Drug development for polyglutamine diseases by a combined phenotype analysis system

• Project/Area Number: 21390265
• Research Category: Grant-in-Aid for Scientific Research (B)
• Allocation Type: Single-year Grants
• Section: 一般
• Research Field: Neurology
• Research Institution: Tokyo Medical and Dental University
• Principal Investigator: OKAZAWA Hitoshi 東京医科歯科大学, 難治疾患研究所, 教授 (50261996)
• Research Collaborator: 田川 一彦 ; 田村 拓也 ; 戚 美玲 ; 伊藤 日加瑠 ; 塩飽 裕紀 ; 榎戸 靖 ; 曽根 雅紀
• Project Period (FY): 2009 – 2011
• Project Status: Completed (Fiscal Year 2011)
• Budget Amount: ¥18,200,000 (Direct Cost: ¥14,000,000、Indirect Cost: ¥4,200,000); Fiscal Year 2011: ¥2,600,000 (Direct Cost: ¥2,000,000、Indirect Cost: ¥600,000); Fiscal Year 2010: ¥5,980,000 (Direct Cost: ¥4,600,000、Indirect Cost: ¥1,380,000); Fiscal Year 2009: ¥9,620,000 (Direct Cost: ¥7,400,000、Indirect Cost: ¥2,220,000)
• Keywords: 神経分子病態学 / ポリグルタミン病 / ハイスループット / ショウジョウバエ / スクリーニング / 神経変性 / 治療薬 / 動物モデル / マウス / 治療薬スクリーニング / ハンチントン病 / 脊髄小脳失調症1型

Research Abstract

We developed strategically a combined model system for analyzing phenotypes and pathomechanisms and available for developing novel therapeutics of polyglutamine diseases. The system consisted of cell, fly and mouse models was very powerful when combined with omics analyses. We could elucidate a new pathomechanism of polyglutamine diseases, DNA repair impairment and could identify key molecules such as HMGB, Ku70, Hsp70, Omi and Maxer. Furthermore, we identified candidate drugs for polyglutamine diseases by using the combined analysis system.

Research Products

• [Journal Article] Ataxin-7 associates with microtubules and stabilizes the cytoskeletal network (2012)
  • Author(s): Nakamura, Y., Tagawa, K., Oka, T., Sasabe, T., Ito, H., Shiwaku, H., La Spada, A. R. and Okazawa, H
  • Journal Title: Hum Mol Genet Volume: 21(5) Issue: 5 Pages: 1099-1110
  • DOI: 10.1093/hmg/ddr539
  • Peer Reviewed
• [Journal Article] The solution model of the intrinsically disordered polyglutamine tract binding protein-1(PQBP-1) (2012)
  • Author(s): Ress, M., Gorba, C., Gorba, C., de Chiara, C., Bui, T. T. T., Garcia-Maya, M., Drake, A. F., Okazawa, H., Pastre, A., Svergun, D. and Chen, Y. W
  • Journal Title: Biophys J Volume: (in press)
• [Journal Article] Dynamic Changes of the Phosphoproteome in Postmortem Mouse Brains (2011)
  • Author(s): Oka, T., Tagawa, K., Ito, H. and Okazawa, H
  • Journal Title: PLoS One Volume: 6 Issue: 6 Pages: e21405-e21405
  • DOI: 10.1371/journal.pone.0021405
  • Peer Reviewed
• [Journal Article] Ku70 alleviates neurodegeneration in Drosophila models of Huntington's disease (2011)
  • Author(s): Tamura, T., Sone, M., Iwatsubo, T., Tagawa, K., Wanker, E. E. and Okazawa, H
  • Journal Title: PLoS One Volume: 6 Issue: 11 Pages: e27408-e27408
  • DOI: 10.1371/journal.pone.0027408
  • Peer Reviewed
• [Journal Article] Mutant huntingtin impairs Ku70-mediated DNA repair (2010)
  • Author(s): Enokido, Y., Tamura, T., Ito, H., Arumughan, A., Komuro, A., Shiwaku, H., Sone, M., Foulle, R., Sawada, H., Ishiguro, H., Ono, T., Murata, M., Kanazawa, I., Tomilin, N., Tagawa, K., Wanker, E. E., and Okazawa, H.
  • Journal Title: J CellBiol Volume: 189 Issue: 3 Pages: 425-443
  • DOI: 10.1083/jcb.200905138
  • Peer Reviewed
• [Journal Article] Polyglutamine tract-binding protein-1 binds to U5-15kD via a continuous 23-residue segment of the C-terminal domain (2010)
  • Author(s): Takahashi, M., Mizuguchi, M., Shinoda, H., Aizawa, T., Demura, M., Okazawa, H., and Kawano, K.
  • Journal Title: Biochim Biophys Acta Volume: 1804 Issue: 7 Pages: 1500-1507
  • DOI: 10.1016/j.bbapap.2010.03.007
  • Peer Reviewed
• [Journal Article] Suppression of the novel ER protein Maxer by mutant ataxin-1 in Bergman glia contributes to non-cell-autonomous toxicity (2010)
  • Author(s): Shiwaku, H., Yoshimura, N., Tamura, T., Sone, M., Ogishima, S., Watase, K., Tagawa, K., and Okazawa, H.
  • Journal Title: EMBO J. Volume: 29 Issue: 14 Pages: 2446-2460
  • DOI: 10.1038/emboj.2010.116
  • Peer Reviewed
• [Journal Article] Copy-number variations on the X chromosome in Japanese patients with mental retardation detected by array-based comparative genomic hybridization analysis (2010)
  • Author(s): Honda, S., Hayashi, S., Imoto, I., Toyama, J., Okazawa, H., Nakagawa, E., Goto, Y., and Inazawa, J.
