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Therapeutic strategy for the polyglutamine diseases by selective degradation of expanded polyglutamine proteins using polyglutamine-binding pepetides

Research Project

Project/Area Number 23390237
Research Category

Grant-in-Aid for Scientific Research (B)

Allocation TypeSingle-year Grants
Section一般
Research Field Neurology
Research InstitutionNational Center of Neurology and Psychiatry

Principal Investigator

NAGAI Yoshitaka  独立行政法人国立精神・神経医療研究センター, 神経研究所疾病研究第四部, 室長 (60335354)

Co-Investigator(Renkei-kenkyūsha) KOMATSU Masaaki  (財)東京都臨床医学総合研究所, プロジェクトリーダー (90356254)
Project Period (FY) 2011-04-01 – 2014-03-31
Project Status Completed (Fiscal Year 2013)
Budget Amount *help
¥19,110,000 (Direct Cost: ¥14,700,000、Indirect Cost: ¥4,410,000)
Fiscal Year 2013: ¥6,500,000 (Direct Cost: ¥5,000,000、Indirect Cost: ¥1,500,000)
Fiscal Year 2012: ¥6,500,000 (Direct Cost: ¥5,000,000、Indirect Cost: ¥1,500,000)
Fiscal Year 2011: ¥6,110,000 (Direct Cost: ¥4,700,000、Indirect Cost: ¥1,410,000)
Keywords神経科学 / 蛋白質 / 脳神経疾患 / 治療 / 蛋白質分解 / 神経変性疾患 / ポリグルタミン病 / 分子デザイン
Research Abstract

Toward developing a therapy for the polyglutamine (polyQ) neurodegenerative diseases by specific degradation of expanded polyQ proteins, 1) we showed that p62 plays an important role in the autophagic clearance of polyQ protein, especially its oligomers, resulting in protection against polyQ-induced neurodegeneration in Drosophila. 2) We further designed the p62-QBP1 chimeric protein, which consists of p62, an adaptor protein for selective autophagy and the expanded polyQ-specific binding peptide QBP1, and demonstrated its therapeutic potential for the polyQ diseases by selective degradation of expanded polyQ proteins.

Report

(4 results)
  • 2013 Annual Research Report   Final Research Report ( PDF )
  • 2012 Annual Research Report
  • 2011 Annual Research Report
  • Research Products

    (101 results)

All 2014 2013 2012 2011 Other

All Journal Article (21 results) (of which Peer Reviewed: 11 results) Presentation (72 results) (of which Invited: 13 results) Book (4 results) Remarks (4 results)

  • [Journal Article] Peptide-based therapeutic approaches for treatment of the polyglutamine diseases2014

    • 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

    • Related Report
      2013 Annual Research Report 2013 Final Research Report
    • Peer Reviewed
  • [Journal Article] Identification of ter94, Drosophila VCP, as a strong modulator of motor neuron degeneration induced by knockdown of Caz, Drosophila FUS2014

    • 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

    • Related Report
      2013 Annual Research Report 2013 Final Research Report
    • Peer Reviewed
  • [Journal Article] Na+/H+ exchangers induce autophagy in neurons and inhibit polyglutamine-induced aggregate formation2013

    • Author(s)
      Togashi K., Wakatsuki S., Furuno A., Tokunaga S., Nagai Y., Araki T
    • Journal Title

      PLoS One

      Volume: 8(11) Issue: 11 Pages: e81313-e81313

    • DOI

      10.1371/journal.pone.0081313

    • Related Report
      2013 Annual Research Report 2013 Final Research Report
    • Peer Reviewed
  • [Journal Article] Inhibition of Protein Misfolding/Aggregation Using Polyglutamine Binding Peptide QBP1 as a Therapy for the Polyglutamine Diseases.2013

    • Author(s)
      H. Akiko Popiel
    • Journal Title

      Neuro therapeutics

      Volume: (印刷中) Issue: 3 Pages: 440-446

    • DOI

      10.1007/s13311-013-0184-7

    • Related Report
      2013 Annual Research Report 2013 Final Research Report 2012 Annual Research Report
    • Peer Reviewed
  • [Journal Article] TDP-43 associates with stalled ribosomes and contributes to cell survival during cellular stress2013

    • Author(s)
      Higashi S., Kabuta T., Nagai Y., Tsuchiya Y., Akiyama H., Wada K
    • Journal Title

      J. Neurochem

      Volume: 126(2) Issue: 2 Pages: 288-300

    • DOI

      10.1111/jnc.12194

    • Related Report
      2013 Annual Research Report 2013 Final Research Report
    • Peer Reviewed
  • [Journal Article] コンフォメーション病としての神経変性疾患2013

