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Molecular bases of pathogenic proteins and mechanisms of the propagation

Planned Research

Project AreaPrevention of brain protein aging and dementia
Project/Area Number 26117005
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 InstitutionTokyo Metropolitan Institute of Medical Science

Principal Investigator

HASEGAWA Masato  公益財団法人東京都医学総合研究所, 認知症・高次脳機能研究分野, 分野長 (10251232)

Co-Investigator(Kenkyū-buntansha) 北本 哲之  東北大学, 医学系研究科, 教授 (20192560)
Research Collaborator NONAKA takashi  
TARUTANI airi  
SHIMOZAWA aki  
HOSOKAWA masato  
SUZUKI genjiro  
SUZUKAKE masami  
TAKEUCHI atsuko  
Project Period (FY) 2014-07-10 – 2019-03-31
Project Status Completed (Fiscal Year 2018)
Budget Amount *help
¥138,970,000 (Direct Cost: ¥106,900,000、Indirect Cost: ¥32,070,000)
Fiscal Year 2018: ¥25,350,000 (Direct Cost: ¥19,500,000、Indirect Cost: ¥5,850,000)
Fiscal Year 2017: ¥25,350,000 (Direct Cost: ¥19,500,000、Indirect Cost: ¥5,850,000)
Fiscal Year 2016: ¥25,350,000 (Direct Cost: ¥19,500,000、Indirect Cost: ¥5,850,000)
Fiscal Year 2015: ¥25,220,000 (Direct Cost: ¥19,400,000、Indirect Cost: ¥5,820,000)
Fiscal Year 2014: ¥37,700,000 (Direct Cost: ¥29,000,000、Indirect Cost: ¥8,700,000)
Keywordsタウ / αシヌクレイン / TDP-43 / C9ORF72 / 神経変性 / プリオン / 伝播 / 感染 / 前頭側頭葉変性症 / プログラニュリン / 神経変性疾患 / 脳神経疾患 / CJD / APP
Outline of Final Research Achievements

MSA-syn showed similar seeding activity to the synthetic fibrils, although DLB-syn showed a lower seeding activity (Tarutani et al, Acta Neuropathol Commun 2018). N-terminally 10- or 30-residues-truncated human α-syn fibrils induced more abundant α-syn pathologies than WT fibrils in mice, whereas other truncated fibrils induced less abundant pathologies (Terada et al, J Biol Chem 2018). Abnormal expansion of a hexanucleotide repeat in an intron of C9ORF72 induced aggregation of poly-GA protein, which may be the mechanism to induce TDP-43, but not tau or α-syn (Nonaka et al, Hum Mol Genet 2018).

Academic Significance and Societal Importance of the Research Achievements

αシヌクレインが蓄積する疾患はパーキンソン病やレビー小体型認知症、多系統萎縮症などいくつかあるが、その病態が異なる理由として、異なる異常構造のαシヌクレインが伝播することによっておこる可能性を示唆した。また、C9ORF72の遺伝子変異は欧米ではALSの原因で最も多い変異と報告されているが、その原因が変異によって生じるGA-repeat蛋白が凝集し、TDP-43が異常型に変化する足場を形成する可能性があることを示唆した。

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

    (87 results)

All 2018 2017 2016 2015 2014 Other

All Int'l Joint Research (9 results) Journal Article (55 results) (of which Int'l Joint Research: 23 results,  Peer Reviewed: 55 results,  Open Access: 33 results,  Acknowledgement Compliant: 5 results) Presentation (16 results) (of which Int'l Joint Research: 4 results,  Invited: 16 results) Remarks (7 results)

  • [Int'l Joint Research] Seoul National University/Yonsei University/Konkuk University(韓国)

    • Related Report
      2016 Annual Research Report
  • [Int'l Joint Research] University of California/Weill Cornell Medical College(米国)

    • Related Report
      2016 Annual Research Report
  • [Int'l Joint Research] The University of Sydney/University of New South Wales(Australia)

    • Related Report
      2016 Annual Research Report
  • [Int'l Joint Research] Manchester Univ/Nat CJDisease Res(英国)

    • Related Report
      2015 Annual Research Report
  • [Int'l Joint Research] Istituto delle Scienze Neurologiche(Italy)

    • Related Report
      2015 Annual Research Report
  • [Int'l Joint Research] MIT and Harvard/Massachusetts General Hospital/Prion Disease Surveillance Center(米国)

    • Related Report
      2015 Annual Research Report
  • [Int'l Joint Research] Australian National CJD Registry(Australia)

    • Related Report
      2015 Annual Research Report
  • [Int'l Joint Research] Nat Ref Center Surv Hum TSE/Center for Neuropathol and Prion Res(Germany)

    • Related Report
      2015 Annual Research Report
  • [Int'l Joint Research]

    • Related Report
      2015 Annual Research Report
  • [Journal Article] C9ORF72 dipeptide repeat poly-GA inclusions promote: intracellular aggregation of phosphorylated TDP-432018

    • Author(s)
      Nonaka T, Masuda-Suzukake M, Hosokawa M, Shimozawa A, Hirai S, Okado, H., Hasegawa, M.
    • Journal Title