  • Journal Title: J Hum Genet Volume: 55 Issue: 9 Pages: 590-599
  • DOI: 10.1038/jhg.2010.74
  • NAID: 10030736689
  • Peer Reviewed
• [Journal Article] Efficiently differentiating vascular endothelial cells from adipose tissue-derived mesenchymal stem cells in serum-free culture (2010)
  • Author(s): Konno, M., Hamazaki, T. S., Fukuda, S., Tokuhara, M., Uchiyama, H., Okazawa, H., Okochi, H., and Asashima, M.
  • Journal Title: Biochem Biophys Res Commun Volume: 400 Issue: 4 Pages: 461
  • DOI: 10.1016/j.bbrc.2010.08.029
  • URL: https://pure.teikyo.jp/en/publications/81e7dddf-cda7-4523-aeab-973b1f8bdbeb
  • Peer Reviewed
• [Journal Article] Drosophila PQBP1 regulates learning acquisition at projection neurons in aversive olfactory conditioning (2010)
  • Author(s): Tamura, T., Horiuchi, D., Chen, Y. C., Sone, M., Miyashita, T., Saitoe, M., Yoshimura, N., Chiang, A. S., and Okazawa, H.
  • Journal Title: J Neurosci Volume: 30 Pages: 14091-14101
• [Journal Article] In-frame Dystrophin Following Exon 51-Skipping Improves Muscle Pathology and Function in the Exon 52-Deficient mdx Mouse (2010)
  • Author(s): Aoki, Y., Nakamura, A., Yokota, T., Saito, T., Okazawa, H., Nagata, T., and Takeda, S
  • Journal Title: Mol Ther Volume: 18 Pages: 1995-200
  • Peer Reviewed
• [Journal Article] Mutant huntingtin impairs Ku70-mediated DNA repair (2010)
  • Author(s): Enokido, Y, Tamura, T., ItO, H, Arumughan, A., Komuro, A., Shiwaku, H., Sone, M., Foulle, R., Sawada, H., Ishiguro, H., Ono, T., Murata, M., Kanazawa, I., Tomilin, N., Tagawa, K, Wanker, E.E., Okazawa.H.
  • Journal Title: The Journal of Cell Biology Volume: 189 Pages: 425-443
  • Peer Reviewed
• [Journal Article] Polyglutamine tract-binding protein-1binds to U5-15kD via a continuous 23-residue segment of the C-terminal domain. (2010)
  • Author(s): Takahashi, M., Mizuguchi, M., Shinoda, E, Aizawa, T., Demura, M., Okazawa, H., Kawano, K.
  • Journal Title: Biochimica et Biophysica Acta Volume: 1804 Pages: 1500-1507
  • Peer Reviewed
• [Journal Article] Suppression of the novel ER protein Maxer by mutant ataxin-1 in Bergman glia contributes to non-cel-autonomous toxicity (2010)
  • Author(s): Shiwaku, H., Yoshimura, N., Tamura, T., Sone, M., Ogishima, S., Watase, K., Tagawa, K., Okazawa.H.
  • Journal Title: The EMBO Journal Volume: 29 Pages: 2446-2460
  • Peer Reviewed
• [Journal Article] Copy-number variations on the X chromosome in Japanese patients withmental retardation detected by array-based comparative genomic hybridization analysis. (2010)
  • Author(s): Honda, S., Hayashi, S., Imoto, I., Tbyama, J., Okazawa, H., Nakagawa, E., Goto, Y, Inazawa, J.
  • Journal Title: Journal of Human Genetics Volume: 55 Pages: 590-599
  • Peer Reviewed
• [Journal Article] Effciently differentiating vascular endothelial cells from adipose tissue-derived mesenchymal stem cells in serum-free culture. (2010)
  • Author(s): Konno, M., Hamazaki, T.S., Fukuda, S., Tobkuhara, M., Uchiyama, H., Okazawa, H., Okochi, H., Asashima, M.
  • Journal Title: Biochemical and Biophysical Research Communications Volume: 400 Pages: 461-465
  • Peer Reviewed
• [Journal Article] Drosophila PQBPl regulates learning acquisition at prqlection neurons in aversive olfactory conditioning. (2010)
  • Author(s): Tamura, T., Horiuchi, D., Chen, YC., Sone, M., Miyashita, T., Saitoe, M, Yoshimura, N., Chiang, A.S., Okazawa, H.
  • Journal Title: The Journal of Neuroscience Volume: 30 Pages: 14091-14101
  • Peer Reviewed
• [Journal Article] In-frame Dystrophin Following Exon 51-Skipping Improves Muscle Pathology and Function in the Exon 52-Deficient mdx Mouse. (2010)
  • Author(s): Aoki, Y, Nakamura, A., Yokota, T, Saito, T, Okazawa.H., Nagata, T, Takeda, S.
  • Journal Title: Molecular Therapy Volume: 18 Pages: 1995-2005
  • Peer Reviewed
• [Journal Article] 天然変性タンパク質PQBP-1の揺らぎと生体機能 (2010)
  • Author(s): 水口峰之、岡澤均
  • Journal Title: メディカルバイオ「揺らぎと生体機能」 Volume: 10月別冊 Pages: 44-48
• [Journal Article] Mutant Huntingtin impairs Ku70-mediated DNA repair. (2010)
  • Author(s): Enokido Y, Tamura T, Ito H, Arumughan A, Komuro A, Shiwaku H, Sone M, Foulle R, Sawada H, Ishiguro H, Ono T, Murata M, Kanazawa I, Tomilin N, Tagawa K, Wanker EE, Okazawa H.