    • Author(s)
      永井義隆, 藤掛伸宏
    • Journal Title

      ファルマシア

      Volume: 49 (9) Pages: 849-853

    • NAID

      110009995811

    • Related Report
      2013 Annual Research Report 2013 Final Research Report
  • [Journal Article] 認知症におけるシナプス病態2013

    • Author(s)
      畑中悠佑, 和田圭司, 永井義隆
    • Journal Title

      Dementia Japan

      Volume: 27 (2) Pages: 128-135

    • Related Report
      2013 Annual Research Report 2013 Final Research Report
  • [Journal Article] 認知症におけるシナプス病態2013

    • Author(s)
      畑中悠佑、和田圭司、永井義隆
    • Journal Title

      Dementia Japan

      Volume: 印刷中

    • Related Report
      2012 Annual Research Report
  • [Journal Article] Hsp40 Gene Therapy Exerts Therapeutic Effects on Polyglutamine Disease Micevia a Non-Cell Autonomous Mechanism.2012

    • Author(s)
      H. Akiko Popiel
    • Journal Title

      PLoS One

      Volume: 7 Issue: 11 Pages: e51069-e51069

    • DOI

      10.1371/journal.pone.0051069

    • Related Report
      2013 Final Research Report 2012 Annual Research Report
    • Peer Reviewed
  • [Journal Article] Calcium leak through ryanodine receptor is involved in neuronal death induced by mutant huntingtin2012

    • Author(s)
      Suzuki M., Nagai Y., Wada K., Koike T
    • Journal Title

      Biochem. Biophys. Res. Commun

      Volume: 429(1-2) Issue: 1-2 Pages: 18-23

    • DOI

      10.1016/j.bbrc.2012.10.107

    • Related Report
      2013 Final Research Report 2012 Annual Research Report
    • Peer Reviewed
  • [Journal Article] Knockdown of the Drosophila Fused in Sarcoma (FUS) homologue causes deficient locomotive behavior and shortening of motoneuron terminal branches.2012

    • Author(s)
      Sasayama, H., Shimamura, M., Tokuda, T., Azuma, Y., Yoshida, T., Mizuno, T., Nakagawa, M., Fujikake, N., Nagai, Y. and Yamaguchi, M
    • Journal Title

      PLoS ONE

      Volume: 7(6), Issue: 6 Pages: e39483-e39483

    • DOI

      10.1371/journal.pone.0039483

    • Related Report
      2013 Final Research Report 2012 Annual Research Report
    • Peer Reviewed
  • [Journal Article] 神経変性疾患病態研究のキーワード2012

    • Author(s)
      永井義隆
    • Journal Title

      臨床神経学

      Volume: 52 (11) Pages: 874-876

    • NAID

      130004505190

    • Related Report
      2013 Final Research Report 2012 Annual Research Report
  • [Journal Article] ショウジョウバエなど小動物を用いた筋萎縮性側索硬化症モデル2012

    • Author(s)
      藤掛伸宏, 長野清一, 永井義隆
    • Journal Title

      神経内科

      Volume: 76 (3) Pages: 266-274

    • Related Report
      2013 Final Research Report
  • [Journal Article] ショウジョウバエなど小動物を用いた筋萎縮性側索硬化症モデル2012

    • Author(s)
      藤掛伸宏
    • Journal Title

      神経内科

      Volume: 76 Pages: 266-274

    • Related Report
      2011 Annual Research Report
  • [Journal Article] The aggregation inhibitor peptide QBP1 as a therapeutic molecule for the polyglutamine neurodegenerative diseases2011

    • Author(s)
      H.Akiko Popiel
    • Journal Title

      Journal of Atnino Acids

      Volume: 2011 Pages: 265084-265084

    • DOI

      10.4061/2011/265084

    • Related Report
      2013 Final Research Report 2011 Annual Research Report
    • Peer Reviewed
  • [Journal Article] Parkinson's disease-associated mutations in alpha-synuclein and UCH-L1 inhibit the unconventional secretion of UCH-L12011

    • Author(s)
      Konya, C.
    • Journal Title

      Neurochem Int

      Volume: 59 Issue: 2 Pages: 251-258

    • DOI

      10.1016/j.neuint.2011.05.012

    • Related Report
      2013 Final Research Report 2011 Annual Research Report
    • Peer Reviewed
  • [Journal Article] Genetic and clinical analysis in a Chinese parkinsonism-predominant spinocerebellar ataxia type 2 family2011

    • Author(s)
      Sun, H.
    • Journal Title

      J Hum Genet

      Volume: 56 Issue: 4 Pages: 330-334

    • DOI

      10.1038/jhg.2011.14

    • Related Report
      2013 Final Research Report 2011 Annual Research Report
    • Peer Reviewed
  • [Journal Article] QBP1を応用した異常伸長ポリグルタミン蛋白質の特異的分解2011