      Hum Mol Genet

      Volume: 27 Issue: 15 Pages: 2658-2670

    • DOI

      10.1093/hmg/ddy174

    • Related Report
      2018 Annual Research Report
    • Peer Reviewed / Open Access
  • [Journal Article] The effect of truncation on prion-like properties of α-synuclein.2018

    • Author(s)
      Terada T, Suzuki G, Nonaka T, Kametani F, Tamaoka A, and Hasegawa M.
    • Journal Title

      J Biol Chem

      Volume: 293 Issue: 36 Pages: 13910-13920

    • DOI

      10.1074/jbc.ra118.001862

    • NAID

      120007133755

    • Related Report
      2018 Annual Research Report
    • Peer Reviewed / Open Access
  • [Journal Article] N-Methyl-D-Aspartate Receptor Link to the MAP Kinase Pathway in Cortical and Hippocampal Neurons and Microglia Is Dependent on Calcium Sensors and Is Blocked by α-Synuclein, Tau, and Phospho-Tau in Non-transgenic and Transgenic APPSw,Ind Mice2018

    • Author(s)
      Franco Rafael、Aguinaga David、Reyes Irene、Canela Enric I.、Lillo Jaume、Tarutani Airi、Hasegawa Masato、del Ser-Badia Anna、del Rio Jose A.、Kreutz Michael R.、Saura Carlos A.、Navarro Gemma
    • Journal Title

      Frontiers in Molecular Neuroscience

      Volume: 11 Pages: 273-273

    • DOI

      10.3389/fnmol.2018.00273

    • Related Report
      2018 Annual Research Report
    • Peer Reviewed / Open Access / Int'l Joint Research
  • [Journal Article] Pathological and immunoblot analysis of phosphorylated TDP-43 in sporadic amyotrophic lateral sclerosis with pallido-nigro-luysian degeneration.2018

    • Author(s)
      Uchino A, Ogino M, Takahashi-Fujigasaki J, Oonuma S, Kanazawa N, Kajita S. Ichinoo M, Hasegawa M, Nishiyama K, Murayama S.
    • Journal Title

      Neuropathology

      Volume: 38 Issue: 2 Pages: 171-178

    • DOI

      10.1111/neup.12430

    • Related Report
      2018 Annual Research Report
    • Peer Reviewed / Open Access
  • [Journal Article] Pathological progression of genetic Creutzfeldt-Jakob disease with a PrP V180I mutation.2018

    • Author(s)
      Akagi A, Iwasaki Y, Mimuro M, Kitamoto T, Yamada M, Yoshida M.
    • Journal Title

      Prion

      Volume: 12 Pages: 54-62

    • Related Report
      2018 Annual Research Report
    • Peer Reviewed
  • [Journal Article] Iatrogenic Creutzfeldt-Jakob disease with Amyloid-β pathology: an international study2018

    • Author(s)
      Cali I, Cohen ML, Haik S, Parchi P, Giaccone G, Collins SJ, Kofskey D, Wang H, McLean CA, Brandel JP, Privat N, Sazdovitch V, Duyckaerts C, Kitamoto T, et al
    • Journal Title

      Acta Neuropathologica Communications

      Volume: 6 Issue: 1 Pages: 5-5

    • DOI

      10.1186/s40478-017-0503-z

    • Related Report
      2018 Annual Research Report
    • Peer Reviewed / Open Access / Int'l Joint Research
  • [Journal Article] Development of a quick bioassay for the evaluation of transmission properties of acquired prion diseases.2018

    • Author(s)
      Munesue Y, Shimazaki T, Qi Z, Isoda N, Sawa H, Aoshima K, Kimura T, Mohri S, Kitamoto T, Kobayashi A
    • Journal Title

      Neuroscience Letters

      Volume: 668 Pages: 43-47

    • DOI

      10.1016/j.neulet.2018.01.014

    • Related Report
      2018 Annual Research Report
    • Peer Reviewed / Int'l Joint Research
  • [Journal Article] Biochemical features of genetic Creutzfeldt-Jakob disease with valine-to-isoleucine substitution at codon 180 on the prion protein gene2018

    • Author(s)
      Ito Yoko、Sanjo Nobuo、Hizume Masaki、Kobayashi Atsushi、Ohgami Tetsuya、Satoh Katsuya、Hamaguchi Tsuyoshi、Yamada Masahito、Kitamoto Tetsuyuki、Mizusawa Hidehiro、Yokota Takanori
    • Journal Title

      Biochem Biophys Res Commun

      Volume: 496 Issue: 4 Pages: 1055-1061

    • DOI

      10.1016/j.bbrc.2018.01.119

    • Related Report
      2018 Annual Research Report
    • Peer Reviewed / Int'l Joint Research
  • [Journal Article] A domain responsible for spontaneous conversion of bank vole prion protein2018

    • Author(s)
      Kobayashi Atsushi、Matsuura Yuichi、Takeuchi Atsuko、Yamada Masahito、Miyoshi Ichiro、Mohri Shirou、Kitamoto Tetsuyuki
    • Journal Title

      Brain Pathology

      Volume: 29 Issue: 2 Pages: 155-163

    • DOI

      10.1111/bpa.12638

    • NAID

      120006803949

    • Related Report
      2018 Annual Research Report
    • Peer Reviewed / Open Access / Int'l Joint Research
  • [Journal Article] Progranulin haploinsufficiency reduces amyloid beta deposition in Alzheimer’s disease model mice2018