  • Journal Title: Journal of Cell Biology 189 Pages: 425-443
  • Peer Reviewed
• [Journal Article] PQBP1異常による発達障害の分子機構 (2010)
  • Author(s): 伊藤日加瑠、岡澤均
  • Journal Title: 実験医学 28 Pages: 706-12
• [Journal Article] Nematode homologue of PQBP1, a mental retardation causative gene, is involved in lipid metabolism (2009)
  • Author(s): Takahashi, K., Yoshina, S., et al(9名中5番目)
  • Journal Title: PLoS ONE Volume: 4 Issue: 1 Pages: e4104-e4104
  • DOI: 10.1371/journal.pone.0004104
  • URL: https://pure.teikyo.jp/en/publications/160e455d-c114-4609-b08f-e02743e62a58
  • Peer Reviewed
• [Journal Article] Glial cell lineage expression of mutant ataxin-1 and huntingtin induces developmental and late-onset neuronal pathologies in Drosophila models (2009)
  • Author(s): Tamura, T., Sone, M., Yamashita, M., Wanker, E. E., and Okazawa, H.
  • Journal Title: PLoS One Volume: 4 Issue: 1 Pages: e4262-e4262
  • DOI: 10.1371/journal.pone.0004262
  • Peer Reviewed
• [Journal Article] Loss of yata, a novel gene regulating the subcellular localization of APPL, induces deterioration of neural tissues and lifespan shortening (2009)
  • Author(s): Sone M, Uchida A, Komatsu A, Suzuki E, Ibuki I, Asada M, Shiwaku H, Tamura T, Hoshino M, OkazawaH, Nabeshima Y
  • Journal Title: PloS ONE Volume: 4 Issue: 2 Pages: e4466-e4466
  • DOI: 10.1371/journal.pone.0004466
  • Peer Reviewed
• [Journal Article] Progressive decrease in the level of YAPdeltaCs, prosurvival isoforms of YAP, in the spinal cord of transgenic mouse carrying a mutant SOD1 gene (2009)
  • Author(s): Morimoto, N., Nagai, M., Miyazaki, K., Kurata, T., Takehisa, Y., Ikeda, Y., Kamiya, T., Okazawa, H., and Abe, K.
  • Journal Title: J Neurosci Res Volume: 87 Issue: 4 Pages: 928-936
  • DOI: 10.1002/jnr.21902
  • Peer Reviewed
• [Journal Article] Polyglutamine tract binding protein-1 is an intrinsically unstructured protein (2009)
  • Author(s): Takahashi, M., Mizuguchi, M., Shinoda, H., Aizawa, T., Demura, M., Okazawa, H., and Kawano, K.
  • Journal Title: Biochim Biophys Acta Volume: 1794 Issue: 6 Pages: 936-943
  • DOI: 10.1016/j.bbapap.2009.03.001
  • Peer Reviewed
• [Journal Article] Neural stem cells express Oct-3/4 (2009)
  • Author(s): Chin, J. H., Shiwaku, H., Goda, O., Komuro, A., and Okazawa, H.
  • Journal Title: Biochem Biophys Res Commun Volume: 388 Issue: 2 Pages: 247-251
  • DOI: 10.1016/j.bbrc.2009.07.165
  • Peer Reviewed
• [Journal Article] Knock-down of PQBP1 impairs anxiety-related cognition in mouse (2009)
  • Author(s): Ito, H., Yoshimura, N., Kurosawa, M., Ishii, S., Nukina, N., and Okazawa, H.
  • Journal Title: Hum Mol Genet Volume: 18 Issue: 22 Pages: 4239-4254
  • DOI: 10.1093/hmg/ddp378
  • Peer Reviewed
• [Journal Article] Polyglutamine tract binding protein-1 is an intrinsically unstructured protein. (2009)
  • Author(s): Takahashi M, Mizuguchi M, Shinoda H, Aizawa T, Demura M, Okazawa H, Kawano K
  • Journal Title: Biochimica et Biophysica Acta 1794 Pages: 936-43
  • Peer Reviewed
• [Journal Article] Knock down of PQBP1 impairs anxiety-related cognition in mouse. (2009)
  • Author(s): Ito H, Yoshimura N, Kurosawa M, Ishii S, Nukina N, Okazawa H.
  • Journal Title: Human Molecular Genetics 18 Pages: 4539-54
  • Peer Reviewed
• [Journal Article] Neural stem cells express Oct-3/4. (2009)
  • Author(s): Chin JH, Shiwaku H, Goda O, Komuro A, Okazawa H.
  • Journal Title: Biochemical and Biophysical Research Communications. 388 Pages: 247-51
  • Peer Reviewed
• [Journal Article] Pol II特異的阻害薬、α-アマニチンが引き起こす非特異的で緩慢な細胞死(TRIAD) (2009)
  • Author(s): 田村拓也、岡澤均
  • Journal Title: ケミカルバイオロジー 12 Pages: 13-13
• [Journal Article] Omi/HtrA2 is relevant to the selective vulnerability of striatal neurons in Huntington's disease (2008)
  • Author(s): Inagaki, R., Tagawa, K., Qi, M. L., Enokido, Y., Ito, H., Tamura, T., Shimizu, S., Oyanagi, K., Arai, N., Kanazawa, I., Wanker E. E, and Okazawa, H.
