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Mechanism of protein aging from the point of turbulence of nucleic acid metabolism or its exclusion mechanism.

Planned Research

Project AreaPrevention of brain protein aging and dementia
Project/Area Number 26117006
Research Category

Grant-in-Aid for Scientific Research on Innovative Areas (Research in a proposed research area)

Allocation TypeSingle-year Grants
Review Section Biological Sciences
Research InstitutionNiigata University

Principal Investigator

Onodera Osamu  新潟大学, 脳研究所, 教授 (20303167)

Research Collaborator Kakita Akiyoshi  
Project Period (FY) 2014-07-10 – 2019-03-31
Project Status Completed (Fiscal Year 2018)
Budget Amount *help
¥113,360,000 (Direct Cost: ¥87,200,000、Indirect Cost: ¥26,160,000)
Fiscal Year 2018: ¥20,800,000 (Direct Cost: ¥16,000,000、Indirect Cost: ¥4,800,000)
Fiscal Year 2017: ¥20,800,000 (Direct Cost: ¥16,000,000、Indirect Cost: ¥4,800,000)
Fiscal Year 2016: ¥20,930,000 (Direct Cost: ¥16,100,000、Indirect Cost: ¥4,830,000)
Fiscal Year 2015: ¥20,800,000 (Direct Cost: ¥16,000,000、Indirect Cost: ¥4,800,000)
Fiscal Year 2014: ¥30,030,000 (Direct Cost: ¥23,100,000、Indirect Cost: ¥6,930,000)
Keywords認知症 / 筋萎縮性側索硬化症 / RNA / スプライシング / エピゲノム / RNA代謝 / ALS / 脳神経疾患 / 核酸 / 循環器 / 蛋白質 / 前頭側頭葉変性症 / TDP-43 / 蛋白 / 遺伝子 / 神経病態
Outline of Final Research Achievements

Proteins that cause dementia are "transformed into pathogenic proteins and accumulated" in "selected nervous systems" with aging. This process is accompanied by "quantitative and qualitative alterations in proteins." The amount of protein is controlled by production and degradation, production is controlled by mRNA, and degradation is controlled by intracellular degradation systems and extracellular efflux mechanisms. We found that the RNA metabolism mechanism of TDP-43 causing age-related neurodegenerative diseases is disturbed. Furthermore, by disrupting RNA metabolism in vivo, we succeeded in causing fragmentation of TDP-43 and inducing apoptosis.

Academic Significance and Societal Importance of the Research Achievements

本研究成果は,TDP-43その物が持つ生理的な制御機構が,ある種の擾乱に対して極めて脆弱であり,それ故に疾患を引き起こすことを示した.この概念は,工学的なロバスト性の概念に類似している.ロバスト性と,その破綻による疾病機序は,老化に伴う疾病を包括する概念である可能性がある.今後は,このロバスト性を生かして,再び秩序を回復する治療方法を模索しうる.このような視点に立った治療方法の開発は新しく,その影響は大きい

Report

(6 results)
  • 2018 Annual Research Report   Final Research Report ( PDF )
  • 2017 Annual Research Report
  • 2016 Annual Research Report
  • 2015 Annual Research Report
  • 2014 Annual Research Report
  • Research Products

    (78 results)

All 2019 2018 2017 2016 2015 2014 Other

All Int'l Joint Research (2 results) Journal Article (19 results) (of which Int'l Joint Research: 3 results,  Peer Reviewed: 18 results,  Open Access: 9 results,  Acknowledgement Compliant: 4 results) Presentation (57 results) (of which Int'l Joint Research: 28 results,  Invited: 11 results)

  • [Int'l Joint Research] National Institute of Mental Health(India)

    • Related Report
      2017 Annual Research Report
  • [Int'l Joint Research] Pennsylvania State University(米国)

    • Related Report
      2017 Annual Research Report
  • [Journal Article] Globular glial tauopathy Type II: Clinicopathological study of two autopsy cases2019

    • Author(s)
      Tanaka Hidetomo、Kawakatsu Shinobu、Toyoshima Yasuko、Miura Takeshi、Mezaki Naomi、Mano Atsushi、Sanpei Kazuhiro、Kobayashi Ryota、Hayashi Hiroshi、Otani Koichi、Ikeuchi Takeshi、Onodera Osamu、Kakita Akiyoshi、Takahashi Hitoshi
    • Journal Title

      Neuropathology

      Volume: 39 Issue: 2 Pages: 111-119

    • DOI

      10.1111/neup.12532

    • Related Report
      2018 Annual Research Report
    • Peer Reviewed
  • [Journal Article] Retinal Vasculopathy With Cerebral Leukodystrophy: Clinicopathologic Features of an Autopsied Patient With a Heterozygous TREX 1 Mutation2018