    • Author(s)
      Hosokawa M, Tanaka Y, Arai T, Kondo H, Akiyama H, Hasegawa M
    • Journal Title

      Experimental Animals

      Volume: 67 Issue: 1 Pages: 63-70

    • DOI

      10.1538/expanim.17-0060

    • NAID

      130006339997

    • ISSN
      0007-5124, 1341-1357, 1881-7122
    • Related Report
      2017 Annual Research Report
    • Peer Reviewed / Open Access / Int'l Joint Research
  • [Journal Article] Isoform-independent and -dependent phosphorylation of microtubule-associated protein tau in mouse brain during postnatal development.2018

    • Author(s)
      Tuerde, D., Kimura, T., Miyasaka, T., Furusawa, K., Shimozawa, A., Hasegawa, M., Ando, A., Hisanaga, S.
    • Journal Title

      J. Biol, Chem.

      Volume: 293 Issue: 5 Pages: 1781-1793

    • DOI

      10.1074/jbc.m117.798918

    • Related Report
      2017 Annual Research Report
    • Peer Reviewed / Open Access
  • [Journal Article] Mutations in bassoon in individuals with familial and sporadic progressive supranuclear palsy-like syndrome2018

    • Author(s)
      Yabe I, Yaguchi H, Kato Y, Miki Y, Takahashi H, Tanikawa S, Shirai S, Takahashi I, Kimura M, Hama Y, Matsushima M, Fujioka S, Kano T, Watanabe M, Nakagawa S, Kunieda Y, Ikeda Y, Hasegawa M, Nishihara H, Ohtsuka T, Tanaka S, Tsuboi Y, Hatakeyama S, Wakabayashi K, Sasaki H
    • Journal Title

      Sci Rep

      Volume: 8 Issue: 1 Pages: 819-819

    • DOI

      10.1038/s41598-018-19198-0

    • NAID

      120006377499

    • Related Report
      2017 Annual Research Report
    • Peer Reviewed / Open Access
  • [Journal Article] Pathological Endogenous α-Synuclein Accumulation in Oligodendrocyte Precursor Cells Potentially Induces Inclusions in Multiple System Atrophy2018

    • Author(s)
      Kaji Seiji、Maki Takakuni、Kinoshita Hisanori、Uemura Norihito、Ayaki Takashi、Kawamoto Yasuhiro、Furuta Takahiro、Urushitani Makoto、Hasegawa Masato、Kinoshita Yusuke、Ono Yuichi、Mao Xiaobo、Quach Tran H.、Iwai Kazuhiro、Dawson Valina L.、Dawson Ted M.、Takahashi Ryosuke
    • Journal Title

      Stem Cell Rep

      Volume: 10 Issue: 2 Pages: 356-365

    • DOI

      10.1016/j.stemcr.2017.12.001

    • NAID

      120006414715

    • Related Report
      2017 Annual Research Report
    • Peer Reviewed / Open Access / Int'l Joint Research
  • [Journal Article] Frontotemporal dementia with trans-activation response DNA-binding protein 13 presenting with cartatonic syndrome.2017

    • Author(s)
      Watanabe R, Kawakami I, Onaya M, Higashi S, Arai N, Akiyama H, Hasegawa M, Arai T
    • Journal Title

      Neuropathology

      Volume: 印刷中 Issue: 3 Pages: 281-287

    • DOI

      10.1111/neup.12442

    • Related Report
      2018 Annual Research Report
    • Peer Reviewed
  • [Journal Article] Accumulation of multiple neurodegenerative disease-related proteins in familial frontotemporal lobar degeneration associated with granulin mutation2017

    • Author(s)
      Hosokawa M, Arai T. Kondo H. Serrano GE. Beach TG. Robinson AC. Mann DM. Akiyama H. Haesgawa M
    • Journal Title

      Scientific Reports

      Volume: 7 Issue: 1 Pages: 1513-1513

    • DOI

      10.1038/s41598-017-01587-6

    • NAID

      120007129118

    • Related Report
      2017 Annual Research Report
    • Peer Reviewed / Open Access / Int'l Joint Research
  • [Journal Article] Progranulin regulates lysosomal function and biogenesis through acidification of lysosomes.2017

    • Author(s)
      Tanaka Y, Suzuki G, Matsuwaki T, Hosokawa M, Serrano G, Beach TG, Yamanouchi K, Hasegawa M, Nishihara M.
    • Journal Title

      Human Molecular Genetics

      Volume: 26 Pages: 969-988

    • DOI

      10.1093/hmg/ddx011

    • Related Report
      2017 Annual Research Report
    • Peer Reviewed / Open Access / Int'l Joint Research / Acknowledgement Compliant
  • [Journal Article] Wild-type monomeric α-synuclein can impair vesicle endocytosis and synaptic fidelity via tubulin polymerization at the calyx of Held.2017

    • Author(s)
      Eguchi K, Taoufiq Z, Thorn-Seshold O, Trauner D, Hasegawa M, Takahashi T
    • Journal Title

      J Neurosci

      Volume: 37 Issue: 25 Pages: 6043-6052

    • DOI

      10.1523/jneurosci.0179-17.2017

    • Related Report
      2017 Annual Research Report
    • Peer Reviewed / Int'l Joint Research
  • [Journal Article] Propagation of pathological α-synuclein in marmoset brain2017