  • Journal Title: Eur J Neurosci Volume: 28 Issue: 1 Pages: 30-40
  • DOI: 10.1111/j.1460-9568.2008.06323.x
  • Peer Reviewed
• [Journal Article] Age-dependent change of HMGB1 and DNA double-strand break accumulation in mouse brain (2008)
  • Author(s): Enokido, Y., Yoshitake, A., Ito, H., and Okazawa, H.
  • Journal Title: Biochem Biophys Res Commun Volume: 376 Issue: 1 Pages: 128-133
  • DOI: 10.1016/j.bbrc.2008.08.108
  • Peer Reviewed
• [Journal Article] The induction levels of heat shock protein 70 differentiate the vulnerabilities to mutant huntingtin among neuronal subtypes (2007)
  • Author(s): Tagawa, K., Marubuchi, S., Qi, M. L., Enokido, Y., Tamura, T., Inagaki, R., Murata, M., Kanazawa, I., Wanker, E. E., and Okazawa, H.
  • Journal Title: J Neurosci Volume: 27 Pages: 868-880
  • Peer Reviewed
• [Journal Article] Proteome analysis of soluble nuclear proteins reveals that HMGB1/2 suppress genotoxic stress in polyglutamine diseases (2007)
  • Author(s): Qi, M. L., Tagawa, K., Enokido, Y., Yoshimura, N., Wada, Y., Watase, K., Ishiura, S., Kanazawa, I., Botas, J., Saitoe, M., Wanker, E. E., and Okazawa, H.
  • Journal Title: Nature Cell Biol Volume: 9 Pages: 402-414
  • Peer Reviewed
• [Journal Article] The solution model of the intrinsically disordered polyglutamine tract binding protein-1(PQBP-1)
  • Author(s): Ress, M., Gorba, C., Gorba, C., de Chiara, C., Bui, T.T.T., Garcia-Maya, M., Drake A.F., Okazawa, H., Pastre, A., Svergun, D., Chen, Y.W.
  • Journal Title: Biophys J Volume: (in press)
  • Peer Reviewed
• [Presentation] ポリグルタミン病の分子標的治療を目指して (2012)
  • Author(s): 岡澤均
  • Organizer: 大阪大学蛋白質研究所セミナー
  • Place of Presentation: 大阪大学蛋白質研究所(大阪)
• [Presentation] ポリグルタミン病の病態解明から治療開発へ (2011)
  • Author(s): 岡澤均
  • Organizer: 京都大学CREST特別講演
  • Place of Presentation: 京都大学(京都)
  • Year and Date: 2011-07-13
• [Presentation] SCA1病態におけるDNA損傷修復異常 (2011)
  • Author(s): 田村拓也、岡澤均
  • Organizer: 平成22年度厚生労働省科学研究費補助金「運動失調症の病態解明と治療法開発に関する研究班」平成22年度研究班会議
  • Place of Presentation: 都市センターホテル(東京)
  • Year and Date: 2011-01-13
• [Presentation] Molecular Mechanisms of PQBP1-Linked MR and Microcephaly (2011)
  • Author(s): Okazawa, H
  • Organizer: 15th International Workshop on Fragile X and Other Early-Onset Cognitive Disorders, Harnack-House
  • Place of Presentation: Berlin, Germany
• [Presentation] PQBP1 a new major causative gene for mental retardation and microcephaly, regulates gene expression through mRNA splicing. Elsevier Conference (2011)
  • Author(s): Okazawa, H
  • Organizer: RNA Binding Proteins in Neurological Disease, Sheraton National
  • Place of Presentation: Arlington, U. S. A
• [Presentation] Molecular Mechanisms of PQBP1-Linked MR and Microcephaly (2011)
  • Author(s): Okazawa, H.
  • Organizer: 15^<th> International Workshop on Fragile X and Other Early-Onset Cognitive Disorders
  • Place of Presentation: Harnack-House, Berlin, Germany
• [Presentation] PQBP1, a new major causative gene for mental retardation and microcephaly, regulates gene expression through mRNA splicing (2011)
  • Author(s): Okazawa, H.
  • Organizer: Elsevier Conference "RNA Binding Proteins in Neurological Disease"
  • Place of Presentation: Sheraton National, Arlington, U.S.A.
• [Presentation] Suppression of the novel ER protein Maxer by mutant ataxin-1 in Bergman glia contributes to non-cell-autonomous toxicity (2011)
  • Author(s): Shiwaku, H., Okazawa, H.
  • Organizer: The 6^<th> International Symposium of Institute Network and the 10^<th> Surugadai International Symposium, Joint Usage/Research Program of Medical Research Institute International Symposium
  • Place of Presentation: Tokyo Medical and Dental University
• [Presentation] Neurodegeneration and DNA damage (2011)
  • Author(s): 岡澤均
  • Organizer: 第88回日本生理学会大会・第116回日本解剖学会総会全国学術集会合同大会
  • Place of Presentation: パシフィコ横浜(横浜)
• [Presentation] Brain size control by PQBP1 in neural stem cells (2011)
  • Author(s): Okazawa, H.
  • Organizer: iPS細胞等の初期ステージ橋渡し研究振興に向けたJST-CIRMワークショップ
  • Place of Presentation: 神戸ポートピアホテル(神戸)
• [Presentation] DNA損傷修復からみた神経変性機序 (2011)
  • Author(s): 岡澤均
  • 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(名古屋)
• [Presentation] Single strand annealing of DNA double strand breaks is involved in the SCA1 pathology (2011)
  • Author(s): Okazawa, H., Tamura, T.