    • Author(s)
      永井義隆, 貫名信行
    • Journal Title

      臨床神経学

      Volume: 51 (11) Pages: 1108-1110

    • Related Report
      2013 Final Research Report
  • [Journal Article] ショウジョウバエモデルから解明されたTDP-43プロテイノパチーの分子病態2011

    • Author(s)
      永井義隆, 藤掛伸宏
    • Journal Title

      Dementia Japan

      Volume: 25 (2) Pages: 129-136

    • Related Report
      2013 Final Research Report
  • [Journal Article] ショウジョウバエモデルから解明されたTDP-43プロテイノパチーの分子病態2011

    • Author(s)
      永井義隆
    • Journal Title

      Dementia Japan

      Volume: 25 Pages: 129-136

    • Related Report
      2011 Annual Research Report
  • [Journal Article] QBP1を応用した異常伸長ポリグルタミン蛋白質の特異的分解2011

    • Author(s)
      永井義隆
    • Journal Title

      臨床神経学

      Volume: 51 Pages: 1108-1110

    • Related Report
      2011 Annual Research Report
  • [Presentation] Oligomerization of TDP-43 is triggered by imbalance of microtubule-dependent transport, leading to neurodegeneration2014

    • Author(s)
      Nagai Y
    • Organizer
      Int Symp "New frontier of molecular neuropathology 2014"
    • Place of Presentation
      Tokyo, Japan
    • Related Report
      2013 Final Research Report
  • [Presentation] High-nutrient diet aggravates protein misfolding-related neurodegeneration in Drosophila2014

    • Author(s)
      Suzuki M., et al
    • Organizer
      Int Symp "New frontier of molecular neuropathology 2014"
    • Place of Presentation
      Tokyo, Japan
    • Related Report
      2013 Final Research Report
  • [Presentation] Oligomerization of TDP-43 is triggered by imbalance of microtubule-dependent transport, leading to neurodegeneration.2014

    • Author(s)
      Nagai Y.
    • Organizer
      International symposium "New frontier of molecular neuropathology 2014"
    • Place of Presentation
      Tokyo, Japan
    • Related Report
      2013 Annual Research Report
    • Invited
  • [Presentation] Disruption of microtubuledependent transport triggers accumulation and oligomerization of TDP-43 in the cytoplasm, leading to neurodegeneration in ALS2013

    • Author(s)
      Nagai Y
    • Organizer
      Niigata Neurosci Res Meeting
    • Place of Presentation
      Niigata, Japan
    • Year and Date
      2013-06-03
    • Related Report
      2013 Final Research Report
  • [Presentation] Toxic protein conformational transition and amyloid fibril formation in the polyglutamine diseases2013

    • Author(s)
      Nagai Y
    • Organizer
      Int Symp on Amyloidosis
    • Place of Presentation
      Tokyo, Japan
    • Year and Date
      2013-01-24
    • Related Report
      2013 Final Research Report
  • [Presentation] Hsp40はエクソソーム分泌により細胞非自律的なポリグルタミン病の治療効果を発揮する2013

    • Author(s)
      永井義隆
    • Organizer
      第32回日本認知症学会学術集会
    • Place of Presentation
      長野
    • Related Report
      2013 Annual Research Report 2013 Final Research Report
  • [Presentation] 蛋白質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2013

    • Author(s)
      永井義隆
    • Organizer
      第13回日本蛋白質科学会
    • Place of Presentation
      鳥取
    • Related Report
      2013 Annual Research Report 2013 Final Research Report
  • [Presentation] Impairment of microtubule-dependent transport of TDP-43 triggers its aggregation, leading to neurodegeneration in Drosophila models of TDP-43 proteinopathies2013

    • Author(s)
      Fujikake, et al
    • Organizer
      43rd SFN Meeting
    • Place of Presentation
      San Diego, CA
    • Related Report
      2013 Final Research Report
  • [Presentation] Expanded UGGAA repeat RNA associated with SCA31 causes neurodegeneration in Drosophila2013

    • Author(s)
      Ishiguro T., et al
    • Organizer
      43rd SFN Meeting
    • Place of Presentation
      San Diego, CA
    • Related Report
      2013 Final Research Report
  • [Presentation] Dietary restriction improves proteostasis and supresses polyglutamine-induced neurodegeneration in Drosophila2013

    • Author(s)
      Nagai Y., et al
    • Organizer
      7th Gordon Res Conf on CAG Triplet Repeat Disorders
    • Place of Presentation
      Waterville Valley, NH
    • Related Report
      2013 Final Research Report
  • [Presentation] Identification of a polyglutamine oligomerization inhibitor with high brain permeability and safety, which exerts therapeutic effects on multiple mouse models of the polyQ diseases2013

    • Author(s)
      Popiel H.A., et al
    • Organizer
      7th Gordon Res Conf on CAG Triplet Repeat Disorders
    • Place of Presentation
      Waterville Valley, NH
    • Related Report
      2013 Final Research Report
  • [Presentation] Aggregation of TDP-43 is triggered by insufficiency of microtubule-dependent transport in the cytoplasm, leading to neurodegeneration in Drosophila2013