    • Author(s)
      Aki Shimozawa, Maiko Ono, Daisuke Takahara, Airi Tarutani, Sei Imura, Masami Masuda-Suzukake, Makoto Higuchi, Kazuhiko Yanai, Shin-ichi Hisanaga, Masato Hasegawa
    • Journal Title

      Acta Neuropathologica Communications

      Volume: 5 Issue: 1 Pages: 12-14

    • DOI

      10.1186/s40478-017-0413-0

    • Related Report
      2017 Annual Research Report 2016 Annual Research Report
    • Peer Reviewed / Open Access / Acknowledgement Compliant
  • [Journal Article] TDP-43 stabilises the processing intermediates of mitochondrial transcripts.2017

    • Author(s)
      Izumikawa K, Nobe Y, Yoshikawa H, Ishikawa H, Miura Y, Nakayama H, Nonaka T, Hasegawa M, Egawa N, Inoue H, Nishikawa K, Yamano K, Simpson RJ, Taoka M, Yamauchi Y, Isobe T, Takahashi N
    • Journal Title

      Sci Rep

      Volume: 7 Issue: 1 Pages: 7709-7709

    • DOI

      10.1038/s41598-017-06953-y

    • NAID

      120006364102

    • Related Report
      2017 Annual Research Report
    • Peer Reviewed / Open Access / Int'l Joint Research
  • [Journal Article] Unusual tau pathology of the cerebellum in patients with amyotrophic lateral sclerosis/parkinsonism-dementia complex from the kii peninsula, Japan.2017

    • Author(s)
      Morimoto S, Hatsuta H, Kokubo Y, Nakano Y, Hasegawa M, Yoneda M, Hirokawa Y, Kuzuhara S, Shiraishi T, Murayama S.
    • Journal Title

      Brain Pathol.

      Volume: - Issue: 2 Pages: 287-291

    • DOI

      10.1111/bpa.12500

    • Related Report
      2017 Annual Research Report
    • Peer Reviewed / Open Access / Int'l Joint Research
  • [Journal Article] Pick's disease with neuronal four-repeat tau accumulation in the basal ganglia, brain stem nuclei and cerebellum.2017

    • Author(s)
      Ikeda C, Yokota O, Miki T, Takenoshita S, Ishizu H, Mori Y, Yamazaki K, Ozaki Y, Ueno SI, Ishihara T, Hasegawa M, Terada S, Yamada N.
    • Journal Title

      Neuropathology

      Volume: 37 Issue: 6 Pages: 544-559

    • DOI

      10.1111/neup.12394

    • Related Report
      2017 Annual Research Report
    • Peer Reviewed
  • [Journal Article] Neuron-specific methylome analysis reveals epigenetic regulation and tau-related dysfunction of BRCA1 in Alzheimer’s disease2017

    • Author(s)
      Mano Tatsuo、Nagata Kenichi、Nonaka Takashi、Tarutani Airi、Imamura Tomohiro、Hashimoto Tadafumi、Bannai Taro、Koshi-Mano Kagari、Tsuchida Takeyuki、Ohtomo Ryo、Takahashi-Fujigasaki Junko、Yamashita Satoshi、Ohyagi Yasumasa、Yamasaki Ryo、Tsuji Shoji、Tamaoka Akira、et al.
    • Journal Title

      Proc Natl Acad Sci U.S.A.

      Volume: 114 Issue: 45

    • DOI

      10.1073/pnas.1707151114

    • Related Report
      2017 Annual Research Report
    • Peer Reviewed / Open Access / Int'l Joint Research
  • [Journal Article] Desulfation of Heparan Sulfate by Sulf1 and Sulf2 Is Required for Corticospinal Tract Formation2017

    • Author(s)
      Okada Takuya、Keino-Masu Kazuko、Nagamine Satoshi、Kametani Fuyuki、Ohto Tatsuyuki、Hasegawa Masato、van Kuppevelt Toin H.、Kunita Satoshi、Takahashi Satoru、Masu Masayuki
    • Journal Title

      Scientific Reports

      Volume: 7 Issue: 1 Pages: 13847-13847

    • DOI

      10.1038/s41598-017-14185-3

    • NAID

      120007134746

    • Related Report
      2017 Annual Research Report
    • Peer Reviewed / Open Access / Int'l Joint Research
  • [Journal Article] Exposure to bacterial endotoxin generates a distinct strain of α-synuclein fibril.2016

    • Author(s)
      Kim C, Lv G, Lee JS, Jung BC, Masuda-Suzukake M, Hong CS, Valera E, Lee HJ, Paik SR, Hasegawa M, Masliah E, Eliezer D, Lee SJ.
    • Journal Title

      Sci Rep.

      Volume: 6 Issue: 1 Pages: 30891-30891

    • DOI

      10.1038/srep30891

    • Related Report
      2016 Annual Research Report
    • Peer Reviewed / Open Access / Int'l Joint Research
  • [Journal Article] α-Synuclein: Experimental Pathology.2016

    • Author(s)
      Hasegawa M, Nonaka T, Masuda-Suzukake M.
    • Journal Title

      Cold Spring Harb Perspect Med.