  • Organizer: Gordon Conference "CAG Triplet Repeat Disorders"
  • Place of Presentation: Il Ciocco Hotel and Resort Lucca, Barga, Italy
• [Presentation] dPQBP1とprqlection neuronと学習障害 (2010)
  • Author(s): 田村拓也、岡澤均
  • Organizer: 第3回分子高次機能研究会
  • Place of Presentation: 冠着荘(長野)
  • Year and Date: 2010-11-23
• [Presentation] Dynamic change of synapse molecule in developmental disorde (2010)
  • Author(s): Okazawa, H
  • Organizer: Kick off symposium of Scientific Research on Innovative Area"Foundation of Synapse and Neurocircuit Pathology
  • Place of Presentation: Tokyo Medical and Dental University(Tokyo)
  • Year and Date: 2010-10-27
• [Presentation] 神経変性概念のパラダイムシフトと治療戦略 (2010)
  • Author(s): 岡澤均
  • Organizer: 第5回四大学連合文化講演会
  • Place of Presentation: 一橋記念講堂(東京)
  • Year and Date: 2010-10-08
• [Presentation] Molecular Mechanisms of PQBP1-linked Developmental Disorders (2010)
  • Author(s): Okazawa, H
  • Organizer: Seminar Henry Hood Research Program
  • Place of Presentation: Weis Center for Research, Geisinger Chnic(Danville, USA)
  • Year and Date: 2010-09-30
• [Presentation] 神経変性における神経細胞死 (2010)
  • Author(s): 岡澤璽
  • Organizer: 第19回日本Cell Death学会学術集会 -細胞死研究の新たなステージ-
  • Place of Presentation: 愛知県産業労働センター(愛知)
  • Year and Date: 2010-07-31
• [Presentation] 細胞内小胞輸送と神経変性:ショウジョウバエモデルを用いた解析 (2010)
  • Author(s): 曽根雅紀、置麹
  • Organizer: 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: ホテル札幌芸文館(北海道)
  • Year and Date: 2010-07-30
• [Presentation] 変性疾患細胞死モデルとしての転写障害性神経細胞死TRIAD (2010)
  • Author(s): 岡澤均
  • Organizer: 第36回東京女子医科大学・神経懇話会
  • Place of Presentation: 東京女子医科大学(東京)
  • Year and Date: 2010-06-29
• [Presentation] Mutant huntingtin impairs Ku70-mediated DNArepair (2010)
  • Author(s): Ito, H., Enokido, Y, Tamura, T, Okazawa, H.
  • Organizer: The First International Conference of Neural Cell Culture
  • Place of Presentation: Seoul(South Korea)
  • Year and Date: 2010-06-25
• [Presentation] Suppression of the novel ER protein MAXER by mutant ataxin-1 in Bergman glia Contributes to non-cell autonomous toxicity (2010)
  • Author(s): Shiwaku, H., Okazawa, H.
  • Organizer: The First International Conference of Neural Cell Culture
  • Place of Presentation: Seoul(South Korea)
  • Year and Date: 2010-06-25
• [Presentation] ショウジョウバエAPPの細胞内輸送を制御する新しい分子yataの同定と分析 (2010)
  • Author(s): 曽根雅紀、鍋島陽一、圖澤璽
  • Organizer: 第51回日本神経学会総会
  • Place of Presentation: 東京国際フォーラム(東京)
  • Year and Date: 2010-05-22
• [Presentation] 細胞機能から見たポリグルタミン病の神経変性 (2010)
  • Author(s): 岡澤均
  • Organizer: 東京大学大学院医学研究科講義
  • Place of Presentation: 東京大学(東京)
  • Year and Date: 2010-05-11
• [Presentation] 神経変性疾患におけるDNA修復異常と脳老化 (2010)
  • Author(s): 岡澤均
  • Organizer: 第5回Bone Forum in Hanno
  • Place of Presentation: 埼玉
  • Year and Date: 2010-03-03
• [Presentation] Ataxin-1/Huntingtinのグリア細胞由来毒性の解析 (2010)
  • Author(s): 田村拓也、曽根雅紀、山下真弓、Wanker Erich E、岡澤均
  • Organizer: 第51回日本神経学会総会
  • Place of Presentation: 東京国際フォーラム(東京)
• [Presentation] HDAC阻害剤によるPQBP1精神遅滞モデルマウスの治療 (2010)
  • Author(s): 伊藤日加瑠、黒澤大、貫名信行、置澤均
  • Organizer: 第51回日本神経学会総会
  • Place of Presentation: 東京国際フォーラム(東京)
• [Presentation] ハンチントン病におけるDNA損傷修復障害 (2010)
  • Author(s): 伊藤目加瑠、岡澤均
  • Organizer: 平成22年度日本生化学会関東支部例会・第51回新潟生化学懇話会合同研究集会
  • Place of Presentation: 長岡技術科学大学(新潟)
• [Presentation] 精神遅滞を伴う自閉症関連遺伝子の解析と治療法開発 (2010)
  • Author(s): 田村拓也、岡澤均
  • Organizer: 「発達障害の神経科学的基盤の解明と治療法開発に関する研究」平成22年度第1回班会議
  • Place of Presentation: 小平市(東京)
• [Presentation] 変異ハンチンチン蛋白質によるDNA損傷修復酵素Ku70の機能阻害 (2010)
  • Author(s): 榎戸靖、田村拓也、伊藤日加瑠、小室晃彦、塩飽裕紀、岡澤均