    • Author(s)
      Fujikake N., et al
    • Organizer
      11th Int Conf on Alzheimer's & Parkinson's Dis
    • Place of Presentation
      Florence, Italy
    • Related Report
      2013 Final Research Report
  • [Presentation] Disruption of microtubule-dependent transport triggers accumulation and oligomerization of TDP-43 in the cytoplasm, leading to neurodegeneration in ALS.2013

    • Author(s)
      Nagai Y.
    • Organizer
      Niigata Neuroscience Research Meeting
    • Place of Presentation
      Niigata, Japan
    • Related Report
      2013 Annual Research Report
    • Invited
  • [Presentation] Dietary restriction improves proteostasis and supresses polyglutamine-induced neurodegeneration in Drosophila.2013

    • Author(s)
      Nagai Y., Suzuki M., Fujikake N., Wada K.
    • Organizer
      7th Gordon Research Conference on CAG Triplet Repeat Disorders
    • Place of Presentation
      Waterville Valley, NH
    • Related Report
      2013 Annual Research Report
  • [Presentation] Identification of a polyglutamine oligomerization inhibitor with high brain permeability and safety, which exerts therapeutic effects on multiple mouse models of the polyQ diseases.2013

    • Author(s)
      Popiel H.A., Yamane H., Takahashi T., Tada M., Saitoh Y., Fujita H., Okamoto Y., Toda T., Wada K., Onodera O., Nagai Y.
    • Organizer
      7th Gordon Research Conference on CAG Triplet Repeat Disorders
    • Place of Presentation
      Waterville Valley, NH
    • Related Report
      2013 Annual Research Report
  • [Presentation] Impairment of microtubule-dependent transport of TDP-43 triggers its aggregation, leading to neurodegeneration in Drosophila models of TDP-43 proteinopathies.2013

    • Author(s)
      Fujikake N., Kimura N., Saitoh Y., Suzuki M., Yokoseki A., Onodera O., Wada K., Nagai Y.
    • Organizer
      43rd Annual Meeting of the Society for Neuroscience
    • Place of Presentation
      San Diego, CA
    • Related Report
      2013 Annual Research Report
  • [Presentation] Expanded UGGAA repeat RNA associated with SCA31 causes neurodegeneration in Drosophila.2013

    • Author(s)
      Ishiguro T., Fujikake N., Sato N., Mizusawa H., Wada K., Nagai Y., Ishikawa K.
    • Organizer
      43rd Annual Meeting of the Society for Neuroscience
    • Place of Presentation
      San Diego, CA
    • Related Report
      2013 Annual Research Report
  • [Presentation] DCTN1依存的輸送の障害によりTDP-43発現ショウジョウバエの神経変性は増悪する2013

    • 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54回日本神経学会学術大会
    • Place of Presentation
      東京
    • Related Report
      2013 Annual Research Report
  • [Presentation] GBAの機能喪失によりパーキンソン病モデルショウジョウバエの神経変性は増悪する2013

    • Author(s)
      鈴木マリ、藤掛伸宏、和田圭司、上村匡、永井義隆
    • Organizer
      第54回日本神経学会学術大会
    • Place of Presentation
      東京
    • Related Report
      2013 Annual Research Report
  • [Presentation] SCA31 (UGGAA)nリピートはショウジョウバエで複眼変性を引き起こす2013

    • Author(s)
      石黒太郎、石川欽也、藤掛伸宏、永井義隆、佐藤望、和田圭司、水澤英洋
    • Organizer
      第54回日本神経学会学術大会
    • Place of Presentation
      東京
    • Related Report
      2013 Annual Research Report
  • [Presentation] ショウジョウバエモデルを用いたVps35遺伝子異常によるパーキンソン病発症機構の解析2013

    • Author(s)
      今野昌俊、長谷川隆文、鈴木マリ、藤掛伸宏、永井義隆、三浦永美子、菅野直人、菊池昭夫、青木正志、武田篤
    • Organizer
      第54回日本神経学会学術大会
    • Place of Presentation
      東京
    • Related Report
      2013 Annual Research Report
  • [Presentation] RNAi-mediated knockdown of VPS35 impairs α-synuclein degradation by inhibiting the maturation of cathepsin D.2013

    • Author(s)
      長谷川隆文、今野昌俊、三浦永美子、菅野直人、永井義隆、藤掛伸宏、鈴木マリ、菊池昭夫、青木正志、武田篤
    • Organizer
      第86回日本生化学会
    • Place of Presentation
      横浜
    • Related Report
      2013 Annual Research Report
  • [Presentation] Hsp40の細胞間伝播によるタンパク質恒常性(プロテオスターシス)の維持機構2013