    • Author(s)
      Saito Rie、Nozaki Hiroaki、Kato Taisuke、Toyoshima Yasuko、Tanaka Hajime、Tsubata Yutaka、Morioka Tetsuo、Horikawa Yoh、Oyanagi Kiyomitsu、Morita Takashi、Onodera Osamu、Kakita Akiyoshi
    • Journal Title

      Journal of Neuropathology & Experimental Neurology

      Volume: 78 Issue: 2 Pages: 181-186

    • DOI

      10.1093/jnen/nly115

    • Related Report
      2018 Annual Research Report
    • Peer Reviewed / Open Access
  • [Journal Article] Clinicopathologic Features of Two Patients With Sporadic Amyotrophic Lateral Sclerosis Who Maintained Communication Ability for Over 30 Years2018

    • Author(s)
      Ito Junko、Shimada Tetsuro、Tada Mari、Shimizu Hiroshi、Wakabayashi Masatoshi、Yokoseki Akio、Onodera Osamu、Takahashi Hitoshi、Kakita Akiyoshi
    • Journal Title

      Journal of Neuropathology & Experimental Neurology

      Volume: 77 Issue: 11 Pages: 981-986

    • DOI

      10.1093/jnen/nly082

    • Related Report
      2018 Annual Research Report
  • [Journal Article] Histopathologic features of an autopsied patient with cerebral small vessel disease and a heterozygous HTRA1 mutation2018

    • Author(s)
      Ito Junko、Nozaki Hiroaki、Toyoshima Yasuko、Abe Takashi、Sato Aki、Hashidate Hideki、Igarashi Shuichi、Onodera Osamu、Takahashi Hitoshi、Kakita Akiyoshi
    • Journal Title

      Neuropathology

      Volume: 38 Issue: 4 Pages: 428-432

    • DOI

      10.1111/neup.12473

    • Related Report
      2018 Annual Research Report
    • Peer Reviewed
  • [Journal Article] Frequency and characteristics of the TBK1 gene variants in Japanese patients with sporadic amyotrophic lateral sclerosis2018

    • Author(s)
      Tohnai Genki、Nakamura Ryoichi、Sone Jun、Nakatochi Masahiro、Yokoi Daichi、Katsuno Masahisa、Watanabe Hazuki、Watanabe Hirohisa、Ito Mizuki、Li Yuanzhe、Izumi Yuishin、et al. Atsuta Naoki、Sobue Gen
    • Journal Title

      Neurobiology of Aging

      Volume: 64 Pages: 158.e15-158.e19

    • DOI

      10.1016/j.neurobiolaging.2017.12.005

    • Related Report
      2017 Annual Research Report
    • Peer Reviewed
  • [Journal Article] Robustness and Vulnerability of the Autoregulatory System That Maintains Nuclear TDP-43 Levels: A Trade-off Hypothesis for ALS Pathology Based on in Silico Data2018

    • Author(s)
      Sugai Akihiro、Kato Taisuke、Koyama Akihide、Koike Yuka、Kasahara Sou、Konno Takuya、Ishihara Tomohiko、Onodera Osamu
    • Journal Title

      Frontiers in Neuroscience

      Volume: 12 Pages: 0-0

    • DOI

      10.3389/fnins.2018.00028

    • Related Report
      2017 Annual Research Report
    • Peer Reviewed / Open Access
  • [Journal Article] CARASIL families from India with 3 novel null mutations in theHTRA1gene2017

    • Author(s)
      Preethish-Kumar Veeramani、Nozaki Hiroaki、Tiwari Sarbesh、Vengalil Seena、Bhat Maya、Prasad Chandrajit、Onodera Osamu、Uemura Masahiro、Doniparthi Seshagiri、Saini Jitender、Nashi Saraswati、Polavarapu Kiran、Nalini Atchayaram
    • Journal Title

      Neurology

      Volume: 89 Issue: 23 Pages: 2392-2394

    • DOI

      10.1212/wnl.0000000000004710

    • Related Report
      2017 Annual Research Report
    • Peer Reviewed / Int'l Joint Research
  • [Journal Article] A CARASIL Patient from Americas with Novel Mutation and Atypical Features: Case Presentation and Literature Review2017

    • Author(s)
      Ibrahimi Muhammad、Nozaki Hiroaki、Lee Angelica、Onodera Osamu、Reichwein Raymond、Wicklund Matthew、El-Ghanem Mohammad
    • Journal Title

      Cerebrovascular Diseases

      Volume: 44 Issue: 3-4 Pages: 135-140

    • DOI

      10.1159/000477358

    • Related Report
      2017 Annual Research Report
    • Peer Reviewed / Int'l Joint Research
  • [Journal Article] Clinicopathological characteristics of patients with amyotrophic lateral sclerosis resulting in a totally locked-in state (communication Stage V).2016