  • Organizer: 包括型脳科学研究推進支援ネットワーク 夏のワークシヨップ
  • Place of Presentation: ホテル札幌芸文館(北海道)
• [Presentation] HDAC阻害剤によるPQBPl精神遅滞モデルマウスの治療 (2010)
  • Author(s): 伊藤日加瑠、黒澤大、貫名信行、岡澤均
  • Organizer: 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: ホテル札幌芸文館(北海道)
• [Presentation] PQBP1関連精神遅滞モデルショウジョウバエにおける学習障害 (2010)
  • Author(s): 田村拓也、堀内大輔、曽根雅紀、齋藤 実、宮下知之、岡澤均
  • Organizer: 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: ホテル札幌芸文館(北海道)
• [Presentation] 脊髄小脳失調症l型原因遺伝子Ataxin-lのnon-cell autonomous毒性はMAXERを介する (2010)
  • Author(s): 塩飽裕紀、田村拓也、曽根雅紀、渡瀬啓、岡澤均
  • Organizer: 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: ホテル札幌芸文館(北海道)
• [Presentation] 変異ハンチンチン職白質によるDNA修復酵素Ku70の阻害 (2010)
  • Author(s): 榎戸靖、田村拓也、伊藤日加瑠、小室晃彦、塩飽裕紀、Wanker ErichE、岡澤均
  • Organizer: Neuro 2010
  • Place of Presentation: 神戸コンベンションセンター(兵庫)
• [Presentation] 感覚二次ニューロン依存的な記憶に関わる新規分子、dPQBP1 (2010)
  • Author(s): 田村拓也、堀内大輔、Yi-Chung Chen、曽根雅紀、宮下知之、齊藤実、吉村奈津恵、Ann-Shyn Chiang、岡澤均
  • Organizer: Neuro 2010
  • Place of Presentation: 神戸コンベンションセンター(兵庫)
• [Presentation] ハンチントン病とDNA修復障害 (2010)
  • Author(s): 岡澤均
  • Organizer: 日本人類遺伝学会第55回大会
  • Place of Presentation: 大宮ソニックシティ(埼玉)
• [Presentation] ポリグルタミン病における凝集毒性概念の変遷と治療 (2010)
  • Author(s): 岡澤均
  • Organizer: 第29回日本認知症学会学術集会
  • Place of Presentation: ウインクあいち(愛知)
• [Presentation] Fluctuation and function of polyglutamine tract binding protein-1 (2010)
  • Author(s): 水口峰之、土谷芳、岡澤均、河野敬一
  • Organizer: 「揺らぎと生体機能」第4回公開シンポジウム
  • Place of Presentation: 滋賀県立県民交流センター(滋賀)
• [Presentation] 変異ataxin-1およびhuntingtinのnon-cell autonomous毒性 (2010)
  • Author(s): 岡澤均
  • Organizer: 平成21年度厚生労働省科学研究費補助金「運動失調症の病態解明と治療法開発に関する研究」平成21年度研究班会議
  • Place of Presentation: 東京
• [Presentation] ハンチントン病におけるDNA損傷修復異常に起因する神経変性分子機構の解析 (2009)
  • Author(s): 伊藤日加瑠、榎戸靖、田村拓也、岡澤均
  • Organizer: 第42回精神神経系薬物治療研究報告会
  • Place of Presentation: 大阪
  • Year and Date: 2009-12-04
• [Presentation] PQBP1異常による発達障害の分子機構 (2009)
  • Author(s): 伊藤日加瑠、岡澤均
  • Organizer: 厚生労働省精神・神経疾患研究委託費「神経学的基盤に基づく発達障害の診断・治療ガイドライン策定に関する総合的研究」平成21年度研究班会議
  • Place of Presentation: 東京
  • Year and Date: 2009-11-29
• [Presentation] 網羅的解析から見える神経変性の特異性と共通性 (2009)
  • Author(s): 岡澤均
  • Organizer: 東京都神経科学総合研究所・先端研究セミナー
  • Place of Presentation: 東京
  • Year and Date: 2009-07-23
• [Presentation] 網羅的解析から見た神経変性の選択性と共通性 (2009)
  • Author(s): 岡澤均
  • Organizer: 第50回日本神経学会総会・イブニングセミナー6
  • Place of Presentation: 仙台
  • Year and Date: 2009-05-20
• [Presentation] PQBP1, a causative gene for mental retardation with microcephaly, regulates neural stem cell proliferation thorugh RNA splicing of APC4. (2009)
  • Author(s): 岡澤均
  • Organizer: Roche-Nature Medicine Translational Neuroscience Symposium
  • Place of Presentation: Lucern, Switzerland
• [Presentation] Molecular mechanisms of PQBP1-linked developmental disorders. (2009)
  • Author(s): 岡澤均
  • Organizer: The 32^<nd> Annual Meeting of the Japan Neuroscience Society
  • Place of Presentation: 名古屋
• [Presentation] Glial cell lineage expression of mutant ataxin-1 and huntingtin induces developmental and late onset neuronal pathologies in Drosphila models. (2009)
  • Author(s): Tamura T, Yamashita M, Sone M, Okazawa H.
  • Organizer: The 32^<nd> Annual Meeting of the Japan Neuroscience Society
  • Place of Presentation: 名古屋
• [Presentation] Null mutation of Drosphila yata/CG1973, which regulates trafficking of Amyloid precursor protein like, results in progressive eye vacuolization brain volume reduction and lifespan shortening. (2009)
  • Author(s): Sone M, Okazawa H, Nabeshima Y.