    • Author(s)
      武内敏秀、藤掛伸宏、鈴木マリ、ポピエル明子、菊地寿枝、和田圭司、永井義隆
    • Organizer
      第8回臨床ストレス応答学会
    • Place of Presentation
      長野
    • Related Report
      2013 Annual Research Report
  • [Presentation] Hsp40はエクソソーム依存的な細胞間伝播により個体レベルのタンパク質恒常性維持に寄与している2013

    • Author(s)
      武内敏秀、藤掛伸宏、鈴木マリ、ポピエル明子、菊地寿枝、和田圭司、永井義隆
    • Organizer
      第36回日本分子生物学会
    • Place of Presentation
      兵庫
    • Related Report
      2013 Annual Research Report
  • [Presentation] 過栄養食はインスリン様シグナルを介して神経変性疾患モデルショウジョウバエの神経変性に影響する2013

    • Author(s)
      鈴木マリ、藤掛伸宏、和田圭司、上村匡、永井義隆
    • Organizer
      第36回日本分子生物学会
    • Place of Presentation
      兵庫
    • Related Report
      2013 Annual Research Report
  • [Presentation] 野生型α-シヌクレイン過剰発現に対する遺伝子発現補正型RNAi誘導法の確立と有効性評価2013

    • Author(s)
      高橋理貴、鈴木マリ、藤掛伸宏、村田美穂、和田圭司、永井義隆、北條浩彦
    • Organizer
      第36回日本分子生物学会
    • Place of Presentation
      兵庫
    • Related Report
      2013 Annual Research Report
  • [Presentation] Toxic protein conformational transition and amyloid fibril formation in the polyglutamine diseases2013

    • Author(s)
      Nagai Y.
    • Organizer
      International Symposium on Amyloidosis - Intra and extracellular amyloid formation mechanism in amyloidosis
    • Place of Presentation
      Tokyo
    • Related Report
      2012 Annual Research Report
    • Invited
  • [Presentation] Aggregation of TDP-43 is triggered by insufficiency of microtubule-dependent transport in the cytoplasm, leading to neurodegeneration in Drosophila2013

    • Author(s)
      Fujikake N., Kimura N., Saitoh Y., Yokoseki A., Onodera O., Wada K., Nagai Y.
    • Organizer
      11th International Conference on Alzheimer’s & Parkinson’s Diseases (AD/PD 2013)
    • Place of Presentation
      Florence, Italy
    • Related Report
      2012 Annual Research Report
  • [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2013

    • Author(s)
      永井義隆
    • Organizer
      東京医科歯科大学連携大学院セミナー
    • Place of Presentation
      東京
    • Related Report
      2012 Annual Research Report
    • Invited
  • [Presentation] Non-cell autonomous therapeutic effects of Hsp40 on polyglutamine disease models via its exosome-mediated secretion2012

    • Author(s)
      Nagai Y., et al
    • Organizer
      2nd Int Conf of Neural Cell Culture
    • Place of Presentation
      Tokyo
    • Year and Date
      2012-06-16
    • Related Report
      2013 Final Research Report
  • [Presentation] Molecular targeted therapy against the toxic protein conformation and aggregation for the polyglutamine neurodegenerative disorders2012

    • Author(s)
      Nagai Y
    • Organizer
      Max Planck Institute& NCNP Joint Symp
    • Place of Presentation
      Munich, Germany
    • Related Report
      2013 Final Research Report
  • [Presentation] 微小管依存的輸送の障害によりTDP-43の細胞質蓄積が促進され, ALSモデルショウジョウバエの神経変性を増悪する2012

    • Author(s)
      永井義隆
    • Organizer
      第35回日本神経科学会
    • Place of Presentation
      名古屋
    • Related Report
      2013 Final Research Report
  • [Presentation] 神経変性疾患病態研究のキーワード2012

    • Author(s)
      永井義隆
    • Organizer
      第53回日本神経学会学術大会
    • Place of Presentation
      東京
    • Related Report
      2013 Final Research Report
  • [Presentation] Increased dendritic spine dynamics is an early feature in a SCA1 model mouse2012

    • Author(s)
      Hatanaka Y., et al
    • Organizer
      2012 CSH Asia meeting : Neural Circuit Basis of Behavior and its Disorders
    • Place of Presentation
      Suzhou, China
    • Related Report
      2013 Final Research Report
  • [Presentation] Aggravation of neurodegeneration by high-nutrient diet in Drosophila models of neurodegenerative diseases2012

    • Author(s)
      Suzuki M., et al
    • Organizer
      Keystone Symp on Aging and Diseases ofAging
    • Place of Presentation
      Tokyo, Japan
    • Related Report
      2013 Final Research Report
  • [Presentation] Exosome-mediated cell-to-cell transmission of heat shock proteins contributes to the maintenance of protein homeostasis2012