    • Author(s)
      Hayashi K, Mochizuki Y, Takeuchi R, Shimizu T, Nagao M, Watabe K, Arai N, Oyanagi K, Onodera O, Hayashi M, Takahashi H, Kakita A, Isozaki E.
    • Journal Title

      Acta Neuropathol Commun

      Volume: 4 Issue: 1 Pages: 107-107

    • DOI

      10.1186/s40478-016-0379-3

    • NAID

      120007113458

    • Related Report
      2016 Annual Research Report
    • Peer Reviewed / Open Access
  • [Journal Article] Heterogeneity of cerebral TDP-43 pathology in sporadic amyotrophic lateral sclerosis: Evidence for clinico-pathologic subtypes.2016

    • Author(s)
      Takeuchi R, Tada M, Shiga A, Toyoshima Y, Konno T, Sato T, Nozaki H, Kato T, Horie M, Shimizu H, Takebayashi H, Onodera O, Nishizawa M, Kakita A, Takahashi H.
    • Journal Title

      Acta Neuropathol Commun

      Volume: 4 Issue: 1 Pages: 61-61

    • DOI

      10.1186/s40478-016-0335-2

    • Related Report
      2016 Annual Research Report
    • Peer Reviewed / Open Access / Acknowledgement Compliant
  • [Journal Article] Increased cytoplasmic TARDBP mRNA in affected spinal motor neurons in ALS caused by abnormal autoregulation of TDP-43.2016

    • Author(s)
      Koyama A, Sugai A, Kato T, Ishihara T, Shiga A, Toyoshima Y, Koyama M, Konno T, Hirokawa S, Yokoseki A, Nishizawa M, Kakita A, Takahashi H, Onodera O.
    • Journal Title

      Nucleic Acids Res

      Volume: 44 Issue: 12 Pages: 5820-5836

    • DOI

      10.1093/nar/gkw499

    • Related Report
      2016 Annual Research Report
    • Peer Reviewed / Open Access / Acknowledgement Compliant
  • [Journal Article] Distinct molecular mechanisms of HTRA1 mutants in manifesting heterozygotes with CARASIL.2016

    • Author(s)
      Nozaki H, Kato T, Nihonmatsu M, Saito Y, Mizuta I, Noda T, Koike R, Miyazaki K, Kaito M, Ito S, Makino M, Koyama A, Shiga A, Uemura M, Sekine Y, Murakami A, Moritani S, Hara K, Yokoseki A, Kuwano R, Endo N, Momotsu T, Yoshida M, Nishizawa M, Mizuno T, Onodera O
    • Journal Title

      Neurology

      Volume: 86 Issue: 21 Pages: 1-10

    • DOI

      10.1212/wnl.0000000000002694

    • Related Report
      2016 Annual Research Report
    • Peer Reviewed / Open Access / Int'l Joint Research
  • [Journal Article] Pathological and Clinical Spectrum of Progressive Supranuclear Palsy: With Special Reference to Astrocytic Tau Pathology.2016

    • Author(s)
      Yokoyama Y, Toyoshima Y, Shiga A, Tada M, Kitamura H, Hasegawa K, Onodera O, Ikeuchi T, Someya T, Nishizawa M, Kakita A, Takahashi H.
    • Journal Title

      Brain Pathol.

      Volume: 26 Issue: 2 Pages: 155-166

    • DOI

      10.1111/bpa.12265

    • Related Report
      2015 Annual Research Report
    • Peer Reviewed
  • [Journal Article] Roles of inositol 1,4,5-trisphosphate receptors in spinocerebellar ataxias.2016

    • Author(s)
      Tada M, Nishizawa M, Onodera O.
    • Journal Title

      Neurochem Int.

      Volume: 94 Pages: 1-8

    • DOI

      10.1016/j.neuint.2016.01.007

    • Related Report
      2015 Annual Research Report
    • Peer Reviewed / Acknowledgement Compliant
  • [Journal Article] Characteristic features and progression of abnormalities on MRI for CARASIL.2015

    • Author(s)
      Nozaki H, Sekine Y, Fukutake T, Nishimoto Y, Shimoe Y, Shirata A, Yanagawa S, Hirayama M, Tamura M, Nishizawa M, Onodera O.
    • Journal Title

      Neurology.

