24
• [Book] HSF1 activation by small chemical compounds for the treatment of neurodegenerative diseases In Heat Shock Factor (2016)
  • Author(s): Fujikake N., Takeuchi T., *Nagai Y.
  • Total Pages: 301
  • Publisher: Springer Publishers, Inc.
• [Book] Drug development for neurodegenerative diseases In Neurodegenerative Disorders as Systemic Diseases (2015)
  • Author(s): Nagai Y., Minakawa E. N.
  • Total Pages: 304
  • Publisher: Springer Publishers, Inc.
• [Remarks] パーキンソン病の新たな発症メカニズムをモデル動物で初めて解明
  • URL: http://www.ncnp.go.jp/press/press_release150916.html
• [Remarks] 細胞同士が助け合って神経細胞の変性を防ぐ仕組みを解明
  • URL: http://www.ncnp.go.jp/press/press_release150428.html
• [Remarks] HP
  • URL: http://www.ncnp.go.jp/nin/guide/r4/index.html
• [Patent(Industrial Property Rights)] 血液脳関門透過性ペプチド (2015)
  • Inventor(s): 武内敏秀、永井義隆、中川慎介、丹羽正美、道具伸也、片岡泰文、他
  • Industrial Property Rights Holder: 京都大学、国立精神・神経医療研究センター、長崎大学・他、福岡大学
  • Industrial Property Rights Type: 特許
  • Industrial Property Number: 2015-052967
  • Filing Date: 2015-03-17
• [Patent(Industrial Property Rights)] ALSの原因タンパク毒性を軽減する核酸 (2014)
  • Inventor(s): 石川欽也、水澤英洋、永井義隆、横田隆徳、石黒太郎、佐藤望、他
  • Industrial Property Rights Holder: 東京医科歯科大学、国立精神・神経医療研究センター
  • Industrial Property Rights Type: 特許
  • Industrial Property Number: 2014-244034
  • Filing Date: 2014-12-02
• [Patent(Industrial Property Rights)] 脊髄小脳失調症31型（SCA31治療剤） (2014)
  • Inventor(s): 石川欽也、水澤英洋、永井義隆、石黒太郎、佐藤望、和田圭司
  • Industrial Property Rights Holder: 東京医科歯科大学、国立精神・神経医療研究センター
  • Industrial Property Rights Type: 特許
  • Industrial Property Number: 2014-244350
  • Filing Date: 2014-12-02