    • Author(s)
      Takeuchi T., et al
    • Organizer
      EMBO Symp on Quality Control
    • Place of Presentation
      Heidelberg, Germany
    • Related Report
      2013 Final Research Report
  • [Presentation] Non-cell autonomous therapeutic effects of Hsp40 on polyglutamine disease models via its exosome-mediated secretion2012

    • Author(s)
      Nagai Y., Takeuchi T., Popiel H.A., Wada K.
    • Organizer
      2nd International Conference of Neural Cell Culture
    • Place of Presentation
      Tokyo
    • Related Report
      2012 Annual Research Report
    • Invited
  • [Presentation] Molecular targeted therapy against the toxic protein conformation and aggregation for the polyglutamine neurodegenerative disorders2012

    • Author(s)
      Nagai Y.
    • Organizer
      Max Planck Institute & National Center of Neurology and Psychiatry Joint Symposium
    • Place of Presentation
      Munich, Germany
    • Related Report
      2012 Annual Research Report
    • Invited
  • [Presentation] Exosome-mediated cell-to-cell transmission of heat shock proteins contributes to the maintenance of protein homeostasis2012

    • Author(s)
      Takeuchi T., Fujikake N., Wada K., Nagai Y.
    • Organizer
      EMBO Symposium on Quality Control
    • Place of Presentation
      Heidelberg, Germany
    • Related Report
      2012 Annual Research Report
  • [Presentation] Aggravation of neurodegeneration by high-nutrient diet in Drosophila models of neurodegenerative diseases2012

    • Author(s)
      Suzuki M., Fujikake N., Wada K., Nagai Y.
    • Organizer
      Keystone Symposia on Aging and Diseases of Aging
    • Place of Presentation
      Tokyo
    • Related Report
      2012 Annual Research Report
  • [Presentation] Increased dendritic spine dynamics is an early feature in a SCA1 model mouse2012

    • Author(s)
      Hatanaka Y., Wada K., Nagai Y.
    • Organizer
      2012 CSH Asia meeting: Neural Circuit Basis of Behavior and its Disorders
    • Place of Presentation
      Suzhou, China
    • Related Report
      2012 Annual Research Report
  • [Presentation] 神経変性疾患に対するタンパク質の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2012

    • Author(s)
      永井義隆
    • Organizer
      東京医科大学 大学院講義
    • Place of Presentation
      東京
    • Related Report
      2012 Annual Research Report
    • Invited
  • [Presentation] 神経変性疾患に対するタンパク質のミスフォールディング・凝集を標的とした治療戦略-ショウジョウバエモデルを活用して2012

    • Author(s)
      永井義隆
    • Organizer
      東北大学神経内科リサーチセミナー
    • Place of Presentation
      宮城
    • Related Report
      2012 Annual Research Report
    • Invited
  • [Presentation] パーキンソン病モデルショウジョウバエにおけるゴーシェ病原因遺伝子glucocerebrosidaseの役割2012

    • Author(s)
      鈴木マリ、藤掛伸宏、和田圭司、上村匡、永井義隆
    • Organizer
      第10回 神経科学研究会
    • Place of Presentation
      東京
    • Related Report
      2012 Annual Research Report
    • Invited
  • [Presentation] 蛋白質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2012

    • Author(s)
      永井義隆
    • Organizer
      東京大学薬学系研究科大学院講義
    • Place of Presentation
      東京
    • Related Report
      2012 Annual Research Report
    • Invited
  • [Presentation] 神経変性疾患の克服をめざして-蛋白質の構造異常・凝集を標的として-2012

    • Author(s)
      永井義隆
    • Organizer
      東京都立神経病院カンファレンス
    • Place of Presentation
      東京
    • Related Report
      2012 Annual Research Report
    • Invited
  • [Presentation] 神経変性疾患病態研究のキーワード2012

    • Author(s)
      永井義隆
    • Organizer
      第53回 日本神経学会学術大会(教育講演)
    • Place of Presentation
      東京
    • Related Report
      2012 Annual Research Report
    • Invited
  • [Presentation] 微小管依存的輸送の障害によりTDP-43の細胞質蓄積が促進され、ALSモデルショウジョウバエの神経変性を増悪する2012

    • Author(s)
      永井義隆
    • Organizer
      第35回 日本神経科学会(シンポジウム)
    • Place of Presentation
      愛知
    • Related Report
      2012 Annual Research Report
    • Invited
  • [Presentation] ポリグルタミン病モデルショウジョウバエの病態においてp62は保護的に機能している2012

    • Author(s)
      斉藤勇二、藤掛伸宏、岡本佑馬、和田圭司、永井義隆
    • Organizer
      第53回 日本神経学会学術大会
    • Place of Presentation
      東京
    • Related Report
      2012 Annual Research Report
  • [Presentation] TDP-43とFUSはショウジョウバエモデルにおいて相乗的に神経変性を惹き起こす2012