      Volume: 6 Issue: 9 Pages: a024273-a024273

    • DOI

      10.1101/cshperspect.a024273

    • Related Report
      2016 Annual Research Report
    • Peer Reviewed / Acknowledgement Compliant
  • [Journal Article] Effect of Fragmented Pathogenic α-Synuclein Seeds on Prion-like Propagation.2016

    • Author(s)
      Tarutani A, Suzuki G, Shimozawa A, Nonaka T, Akiyama H, Hisanaga S, and Hasegawa M.
    • Journal Title

      J. Biol. Chem., J. Biol. Chem.

      Volume: 291 Issue: 36 Pages: 18675-18688

    • DOI

      10.1074/jbc.m116.734707

    • Related Report
      2016 Annual Research Report
    • Peer Reviewed / Open Access
  • [Journal Article] α-Synuclein fibrils exhibit gain-of-toxic-function, promoting tau aggregation and inhibiting microtubule assembly.2016

    • Author(s)
      Oikawa T, Nonaka T, Hisanaga S, Hasegawa M.
    • Journal Title

      J Biol Chem.

      Volume: 291 Issue: 29 Pages: 15046-15056

    • DOI

      10.1074/jbc.m116.736355

    • Related Report
      2016 Annual Research Report
    • Peer Reviewed / Open Access
  • [Journal Article] Biochemical classification of tauopathies by immunoblot, protein sequence and mass spectrometric analyses of sarkosyl-insoluble and trypsin-resistant tau.2016

    • Author(s)
      Taniguchi-Watanabe S, Arai T, Kametani F, Nonaka T, Masuda-Suzukake M, Tarutani A, Murayama S, Saito Y, Arima K, Yoshida M, Akiyama H, Robinson A, Mann DM, Iwatsubo T, Hasegawa M.
    • Journal Title

      Acta Neuropathol.

      Volume: 131(2) Issue: 2 Pages: 267-280

    • DOI

      10.1007/s00401-015-1503-3

    • NAID

      120007128537

    • Related Report
      2015 Annual Research Report
    • Peer Reviewed / Open Access / Int'l Joint Research
  • [Journal Article] Interplay between TDP-43 and docosahexaenoic acid-treated processes in amyotrophic lateral sclerosis.2016

    • Author(s)
      Cacabelosa D, Ayalaa V, Belen Granado-Serranoa A, Jove M, Torresa P, Boadaa J, Cabre R, Ramirez-Nuneza O, Gonzaloa H, Soler-Canterob A, Carlos Enrique Serranoa J, Josep Bellmunta M, Paz Romerob M, Jose Motilvab M, Nonaka T, Hasegawa M, Ferrerd I, Pamplonaa R, and Portero-Otin M.
    • Journal Title

      Neurobiol. Dis.

      Volume: 88 Pages: 148-160

    • DOI

      10.1016/j.nbd.2016.01.007

    • Related Report
      2015 Annual Research Report
    • Peer Reviewed / Int'l Joint Research
  • [Journal Article] The Abundance of Nonphosphorylated Tau in Mouse and Human Tauopathy Brains Revealed by the Use of Phos-Tag Method2016

    • Author(s)
      Kimura T, Hatsuta H, Masuda-Suzukake M, Hosokawa M, Ishiguro K, Akiyama H, Murayama S, Hasegawa M, Hisanaga S
    • Journal Title

      American Journal of Pathology

      Volume: 186 Issue: 2 Pages: 398-409

    • DOI

      10.1016/j.ajpath.2015.10.009

    • Related Report
      2015 Annual Research Report
    • Peer Reviewed / Open Access
  • [Journal Article] Quantifying prion disease penetrance using large population control cohorts.2016

    • Author(s)
      Minikel EV, Vallabh SM, Lek M, Estrada K, Samocha KE, et al
    • Journal Title

      Sci Transl Med.

      Volume: 8 Issue: 322

    • DOI

      10.1126/scitranslmed.aad5169

    • Related Report
      2015 Annual Research Report
    • Peer Reviewed / Open Access / Int'l Joint Research
  • [Journal Article] Extracellular association of APP and tau fibrils induces intracellular aggregate formation of tau.2015

    • Author(s)
      Takahashi M, Miyata H, Kametani F, Nonaka T, Akiyama H, Hisanaga SI, Hasegawa M.
    • Journal Title

      Acta Neuropathol.

      Volume: 129 Issue: 6 Pages: 895-907

    • DOI

      10.1007/s00401-015-1415-2

    • Related Report
      2015 Annual Research Report 2014 Annual Research Report
    • Peer Reviewed / Open Access / Int'l Joint Research
  • [Journal Article] The twenty-four KDa C-terminal tau fragment increases with aging in tauopathy mice: implications of prion-like properties.2015

    • Author(s)
      Matsumoto, S.E.;Motoi, Y.;Ishiguro, K.;Tabira, T.;Kametani, F.;Hasegawa, M.;Hattori, N.
    • Journal Title

      Hum Mol Genet

      Volume: 24 Issue: 22 Pages: 6403-6416

    • DOI

      10.1093/hmg/ddv351

    • Related Report
      2015 Annual Research Report
    • Peer Reviewed / Int'l Joint Research
  • [Journal Article] Progranulin reduction is associated with tau phosphorylation in P301L tau transgenic mice2015

    • Author(s)
      Hosokawa M, Arai T, Masuda-Suzukake M, Kondo H, Matsuwaki T, Nishihara M, Hasegawa M, Akiyama H
    • Journal Title