      Volume: 85 Issue: 5 Pages: 459-463

    • DOI

      10.1212/wnl.0000000000001803

    • Related Report
      2015 Annual Research Report
    • Peer Reviewed
  • [Journal Article] Redefining cerebellar ataxia in degenerative ataxias: lessons from recent research on cerebellar systems.2015

    • Author(s)
      Tada M, Nishizawa M, Onodera O
    • Journal Title

      J Neurol Neurosurg Psychiatry

      Volume: - Issue: 8 Pages: 922-928

    • DOI

      10.1136/jnnp-2013-307225

    • Related Report
      2015 Annual Research Report
    • Peer Reviewed / Acknowledgement Compliant
  • [Journal Article] A blinded international study on the reliability of genetic testing for GGGGCC-repeat expansions in C9orf72 reveals marked differences in results among 14 laboratories.2014

    • Author(s)
      Akimoto C, Volk AE, van Blitterswijk M, Van den Broeck M, Leblond CS, Lumbroso S, Camu W, Neitzel B, Onodera O, et al.
    • Journal Title

      J Med Genet.

      Volume: 51 Pages: 419-424

    • Related Report
      2014 Annual Research Report
    • Peer Reviewed / Open Access
  • [Journal Article] Bunina bodies in motor and non-motor neurons revisited: A pathological study of an ALS patient after long-term survival on a respirator.2014

    • Author(s)
      Kimura T, Jiang H, Konno T, Seto M, Iwanaga K, Tsujihata M, Satoh A, Onodera O, Kakita A, Takahashi H.
    • Journal Title

      Neuropathology

      Volume: なし Issue: 4 Pages: 392-397

    • DOI

      10.1111/neup.12105

    • Related Report
      2014 Annual Research Report
    • Peer Reviewed / Open Access
  • [Journal Article] C9ORF72 repeat-associated non-ATG-translated polypeptides are distributed independently of TDP-43 in a Japanese patient with c9ALS2014

    • Author(s)
      Konno T ほか
    • Journal Title

      Neuropathol Appl Neurobiol

      Volume: 40 Issue: 6 Pages: 783-788

    • DOI

      10.1111/nan.12157

    • Related Report
      2014 Annual Research Report
    • Peer Reviewed / Open Access
  • [Presentation] Gene therapy for DRPLA model mice by AAV-delivered CRISPR / Cas92019

    • Author(s)
      加藤泰介 小野寺理ら
    • Organizer
      Keystone symposia (Genome Engineering: From Mechanisms to Therapies (B4))
    • Related Report
      2018 Annual Research Report
    • Int'l Joint Research
  • [Presentation] Development of Neurofilament-L high sensitivity assay by proximity-dependent DNA ligation method2018

    • Author(s)
      笠原壮 小野寺理ら
    • Organizer
      第59回日本神経学会学術大会
    • Related Report
      2018 Annual Research Report
  • [Presentation] 成人発症脳症血管病を呈する一卵性双生児に認めたCOL4A1スプライスサイト多型の検討2018

    • Author(s)
      酒井直子 小野寺理ら
    • Organizer
      第59回日本神経学会学術大会
    • Related Report
      2018 Annual Research Report
  • [Presentation] The alternative splicing of TARDBP mRNA is affected by the DNA methylation status of TARDBP gene.2018

    • Author(s)
      小池 佑佳 小野寺理ら
    • Organizer
      第59回日本神経学会学術大会
    • Related Report
      2018 Annual Research Report
  • [Presentation] TARDBP mutations and ALS-related splicing factors that cause disturbances in TDP-43 autoregulation2018

    • Author(s)
      須貝章弘 小野寺理ら
    • Organizer
      第59回日本神経学会学術大会
    • Related Report
      2018 Annual Research Report
  • [Presentation] The decrease of copy number of SMN2 gene in progressive muscular atrophy.2018

    • Author(s)
      石原 智彦 小野寺理ら
    • Organizer
      第59回日本神経学会学術大会
    • Related Report
      2018 Annual Research Report
  • [Presentation] The frequency of mutations in NOTCH3 or HTRA1 gene among _Japanese CSVD patients2018

    • Author(s)
      野崎洋明 小野寺理ら
    • Organizer
      第59回日本神経学会学術大会
    • Related Report
      2018 Annual Research Report
  • [Presentation] The DNA methylation status of TARDBP 3’-UTR affects its alternative splicing.2018

    • Author(s)
      小池 佑佳 小野寺理ら
    • Organizer
      Advances in Neurodegenerative Disease Research and Therapy
    • Related Report
      2018 Annual Research Report
    • Int'l Joint Research
  • [Presentation] The SMN2 gene copy number states can affect the onset risk and survival time in Japanese ALS.2018

    • Author(s)
      石原 智彦 小野寺理ら
    • Organizer
      PACTALS soul 2018
    • Related Report
      2018 Annual Research Report
    • Int'l Joint Research
  • [Presentation] 片頭痛を認める遺伝性脳小血管病の臨床的検討2018

    • Author(s)
      酒井直子 小野寺理ら
    • Organizer
      第46回頭痛学会総会
    • Related Report
      2018 Annual Research Report
  • [Presentation] Does the DNA methylation state of the TARDBP 3'-UTR cause sporadic ALS?2018