    • Author(s)
      藤掛伸宏、高橋光里、斉藤勇二、小野寺理、和田圭司、永井義隆
    • Organizer
      第53回 日本神経学会学術大会
    • Place of Presentation
      東京
    • Related Report
      2012 Annual Research Report
  • [Presentation] SCA6トランスジェニックショウジョウバエによるCav2.1のCTF毒性の検証2012

    • Author(s)
      石黒太郎、石川欽也、藤掛伸宏、永井義隆、水澤英洋
    • Organizer
      第53回 日本神経学会学術大会
    • Place of Presentation
      東京
    • Related Report
      2012 Annual Research Report
  • [Presentation] SCA1モデルマウスにおけるプルキンエ細胞の樹状突起の初期変化とアストロサイト病変の検討2012

    • Author(s)
      古田晶子、永井義隆、和田圭司、内山安男、H. Brent Clark
    • Organizer
      第53回 日本神経病理学会総会
    • Place of Presentation
      新潟
    • Related Report
      2012 Annual Research Report
  • [Presentation] Disruption of microtubule-dependent transport triggers accumulation and oligomer formation of TDP-43 in the cytoplasm, leading to neurodegeneration in ALS2012

    • Author(s)
      藤掛伸宏、木村展之、斉藤勇二、横関明男、小野寺理、和田圭司、永井義隆
    • Organizer
      第35回 日本神経科学会
    • Place of Presentation
      愛知
    • Related Report
      2012 Annual Research Report
  • [Presentation] パーキンソン病モデルショウジョウバエにおけるα-synuclein毒性はglucocerebrosidaseの機能喪失により増悪す2012

    • Author(s)
      鈴木マリ、藤掛伸宏、和田圭司、永井義隆
    • Organizer
      第35回 日本神経科学会
    • Place of Presentation
      愛知
    • Related Report
      2012 Annual Research Report
  • [Presentation] Disruption of microtubule-dependent transport of TDP-43 triggers aggregation of TDP-43, leading to neurodegeneration in Drosophila models of human disease2012

    • Author(s)
      藤掛伸宏、斉藤勇二、横関明男、小野寺理、和田圭司、永井義隆
    • Organizer
      第10回 日本ショウジョウバエ研究集会
    • Place of Presentation
      東京
    • Related Report
      2012 Annual Research Report
  • [Presentation] High-nutrient diet aggravates neurodegeneration through metabolic signaling pathways in Drosophila models of neurodegenerative diseases2012

    • Author(s)
      鈴木マリ、藤掛伸宏、和田圭司、永井義隆
    • Organizer
      第10回 日本ショウジョウバエ研究集会
    • Place of Presentation
      東京
    • Related Report
      2012 Annual Research Report
  • [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治療研究~ALSモデルを中心に~2012

    • Author(s)
      永井義隆
    • Organizer
      神経疾患のモデル動物研究会
    • Place of Presentation
      大阪(招待講演)
    • Related Report
      2011 Annual Research Report
  • [Presentation] QBP1を応用した異常伸長ポリグルタミン蛋白質の特異的分解2011

    • Author(s)
      永井義隆, 貫名信行
    • Organizer
      第52回日本神経学会
    • Place of Presentation
      名古屋
    • Related Report
      2013 Final Research Report
  • [Presentation] 異常凝集病と病態2011

    • Author(s)
      永井義隆
    • Organizer
      平成23年度大阪大学蛋白質研究所セミナー「蛋白質異常凝集の原理と制御」
    • Place of Presentation
      大阪
    • Related Report
      2013 Final Research Report
  • [Presentation] Molecular mechanism of novel unconventional secretion of Hsp40 to function extracellularly2011

    • Author(s)
      Nagai Y, et al
    • Organizer
      6th Gordon Res Conf on CAG Triplet Repeat Disorders
    • Place of Presentation
      Barga, Italy
    • Related Report
      2013 Final Research Report
  • [Presentation] Hsp40 exerts non-cell autonomous therapeutic effects on polyglutamine disease mice via its unconventional secretion2011

    • Author(s)
      Popiel HA, et al
    • Organizer
      6th Gordon Res Conf on CAG Triplet Repeat Disorders
    • Place of Presentation
      Barga, Italy
    • Related Report
      2013 Final Research Report
  • [Presentation] Molecular mechanism of novel unconventional secretion of Hsp40 to function extracellularly2011

    • Author(s)
      Nagai Y.
    • Organizer
      6th Gordon Research Conference on CAG Triplet Repeat Disorders
    • Place of Presentation
      Garga, Italy
    • Related Report
      2011 Annual Research Report
  • [Presentation] Hsp40 exerts non-cell autonomous therapeutic effects on polyglutamine disease mice via its unconventional secretion2011