      J Neuropahol Exp Neurol

      Volume: 74 Issue: 2 Pages: 158-165

    • DOI

      10.1097/nen.0000000000000158

    • Related Report
      2015 Annual Research Report 2014 Annual Research Report
    • Peer Reviewed
  • [Journal Article] Accumulation of dipeptide repeat proteins predates that of TDP-43 in Frontotemporal Lobar Degeneration associated with hexanucleotide repeat expansions in C9ORF72 gene2015

    • Author(s)
      2.Baborie A, Griffiths TD, Jaros E, Perry R, McKeith IG, Burn DJ, Masuda-Suzukake M, Hasegawa M, Rollinson S, Pickering-Brown S, Robinson AC, Davidson YS, Mann DM
    • Journal Title

      Neuropathol Appl Neurobiol

      Volume: in press Issue: 5 Pages: 601-12

    • DOI

      10.1111/nan.12178

    • Related Report
      2015 Annual Research Report 2014 Annual Research Report
    • Peer Reviewed / Int'l Joint Research
  • [Journal Article] An autopsy case of familial amyotrophic lateral sclerosis with a TARDBP Q343R mutation.2015

    • Author(s)
      Okamoto K, Fujita Y, Hoshino E, Tamura Y, Fukuda T, Hasegawa M, Takatama M.
    • Journal Title

      Neuropathology

      Volume: 35 Issue: 5 Pages: 462-8

    • DOI

      10.1111/neup.12209

    • Related Report
      2015 Annual Research Report
    • Peer Reviewed
  • [Journal Article] Decreased regional cerebral blood flow in the bilateral thalami and medulla oblongata determined by an easy Z-score (eZIS) analysis of (99m)Tc-ECD-SPECT images in a case of MM2-thalamic-type sporadic Creutzfeldt-Jakob disease.2015

    • Author(s)
      Hayashi Y, Iwasaki Y, Yoshikura N, Asano T, Hatano T, Tatsumi S, Satoh K, Kimura A, Kitamoto T, Yoshida M, Inuzuka T.
    • Journal Title

      J Neurol Sci

      Volume: 358 Issue: 1-2 Pages: 447-452

    • DOI

      10.1016/j.jns.2015.09.356

    • Related Report
      2015 Annual Research Report
    • Peer Reviewed / Int'l Joint Research
  • [Journal Article] Factors influencing the survival period in Japanese patients with sporadic Creutzfeldt-Jakob disease.2015

    • Author(s)
      Iwasaki Y, Akagi A, Mimuro M, Kitamoto T, Yoshida M.
    • Journal Title

      J Neurol Sci.

      Volume: 357 Issue: 1-2 Pages: 63-8

    • DOI

      10.1016/j.jns.2015.06.065

    • Related Report
      2015 Annual Research Report
    • Peer Reviewed
  • [Journal Article] The influence of PRNP polymorphisms on human prion disease susceptibility: an update2015

    • Author(s)
      Kobayashi A, Teruya K, Matsuura Y, Shirai T, Nakamura Y, Yamada M, Mizusawa H, Mohri S, Kitamoto T.
    • Journal Title

      Acta Neuropathologica

      Volume: 130 Issue: 2 Pages: 159-170

    • DOI

      10.1007/s00401-015-1447-7

    • Related Report
      2015 Annual Research Report
    • Peer Reviewed / Open Access
  • [Journal Article] Sporadic Creutzfeldt-Jakob Disease MM1+2C and MM1 Are Identical in Transmission Properties.2015

    • Author(s)
      Kobayashi A, Matsuura Y, Iwaki T, Iwasaki Y, Yoshida M, Takahashi H, Murayama S, Takao M, Kato S, Yamada M, Mohri S, Kitamoto T.
    • Journal Title

      Brain Pathol

      Volume: in press Issue: 1 Pages: 95-101

    • DOI

      10.1111/bpa.12264

    • Related Report
      2015 Annual Research Report 2014 Annual Research Report
    • Peer Reviewed / Int'l Joint Research
  • [Journal Article] Transmission properties of atypical Creutzfeldt-Jakob disease: a clue to disease etiology?.2015

    • Author(s)
      Kobayashi A, Parchi P, Yamada M, Brown P, Saverioni D, Matsuura Y, Takeuchi A, Mohri S, Kitamoto T
    • Journal Title

      J. Virol

      Volume: 89(7) Issue: 7 Pages: 3939-46

    • DOI

      10.1128/jvi.03183-14

    • Related Report
      2015 Annual Research Report 2014 Annual Research Report
    • Peer Reviewed / Acknowledgement Compliant
  • [Journal Article] Descriptive Epidemiology of Prion Disease in Japan: 1999–20122015

    • Author(s)
      Nakamaura Y, Ae R, Takumi I, Sanjo N, Kitamoto T, Yamada M, Mizusawa H.
    • Journal Title

      Journal of Epidemiology

      Volume: 25 Issue: 1 Pages: 8-14

    • DOI

      10.2188/jea.JE20140022

    • NAID

      130004694366

    • ISSN
      0917-5040, 1349-9092
    • Related Report
      2014 Annual Research Report
    • Peer Reviewed
  • [Journal Article] Creutzfeldt-Jakob disease with homozygous M232R mutation: A case report.2015