    • Author(s)
      小池 佑佳 小野寺理ら
    • Organizer
      第41回 日本分子生物学会年会
    • Related Report
      2018 Annual Research Report
  • [Presentation] Efficacy evaluation of nusinersen in an adult spinal muscular atrophy case using peripheral blood mRNA2018

    • Author(s)
      石原 智彦 小野寺理ら
    • Organizer
      29th international symposium on ALS/MND
    • Related Report
      2018 Annual Research Report
    • Int'l Joint Research
  • [Presentation] TARDBP pre-mRNA with a mutation causes disturbance of TDP-43 autoregulation via altered interaction with ALS-related splicing factors2018

    • Author(s)
      須貝章弘 小野寺理ら
    • Organizer
      29th International Symposium on ALS/MND
    • Related Report
      2018 Annual Research Report
    • Int'l Joint Research
  • [Presentation] Phenotypic suppression of DRPLA model mice using CRISPR / Cas9 system.2018

    • Author(s)
      加藤泰介 小野寺理ら
    • Organizer
      Keystone symposia (Advances in Neurodegenerative Disease Research and Therapy (Z3))
    • Related Report
      2018 Annual Research Report
    • Int'l Joint Research
  • [Presentation] 遺伝性脳小血管病における微小出血の特徴2018

    • Author(s)
      上村昌寛, 野崎洋明, 酒井直子先生, 小野寺理
    • Organizer
      第43回日本脳卒中学会学術集会
    • Related Report
      2017 Annual Research Report
  • [Presentation] Promoting excellence in ALS registry and research. Hot topics: Molecular mechanism. ALS registry and research in Japan2017

    • Author(s)
      小野寺理
    • Organizer
      “PACTALS” and “Brain Protein Aging and Dementia Control” Project Joint Meeting
    • Related Report
      2017 Annual Research Report
    • Int'l Joint Research / Invited
  • [Presentation] How are neurovascular units maintained in aging? From a study of HSCVD2017

    • Author(s)
      小野寺理
    • Organizer
      Brain Protein Aging and Dementia Control, 2nd International Symposium
    • Related Report
      2017 Annual Research Report
    • Int'l Joint Research / Invited
  • [Presentation] 脳小血管病の分子病態:CARASILからのアプローチ2017

    • Author(s)
      小野寺理
    • Organizer
      第36回日本認知症学会学術集会
    • Related Report
      2017 Annual Research Report
    • Invited
  • [Presentation] タンパク質老化に寄与する脳小血管ユニットの異常 -非神経細胞の視点から-2017

    • Author(s)
      小野寺理
    • Organizer
      新学術領域研究[グリアアセンブリ][脳タンパク質老化]公開シンポジウム
    • Related Report
      2017 Annual Research Report
    • Invited
  • [Presentation] CARASIL:HTRA1活性の低下と脳小血管病2017

    • Author(s)
      小野寺理
    • Organizer
      第49回日本動脈硬化学会総会
    • Related Report
      2017 Annual Research Report
    • Invited
  • [Presentation] 脳小血管の機能と疾病の分子病態2017

    • Author(s)
      小野寺理
    • Organizer
      第8回日本脳血管・認知症学会総会
    • Related Report
      2017 Annual Research Report
    • Invited
  • [Presentation] The SMN2 gene copy number states can affect the onset risk and survival time in Japanese ALS2017

    • Author(s)
      Ishihara T, Toyoda S, Koyama A, Tada , Atsuta N, Nakamura R, Tohnai G, Sone J, Izumi Y, Kaji R, Morita M, Taniguchi A, Kakita A, Sobue G, Nishizawa M, Onodera O Japanese Consortium for Amyotrophic Lateral Sclerosis Research (JaCALS)
    • Organizer
      28th international symposium on ALS/MND
    • Related Report
      2017 Annual Research Report
    • Int'l Joint Research
  • [Presentation] The alternative splicing of TARDBP mRNA is affected by the methylation status2017

    • Author(s)
      Yuka Koike, Akihiro Sugai, Akio Yokoseki, Osamu Onodera
    • Organizer
      28th international symposium on ALS/MND
    • Related Report
      2017 Annual Research Report
    • Int'l Joint Research
  • [Presentation] A non-genetically modified iPSC-derived neuron model with an increased expression of intrinsic TDP-432017

    • Author(s)
      Akihiro Sugai, Genri Tohyama, Taisuke Kato, Tomohiko Ishihara, Osamu Onodera
    • Organizer
      Amyotrophic lateral sclerosis & Related Motor Neuron Disease (Gordon Research Conference)
    • Related Report
      2017 Annual Research Report
    • Int'l Joint Research
  • [Presentation] The alternative splicing of TARDBP mRNA is affected by the ALS10 associated mutations and methylation status2017