  • Organizer: The 32^<nd> Annual Meeting of the Japan Neuroscience Society
  • Place of Presentation: 名古屋
• [Presentation] 神経幹細胞に発現する新規微小管結合分子Maxcellの同定と神経発生制御 (2009)
  • Author(s): 塩飽裕紀、田村拓也、曽根雅紀、岡澤均
  • Organizer: 第50回日本神経学会総会
  • Place of Presentation: 仙台
• [Presentation] マウス脳におけるHMGB1発現とDNA二重鎖切断の加齢依存的変化 (2009)
  • Author(s): 榎戸靖、吉武綾薫、伊藤日加瑠、岡澤均
  • Organizer: 第50回日本神経学会総会
  • Place of Presentation: 仙台
• [Presentation] Analysis of molecular mechanisms of neurodegeneration with Drosophila. (2009)
  • Author(s): 岡澤均
  • Organizer: The 9th Japanese Drosophila Research Conference
  • Place of Presentation: 掛川
• [Presentation] Loss of yata/CG1973, a novel gene regulating the trafficking of the Amyloid precursor protein orthologue, induces developmental defects, deterioration of neural tissues and lifespan shortening. (2009)
  • Author(s): Sone M, Okazawa H, Nabeshima Y
  • Organizer: The 9th Japanese Drosophila Research Conference
  • Place of Presentation: 掛川
• [Presentation] ポリグルタミン病関連タンパクPQBP1の認知記憶機能への関与 (2009)
  • Author(s): 伊藤日加瑠、岡澤均
  • Organizer: 平成21年度 特定領域研究「統合脳」夏のワークショップ
  • Place of Presentation: 札幌
• [Presentation] ショウジョウバエAPPホモログの細胞内輸送を制御する新規分子yataの同定と遺伝学的解析 (2009)
  • Author(s): 曽根雅紀、岡澤均、鍋島陽一
  • Organizer: 第28回日本認知症学会学術集会
  • Place of Presentation: 仙台
• [Presentation] Molecular genetic analysis of the Drosphilia yata gene. genetic interaction with the APP homologue and involvement in the trafficking regulati on of APP-related proteins. (2009)
  • Author(s): Sone N, Nabeshima Y, Okazawa H.
  • Organizer: 第32回日本分子生物学会年会
  • Place of Presentation: 横浜
• [Presentation] Knock down of PQBP1 inpairs anxiety-related cognition in mouse. (2009)
  • Author(s): Ito H, Yoshimura N, Kurosawa M, Ishii S, Nuking N, Okazawa H.
  • Organizer: 第32回日本分子生物学会年会
  • Place of Presentation: 横浜
• [Presentation] Fluctuation and function of polyglutamine tract binding protein-1. (2009)
  • Author(s): Mizuguchi M, Takahashi M, Okazawa H, Kawano K.
  • Organizer: 新学術領域研究「揺らぎと生体機能」第3回公開シンポジウム
  • Place of Presentation: 名古屋
• [Presentation] ポリグルタミン病態における核ストレスの解析と治療応用 (2009)
  • Author(s): 岡澤均
  • Organizer: 平成21年度特定領域研究「統合脳」冬の班会議
  • Place of Presentation: 東京
• [Presentation] PQBP1 controls brain size through cell cycle regulation of neural stem cells (2008)
  • Author(s): Luo, H., Okazawa, H
  • Organizer: Days of Molecular Medicine 2008, Karolinska Institutet
  • Place of Presentation: Stockholm, Sweden
• [Presentation] Proteome analysis of soluble nuclear proteins unravels a novel cell-protective role of HMGB1/2 to suppress genotoxic stress in the polyglutamine disease pathology (2007)
  • Author(s): Okazawa, H., Qi, M. L
  • Organizer: Gordon Research Conferences 2007 Meeting, CAG Triplet Repeat Disorders, Centre Paul Langevin
  • Place of Presentation: Aussois, France
• [Presentation] Proteome analysis of soluble nuclear proteins unravels a novel cell-protective role of HMGB1/2 to suppress genotoxic stress in the polyglutamine disease pathology (2007)
  • Author(s): Okazawa, H., Qi, M. L
  • Organizer: III Meeting on the Molecular Mechanisms of Neurodegeneration
  • Place of Presentation: Milano, Italy
• [Book] はじめに、精神発達遅滞・自閉症の分子医学 (2011)
  • Author(s): 岡澤均
  • Publisher: 医学のあゆみ
• [Book] DNA損傷修復からみた神経変性機序 (2011)
  • Author(s): 岡澤均
  • Publisher: 臨床神経学
• [Book] ポリグルタミン病における凝集毒性概念の変遷と治療 (2011)
  • Author(s): 岡澤均
  • Publisher: 日本認知症学会誌
• [Book] ハンチントン病の分子病態解明 (2011)
  • Author(s): 岡澤均
  • Publisher: 臨床神経学
• [Book] POLYGLUTAMINEDISEASES in Handbook of Neurochemistry and Molecular Neurobiology : Neural Protein Metabolism and Function
  • Author(s): Okazawa, H.