    • Author(s)
      Kon T, Miki Y, Arai A, Funamizu Y, Ueno T, Haga R, Nishijima H, Suzuki C, Nunomura J, Baba M, Oyama Y, Shiga Y, Kitamoto T, Tomiyama M.
    • Journal Title

      J Neurol Sci

      Volume: 352 Issue: 1-2 Pages: 108-9

    • DOI

      10.1016/j.jns.2015.03.017

    • Related Report
      2014 Annual Research Report
    • Peer Reviewed
  • [Journal Article] Distinct pathways leading to TDP-43-induced cellular dysfunctions.2014

    • Author(s)
      Yamashita M, Nonaka T, Hirai S, Miwa A, Okado H, Arai T, Hosokawa M, Akiyama H, Hasegawa M.
    • Journal Title

      Hum Mol Genet.

      Volume: 23 Issue: 16 Pages: 4345-56

    • DOI

      10.1093/hmg/ddu152

    • Related Report
      2014 Annual Research Report
    • Peer Reviewed / Open Access
  • [Journal Article] Pathological alpha-synuclein propagates through neural networks2014

    • Author(s)
      Masuda-Suzukake M, Nonaka T, Hosokawa M, Kubo M, Shimozawa A, Akiyama H, Hasegawa M
    • Journal Title

      Acta Neuropathol Commun

      Volume: 2 Issue: 1 Pages: 88-88

    • DOI

      10.1186/preaccept-1296467154135944

    • Related Report
      2014 Annual Research Report
    • Peer Reviewed / Open Access
  • [Journal Article] Concomitant accumulation of α-synuclein and TDP-43 in a patient with corticobasal degeneration.2014

    • Author(s)
      Yamashita S, Sakashita N, Yamashita T, Tawara N, Tasaki M, Kawakami K, Komohara Y, Fujiwara Y, Kamikawa M, Nakagawa T, Hirano T, Maeda Y, Hasegawa M, Takeya M, Ando Y
    • Journal Title

      Journal of Neurology

      Volume: 261 Issue: 11 Pages: 2209-2217

    • DOI

      10.1007/s00415-014-7491-8

    • Related Report
      2014 Annual Research Report
    • Peer Reviewed / Open Access
  • [Journal Article] Tau accumulation in the nucleus accumbens in tangle-predominant dementia2014

    • Author(s)
      Kawakami I, Hasegawa M, Arai T, Ikeda K, Oshima K, Niizato K, Aoki N, Katsuse O, Higashi S, Hosokawa M, Hirayasu Y, Akiyama H
    • Journal Title

      Acta Neuropathol

      Volume: Commun 2 Issue: 1 Pages: 40-40

    • DOI

      10.1186/2051-5960-2-40

    • Related Report
      2014 Annual Research Report
    • Peer Reviewed / Open Access
  • [Journal Article] Brain distribution of dipeptide repeat proteins in frontotemporal lobar degeneration and motor neurone disease associated with expansions in C9ORF72.2014

    • Author(s)
      Davidson YS, Barker H, Robinson AC, Thompson JC, Harris J, Troakes C, Smith B, Al-Saraj S, Shaw C, Rollinson S, Masuda-Suzukake M, Hasegawa M, Pickering-Brown S, Snowden JS, Mann DM.
    • Journal Title

      Acta Neuropathol Commun

      Volume: 2 Issue: 1 Pages: 70-70

    • DOI

      10.1186/2051-5960-2-70

    • 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
  • [Journal Article] Cyclin-dependent kinase 5 phosphorylates and induces the degradation of ataxin-22014

    • Author(s)
      Asada, A., Yamazaki, R., Kino, Y., Saito, T., Kimura, T., Miyake, M., Hasegawa, M., Nukina, N. & Hisanaga, S.
    • Journal Title

      Neurosci. Lett.

      Volume: 563 Pages: 112-117

    • DOI

      10.1016/j.neulet.2014.01.046

    • Related Report
      2014 Annual Research Report
    • Peer Reviewed
  • [Journal Article] Evaluating Prion Models Based on Comprehensive Mutation Data of Mouse PrP.2014

    • Author(s)
      Shirai T, Saito M, Kobayashi A, Asano M, Hizume M, Ikeda S, Teruya K, Morita M, Kitamoto T.
    • Journal Title

      Structure.

      Volume: 22 Issue: 4 Pages: 560-571

    • DOI

      10.1016/j.str.2013.12.019

    • Related Report
      2014 Annual Research Report
    • Peer Reviewed
  • [Journal Article] Creutzfeldt-Jakob disease with a codon 210 mutation: first pathological observation in a Japanese patient.2014

    • Author(s)
      Tajima Y, Satoh C, Mito Y, Kitamoto T
    • Journal Title

      Intern Med

      Volume: 53 Pages: 483-7

    • NAID

      130003392674

    • Related Report
      2014 Annual Research Report
    • Peer Reviewed
  • [Journal Article] Distinct origins of dura mater graft-associated Creutzfeldt-Jakob disease: past and future problems.2014

    • Author(s)
      Kobayashi A, Matsuura Y, Mohri S, Kitamoto T.
    • Journal Title

      Acta Neuropathol Commun.