    • Author(s)
      Yuka Koike, Akihiro Sugai, Takuya Konno, Akihide Koyama, Misaki Koyama, Taisuke Kato, Tomohiko Ishihara, Akio Yokoseki, Osamu Onodera
    • Organizer
      Amyotrophic lateral sclerosis & Related Motor Neuron Disease (Gordon Research Conference)
    • Related Report
      2017 Annual Research Report
    • Int'l Joint Research
  • [Presentation] Molecular analysis of mutant high-temperature requirement serine protease A1 identified in patients with familial cerebral small vessel disease2017

    • Author(s)
      Uemura M, Nozaki H, Kato T, Koyama A, Onodera O
    • Organizer
      Neuroscience 2017
    • Related Report
      2017 Annual Research Report
    • Int'l Joint Research
  • [Presentation] The absence of hypertension and the presence of psychiatric symptoms suggest a genetic disorder among patients suspected for hereditary cerebral small vessel disease2017

    • Author(s)
      Uemura M, Nozaki H, Sakai N, Onodera O
    • Organizer
      The 8th Korea Joint Stroke Conference
    • Related Report
      2017 Annual Research Report
    • Int'l Joint Research
  • [Presentation] The prevalence estimates of HTRA1-associated cerebral small vessel disease2017

    • Author(s)
      Hiroaki Nozaki, Akihide Koyama, Masahiro Uemura, Taisuke Kato, Osamu Onodera
    • Organizer
      The 8th Korea-Japan Joint Stroke Conference
    • Related Report
      2017 Annual Research Report
    • Int'l Joint Research
  • [Presentation] A NON-GENETICALLY MODIFIED HUMAN NEURONAL MODEL OF ALS WITH AN INCREASED EXPRESSION OF INTRINSIC TDP-432017

    • Author(s)
      A. Sugai, T. Kato, T. Ishihara, O. Osamu
    • Organizer
      XXIII World Congress of Neurology
    • Related Report
      2017 Annual Research Report
    • Int'l Joint Research
  • [Presentation] The SMN2 gene copy number states can affect the onset risk and survival time in Japanese ALS2017

    • Author(s)
      Ishihara T, Toyoda S, Koyama A, Tada , Atsuta N, Nakamura R, Tohnai G, Sone J, Izumi Y, Kaji R, Morita M, Taniguchi A, Kakita A, Sobue G, Nishizawa M, Onodera O Japanese Consortium for Amyotrophic Lateral Sclerosis Research (JaCALS)
    • Organizer
      XXIII World Congress of Neurology
    • Related Report
      2017 Annual Research Report
    • Int'l Joint Research
  • [Presentation] Molecular analysis of mutant high-temperature requirement serine protease A1 identified in patients with familial cerebral small vessel disease2017

    • Author(s)
      Uemura M, Nozaki H, Kato T, Koyama A, Onodera O
    • Organizer
      XXIII World Congress of Neurology
    • Related Report
      2017 Annual Research Report
    • Int'l Joint Research
  • [Presentation] “CORRECT” for introducing nucleotide substitution with CRISPR/Cas9 system in MAPT gene2017

    • Author(s)
      Yuka Koike, Akihide Koyama, Atsushi Shiga, Akio Yokoseki, Osamu Onodera
    • Organizer
      XXIII World Congress of Neurology
    • Related Report
      2017 Annual Research Report
    • Int'l Joint Research
  • [Presentation] The prevalence estimates of HTRA1-associated cerebral small vessel disease2017

    • Author(s)
      Hiroaki Nozaki, Akihide Koyama, Masahiro Uemura, Taisuke Kato, Osamu Onodera
    • Organizer
      XXIII World Congress of Neurology
    • Related Report
      2017 Annual Research Report
    • Int'l Joint Research
  • [Presentation] <Altered allele-specific gene expression by an ALS-causative TDP-43 mutation2017

    • Author(s)
      須貝章弘、廣川祥子、小山哲秀、今野卓哉、小野寺理
    • Organizer
      2017年度生命科学系学会合同年次大会
    • Related Report
      2017 Annual Research Report
  • [Presentation] Serine protease HTRA1 deficiency induces arteriopathy in cerebral small vessels2017

    • Author(s)
      Kato T, Sekine Y, Fujita N, Nozaki H, Hirokawa S, Sato T, Onodera O
    • Organizer
      2017年度生命科学系学会合同年次大会
    • Related Report
      2017 Annual Research Report
  • [Presentation] 家族性脳小血管病患者で報告された変異型HTRA1蛋白質の機能解析2017