  • Publisher: Edited by A Lajtha, Springer
• [Remarks] 2007. 3. 26「東京医科歯科大、神経変性疾患、関連タンパク特定-治療法開発も」日本経済産業新聞
• [Remarks] 2007. 3. 26「東京医科歯科大、JST、HMGBたんぱく質が神経細胞を正常な状態に保つ作用を発見」日経Biotechnology Japan
• [Remarks] 2007. 4. 2「DNA修復タンパクが減少難病の治療につながる可能性」共同通信
• [Remarks] 2007. 4. 2「DNA修復タンパクが減少難病の神経変性疾患で」東京新聞
• [Remarks] 2007. 4. 2「DNA修復タンパクが減少難病の神経変性疾患で」京都新聞
• [Remarks] 2010. 4. 28プレスリリース「ハンチントン病の主要病態がDNA損傷修復障害による神経変性であることを解明」? DNA修復機能回復によるハンチントン病の新たな治療法の開発-
• [Remarks] 2010. 5. 4「DNA修復障害が原因ハンチントン病マウス実験で確認」産経新聞朝刊20面
• [Remarks] 2010. 5. 4「神経の難病『ハンチントン病』原因の一端解明医科歯科大など」日本経済新聞朝刊26面
• [Remarks] 2010. 5. 4「ハンチントン病損傷DNA修復を阻害東京医科歯科大教授ら原因たんぱく質解明」毎日新聞朝刊2面
• [Remarks] 2010. 5. 4「ハンチントン病はDNA修復障害東京医科歯科大教授ら発表」共同通信
• [Remarks] 2010. 5. 4「神経難病の原因解明」東京新聞3面
• [Remarks] 2010. 5. 4「原因はDNA修復障害ハンチントン病新たな治療法へ道岡澤教授(東京医科歯科大)ら米誌発表」東奥日報朝刊15面
• [Remarks] 2010. 5. 4「ハンチントン病DNA修復障害が原因」山形新聞朝刊19面
• [Remarks] 2010. 5. 4「神経難病『ハンチントン病』DNA修復障害が原因」茨城新聞朝刊17面
• [Remarks] 2010. 5. 4「DNAの修復阻害ハンチントン病病態を解明東京医歯大教授ら」静岡新聞朝刊21面
• [Remarks] 2010. 5. 4「ハンチントン病の原因DNA修復機能せず東京医科歯科大教授ら発表」信濃毎日新聞朝刊22面
• [Remarks] 2010. 5. 4「神経難病『ハンチントン病』原因タンパク質DNA修復障害東京医歯大教授ら確認」愛媛新聞朝刊3面
• [Remarks] 2010. 5. 4「神経難病ハンチントン病DNA修復障害原因東京医歯大教授ら発表」宮崎日日新聞朝刊5面
• [Remarks] 2010. 5. 4「神経難病ハンチントン病DNA修復障害が原因」大分合同新聞朝刊3面
• [Remarks] 2010. 5. 7「ハンチントン病の発症にDNA修復蛋白Ku70が関与東京医科歯科大グループが発表、治療法の開発に光明か」メディカルトリビューン
• [Remarks] 2010. 5. 7「ハンチントン病原因解明東京医科歯科大DNA修復の酵素不足」読売新聞夕刊16面
• [Remarks] 2010. 5. 21「ハンチントン病の発症解明」朝日新聞25面
• [Remarks] 2010. 6. 8 プレスリリース（JST と共同） 「小脳変性に関与する分子メカニズムを解明（神経変性疾患の治療開発につながることが期待）」
• [Remarks] 2010. 6. 9「小脳の神経細胞変性分子メカニズム解明東京医科歯科大」日刊工業21面
• [Remarks] 2010. 6. 25「「1リットルの涙」難病の原因に迫る」朝日新聞朝刊33面
• [Remarks] 2010. 10. 20「東京医科歯科大学、NMDA受容体のNR1サブユニットの減少がポリグルタミン病による認知障害に関連」BTJアカデミック
• [Patent(Industrial Property Rights)] Prophylactic/Therapeutic Agent for Neurodegenerative Disease (2010)
  • Inventor(s): Hitoshi Okazawa
  • Industrial Property Rights Holder: National Corporation Tokyo Medical and Dental University
  • Filing Date: 2010-09-17
  • Acquisition Date: 2010-11-16
  • Overseas
• [Patent(Industrial Property Rights)] Prophylactic/therapeutic agent for neurodegen erative disease. (2009)
  • Inventor(s): 岡澤均
  • Industrial Property Rights Holder: 東京医科歯科大学
  • Filing Date: 2009-11-12
  • Overseas
• [Patent(Industrial Property Rights)] Prophylactic/Therapeutic Agent for Neurodegenerative Disease (2007)
  • Inventor(s): Hitoshi Okazawa
  • Industrial Property Rights Holder: National Corporation Tokyo Medical and Dental University
  • Filing Date: 2007-04-24
  • Acquisition Date: 2007-04-27
  • Overseas
• [Patent(Industrial Property Rights)] Gene Encoding a Protein and Preventive/Remedy for Neurodegenerative Diseases such as Polyglutamine Diseases by Utilizing the Same (2005)
  • Inventor(s): Hitoshi Okazawa
  • Industrial Property Rights Holder: National Corporation Tokyo Medical and Dental University
  • Filing Date: 2005-11-16
  • Acquisition Date: 2011-05-31
  • Overseas
• [Patent(Industrial Property Rights)] Novel Protein and Preventive/Remedy for Neurodegenerative Disease such as Polyglutamine Disease Using the Same (2005)
  • Inventor(s): Hitoshi Okazawa
  • Industrial Property Rights Holder: National Corporation Tokyo Medical and Dental University
  • Filing Date: 2005-11-16
  • Acquisition Date: 2011-08-24
  • Overseas