    • Author(s)
      Popiel H.A.
    • Organizer
      6th Gordon Research Conference on CAG Triplet Repeat Disorders
    • Place of Presentation
      Garga, Italy
    • Related Report
      2011 Annual Research Report
  • [Presentation] 異常凝集病と病態2011

    • Author(s)
      永井義隆
    • Organizer
      平成23年度大阪大学蛋白質研究所セミナー「蛋白質異常凝集の原理と制御」
    • Place of Presentation
      大阪(招待講演)
    • Related Report
      2011 Annual Research Report
  • [Presentation] QBP1を応用した異常伸長ポリグルタミン蛋白質の特異的分解2011

    • Author(s)
      永井義隆
    • Organizer
      第52回日本神経学会学術大会
    • Place of Presentation
      愛知(招待講演)
    • Related Report
      2011 Annual Research Report
  • [Presentation] 神経変性疾患の創薬に向けた動物モデル~筋萎縮性側索硬化症、パーキンソン病を中心に~2011

    • Author(s)
      永井義隆
    • Organizer
      技術情報協会セミナー「神経変性疾患に求められる薬剤プロファイルと試験デザイン」
    • Place of Presentation
      東京(招待講演)
    • Related Report
      2011 Annual Research Report
  • [Presentation] 凝集阻害分子の遺伝子治療によるポリグルタミン病モデルマウスに対する治療効果2011

    • Author(s)
      ポピエル明子
    • Organizer
      第52回日本神経学会学術大会
    • Place of Presentation
      愛知
    • Related Report
      2011 Annual Research Report
  • [Presentation] TDP-43を発現するALSモデルショウジョウバエにおけるオートファジー系蛋白質分解の関与2011

    • Author(s)
      藤掛伸宏
    • Organizer
      第52回日本神経学会学術大会
    • Place of Presentation
      愛知
    • Related Report
      2011 Annual Research Report
  • [Presentation] ポリグルタミン病モデルマウスに対する凝集阻害分子の遺伝子治療による効果2011

    • Author(s)
      ポピエル明子
    • Organizer
      平成23年度包括型脳科学研究推進支援ネットワーク夏のワークショップ
    • Place of Presentation
      兵庫
    • Related Report
      2011 Annual Research Report
  • [Presentation] HSP40は新規の細胞外分泌機序によりポリグルタミン病モデルに対して細胞非自律的な治療効果を発揮する2011

    • Author(s)
      武内敏秀
    • Organizer
      第34回日本神経科学会
    • Place of Presentation
      神奈川
    • Related Report
      2011 Annual Research Report
  • [Presentation] 高栄養負荷は神経変性疾患モデルショウジョウバエにおける神経変性を増悪する2011

    • Author(s)
      鈴木マリ
    • Organizer
      第34回日本神経科学会
    • Place of Presentation
      神奈川
    • Related Report
      2011 Annual Research Report
  • [Presentation] エクソソームを介したHsp40の細胞外分泌2011

    • Author(s)
      武内敏秀
    • Organizer
      第6回臨床ストレス応答学会
    • Place of Presentation
      愛知
    • Related Report
      2011 Annual Research Report
  • [Book] ポリグルタミン鎖の伸長によるSCA.<アクチュアル脳・神経疾患の臨床⑤>「小脳と運動失調」2013

    • Author(s)
      永井義隆
    • Publisher
      中山書店
    • Related Report
      2013 Final Research Report
  • [Book] <アクチュアル 脳・神経疾患の臨床⑤>「小脳と運動失調」2013

    • Author(s)
      永井義隆
    • Total Pages
      10
    • Publisher
      中山書店
    • Related Report
      2012 Annual Research Report
  • [Book] ハンチントン病.稀少疾患・難病の診断/治療技術と製品開発2012

    • Author(s)
      永井義隆, 和田圭司
    • Publisher
      技術情報協会
    • Related Report
      2013 Final Research Report
  • [Book] 稀少疾患・難病の診断/治療技術と製品開発2012

    • Author(s)
      永井義隆、和田圭司
    • Total Pages
      7
    • Publisher
      技術情報協会
    • Related Report
      2012 Annual Research Report
  • [Remarks]

    • URL

      http://www.ncnp.go.jp/nin/guide/r4/index.html

    • Related Report
      2013 Final Research Report
  • [Remarks] ホームページ

    • URL

      http://www.ncnp.go.jp/nin/guide/r4/index.html

    • Related Report
      2013 Annual Research Report
  • [Remarks] ホームページ

    • URL

      http://www.ncnp.go.jp/nin/guide/r4/index.html

    • Related Report
      2012 Annual Research Report
  • [Remarks]

    • URL

      http://www.ncnp.go.jp/nin/guide/r4/index.html

    • Related Report
      2011 Annual Research Report

URL: 

Published: 2011-04-06   Modified: 2019-07-29  

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