    • Author(s)
      上村昌寛, 野崎洋明, 加藤泰介, 小山哲秀, 小野寺理
    • Organizer
      2017年度生命科学系学会合同年次大会
    • Related Report
      2017 Annual Research Report
  • [Presentation] Distinct mechanisms of HTRA1 mutants in manifesting heterozygotes with CARASIL2017

    • Author(s)
      野崎 洋明, 加藤 泰介, 水田 依久子, 水野 敏樹, 西澤 正豊,小野寺 理
    • Organizer
      第114回日本内科学会総会
    • Related Report
      2017 Annual Research Report
  • [Presentation] CARASIL症候性キャリアの一例2017

    • Author(s)
      野崎洋明, 伊藤 絢子, 阿部崇, 豊島靖子,佐藤晶, 橋立英樹, 五十嵐修一, 高橋均, 小野寺理, 柿田明美
    • Organizer
      第36回日本認知症学会学術集会
    • Related Report
      2017 Annual Research Report
  • [Presentation] Therapeutic strategy utilizing gene editing by CRISPR/Cas9 for polyglutamine disease using DRPLA model2017

    • Author(s)
      Kato T, Hirokawa S, Fujita N, Sato T, Tsuji S, Onodera O
    • Organizer
      第40回日本神経科学大会
    • Related Report
      2017 Annual Research Report
  • [Presentation] セリンプロテアーゼHTRA1の機能欠損は、脳小血管病を引き起こす; Serine protease HTRA1 deficiency induces arteriopathy in cerebral small vessels2017

    • Author(s)
      Kato T, Sekine Y, Fujita N, Nozaki H, Hirokawa S, Sato T, Onodera O
    • Organizer
      第40回日本神経科学大会
    • Related Report
      2017 Annual Research Report
  • [Presentation] Protease activity analysis of mutant high-temperature requirement serine protease A1 identified in patients with familial cerebral small vessel disease2017

    • Author(s)
      Uemura M, Nozaki H, Kato T, Koyama A, Onodera O
    • Organizer
      第40回日本神経科学大会
    • Related Report
      2017 Annual Research Report
  • [Presentation] “CORRECT” for introducing nucleotide substitution with CRISPR/Cas9 system in MAPT gene2017

    • Author(s)
      Yuka Koike, Akihide Koyama, Atsushi Shiga, Akio Yokoseki, Osamu Onodera
    • Organizer
      第40回日本神経科学大会.
    • Related Report
      2017 Annual Research Report
  • [Presentation] 家族性脳小血管病患者で同定された変異型HTRA1蛋白質のプロテアーゼ活性解析2017

    • Author(s)
      上村昌寛, 野崎洋明, 加藤泰介, 小山哲秀, 小野寺理
    • Organizer
      第47回新潟神経学夏季セミナー
    • Related Report
      2017 Annual Research Report
  • [Presentation] RVCL関連変異の細胞内局在についての検討.2017

    • Author(s)
      笠原杏子, 加藤泰介, 野崎洋明, 小山哲秀, 小野寺理.
    • Organizer
      第36回日本認知症学会学術集会
    • Related Report
      2017 Annual Research Report
  • [Presentation] A trade-off theory for TDP-43 proteinopathy; a 'robust yet fragile' nature of the autoregulation.2016

    • Author(s)
      Akihiro Sugai, Osamu Onodera
    • Organizer
      4th Symposium on RNA Metabolism in Neurological Disease.
    • Place of Presentation
      San Diego. (USA)
    • Year and Date
      2016-11-10
    • Related Report
      2016 Annual Research Report
    • Int'l Joint Research
  • [Presentation] Applying "CORRECT" for introducing a mutation in the MAPT gene.2016

    • Author(s)
      Yuka Koike, Akihide Koyama, Atsushi Shiga, Akio Yokoseki, Osamu Onodera
    • Organizer
      4th Symposium on RNA Metabolism in Neurological Disease.
    • Place of Presentation
      San Diego. (USA)
    • Year and Date
      2016-11-10
    • Related Report
      2016 Annual Research Report
    • Int'l Joint Research
  • [Presentation] Analysis of intracellular distribution of TDP-43 mRNA in affected spinal motor neuron with ALS.2016

    • Author(s)
      Taisuke Kato, Akihide Koyama, Akihiro Sugai, Yasuko Toyoshima, Akiyoshi Kakita, Hitoshi Takahashi, Masatoyo Nishizawa, Osamu Onodera.
    • Organizer
      Key stone symposium. Common Mechanisms of Neurodegeneration
    • Place of Presentation
      Keystone, Colorado (USA)
    • Year and Date
      2016-06-12
    • Related Report
      2016 Annual Research Report
    • Int'l Joint Research
  • [Presentation] The expression of Human endogenous retrovirus-K in the neuronal tissues of Japanese ALS patients.2016

    • Author(s)
      Tomohiko Ishihara, Akihide Koyama, Taisuke Kato, Ryoko Takeuchi, Mari Tada, Akiyoshi Kakita, Osamu Onodera.
    • Organizer
      Key stone symposium. Common Mechanisms of Neurodegeneration
    • Place of Presentation
      Keystone, Colorado (USA)
    • Year and Date
      2016-06-12
    • Related Report
      2016 Annual Research Report
    • Int'l Joint Research
  • [Presentation] Depletion of TDP-43 induces mitochondrial fragmentation.2015

    • Author(s)
      Gaku Ito, Akihide Koyama, Atsushi Shiga, Sachiko Hirokawa, Yasuko Toyoshima, Akiyoshi Kakita, Masatoyo Nihizawa, Osamu Onodera.
    • Organizer
      Neuroscience 2015
    • Place of Presentation
      Chicago, (USA)
    • Year and Date
      2015-10-17
    • Related Report
      2015 Annual Research Report
    • Int'l Joint Research
  • [Presentation] he SMN gene copy number states in Japanese ALS patients.2015

    • Author(s)
      T.Ishihara, S.Toyoda, A.Koyama, R.Nakamura, G. Tohnai, N.Atsuta, G.Sobue, M.Nishizawa, O. Onodera
    • Organizer
      10th Brain Research Conference: RNA Metabolism in Neurological Disease
    • Place of Presentation
      Chicago, (USA)
    • Year and Date
      2015-10-15
    • Related Report
      2015 Annual Research Report
    • Int'l Joint Research
  • [Presentation] Detection of CRISPR/Cas9 induced-rare genome editing events by droplet-digital PCR.2015

    • Author(s)
      Siga A, Onodera O.
    • Organizer
      10th Brain Research Conference: RNA Metabolism in Neurological Disease
    • Place of Presentation
      Chicago, (USA)
    • Year and Date
      2015-10-15
    • Related Report
      2015 Annual Research Report
    • Int'l Joint Research
  • [Presentation] verexpression of intrinsic TDP-43 by disruption of the autoregulation in vivo.2015

    • Author(s)
      Akihiro Sugai, Taisuke Kato, Masatoyo Nishizawa, Osamu Onodera.
    • Organizer
      EMBO | EMBL Symposia Mechanisms of Neurodegeneration
    • Place of Presentation
      Heidelberg, (Germany)
    • Year and Date
      2015-06-14
    • Related Report
      2015 Annual Research Report
    • Int'l Joint Research
  • [Presentation] Droplet digital PCR system fascilitate to clone the genome-edited iPS cells.2015

    • Author(s)
      Atsushi Shiga, Masatoyo Nishizaw, Osamu Onodera.
    • Organizer
      Molecular Med Tri-Con 2015
    • Place of Presentation
      San Francisco CA Mosco center
    • Year and Date
      2015-02-16 – 2015-02-18
    • Related Report
      2014 Annual Research Report
    • Invited
  • [Presentation] The reduction of minor spliceosome and aberrant splicing in ALS.2014

    • Author(s)
      T. Ishihara., A. Koyama M., Nishizawa. O. Onodera.
    • Organizer
      Cell Symposium
    • Place of Presentation
      Berkeley, CA, USA Claremont Hotel & Spa
    • Year and Date
      2014-10-19 – 2014-10-21
    • Related Report
      2014 Annual Research Report
    • Invited
  • [Presentation] Endogenous TDP-43 over-expression model with disrupted auto-regulation2014

    • Author(s)
      3.Akihiro Sugai, Taisuke Kato, Akihide Koyama, Masatoyo Nishizawa, Osamu Onodera
    • Organizer
      10th Annual Meeting of the Oligonucleotide Therapeutics Society.
    • Place of Presentation
      Hilton San Diego Resort & Spa, San Diego, California
    • Year and Date
      2014-10-12 – 2014-10-15
    • Related Report
      2014 Annual Research Report
    • Invited
  • [Presentation] Update in amyotrophic lateral sclerosis2014

    • Author(s)
      Osamu Onodera, Masatoyo Nishizawa, Hitoshi Takahashi
    • Organizer
      The XVIII International Congress of Neuropathology.
    • Place of Presentation
      Rio de Janeiro, Brazil Sheraton Rio Hotel & Resort
    • Year and Date
      2014-09-14 – 2014-09-18
    • Related Report
      2014 Annual Research Report
    • Invited
  • [Presentation] RNA metabolism and ALS2014

    • Author(s)
      Osamu Onodera
    • Organizer
      The 37th Annual Meeting of the Japan Neuroscience Society
    • Place of Presentation
      横浜 パシフィコ横浜
    • Year and Date
      2014-09-11 – 2014-09-13
    • Related Report
      2014 Annual Research Report
    • Invited

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Published: 2014-11-20   Modified: 2022-02-28  

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