      Volume: 2 Issue: 1 Pages: 32-32

    • DOI

      10.1186/2051-5960-2-32

    • Related Report
      2014 Annual Research Report
    • Peer Reviewed / Open Access / Acknowledgement Compliant
  • [Journal Article] Clinical features of genetic Creutzfeldt-Jakob disease with V180I mutation in the prion protein gene.2014

    • Author(s)
      Qina, T., N. Sanjo, M. Hizume, M. Higuma, M. Tomita, R. Atarashi, K. Satoh, I. Nozaki, T. Hamaguchi, Y. Nakamura, A. Kobayashi, T. Kitamoto, S. Murayama, H. Murai, M. Yamada and H. Mizusawa
    • Journal Title

      BMJ Open

      Volume: 4 Issue: 5 Pages: e004968-e004968

    • DOI

      10.1136/bmjopen-2014-004968

    • NAID

      120006986552

    • Related Report
      2014 Annual Research Report
    • Peer Reviewed / Open Access
  • [Journal Article] Gerstmann-Straeussler-Scheinker disease with P102L prion protein gene mutation presenting with rapidly progressive clinical course.2014

    • Author(s)
      Iwasaki Y, Mori K, Ito M, Nokura K, Tatsumi S, Mimuro M, Kitamoto T, Yoshida M.
    • Journal Title

      Clin Neuropathol

      Volume: 33(5) Issue: 09 Pages: 344-53

    • DOI

      10.5414/np300733

    • Related Report
      2014 Annual Research Report
    • Peer Reviewed
  • [Presentation] αSynuclein in Parkinson’s disease and related disorders.2018

    • Author(s)
      Hasegawa M
    • Organizer
      WCP2018 KYOTO (18th World Congress of Basic and Clinical Pharmacology), Kyoto. 2018. 7. 5.
    • Related Report
      2018 Annual Research Report
    • Int'l Joint Research / Invited
  • [Presentation] α-Synuclein Propagation in Parkinson’s Disease and Multiple System Atrophy.2018

    • Author(s)
      Hasegawa M
    • Organizer
      Basic Science Summer School MDS/Education/Conferences & Courses. 2018. 8. 3. Osaka.
    • Related Report
      2018 Annual Research Report
    • Int'l Joint Research / Invited
  • [Presentation] Prion-like propagation of pathological tau in neurodegenerative diseases.2018

    • Author(s)
      Hasegawa M
    • Organizer
      ICN2018 2018. 9.24, Tokyo.
    • Related Report
      2018 Annual Research Report
    • Int'l Joint Research / Invited
  • [Presentation] Prion-like propagation of pathological tau in neurodegenerative diseases.2018

    • Author(s)
      Hasegawa M
    • Organizer
      Japan-UK Neuroscience Symposium, 2019. 2. 10, Chiba.
    • Related Report
      2018 Annual Research Report
    • Int'l Joint Research / Invited
  • [Presentation] タンパク質の伝播から考える神経変性疾患治療の可能性2015

    • Author(s)
      長谷川成人
    • Organizer
      第33回 日本神経治療学会総会
    • Place of Presentation
      名古屋国際会議場(愛知県名古屋市)
    • Year and Date
      2015-11-28
    • Related Report
      2015 Annual Research Report
    • Invited
  • [Presentation] 神経変性疾患における異常タンパク質の伝播.2015

    • Author(s)
      長谷川成人
    • Organizer
      第20回日本神経感染症学会
    • Place of Presentation
      ホテルメルパルク長野(長野県長野市)
    • Year and Date
      2015-10-23
    • Related Report
      2015 Annual Research Report
    • Invited
  • [Presentation] タウオパチーの生化学.2015

    • Author(s)
      長谷川成人
    • Organizer
      第9回パーキンソン病・運動障害疾患コングレス
    • Place of Presentation
      品川プリンスホテル(東京都品川区)
    • Year and Date
      2015-10-16
    • Related Report
      2015 Annual Research Report
    • Invited
  • [Presentation] 脳タンパク質老化と認知症制御2015

    • Author(s)
      長谷川成人
    • Organizer
      第15回日本蛋白質科学会年会
    • Place of Presentation
      あわぎんホール(徳島県徳島市)
    • Year and Date
      2015-06-26
    • Related Report
      2015 Annual Research Report
    • Invited
  • [Presentation] シンポジウム16「精神医学における各種仮説を問う」アルツハイマー病のタウ仮説.2015

    • Author(s)
      長谷川成人
    • Organizer
      第111回日本精神神経学会学術総会
    • Place of Presentation
      大阪国際会議場(大阪府大阪市)
    • Year and Date
      2015-06-05
    • Related Report
      2015 Annual Research Report
    • Invited
  • [Presentation] 神経変性疾患の病態進行とプリオン仮説2014

    • Author(s)
      長谷川成人
    • Organizer
      川棚神経科学の会, 特別講演3
    • Place of Presentation
      長崎川棚医療センター(長崎県・川棚町)
    • Year and Date
      2014-12-06
    • Related Report
      2014 Annual Research Report
    • Invited
  • [Presentation] 「タウは認知症治療標的として魅力的か?」2014

    • Author(s)
      長谷川成人
    • Organizer
      第33回日本認知症学会学術集会シンポジウム
    • Place of Presentation
      パシフィコ横浜(神奈川県・横浜市)
    • Year and Date
      2014-11-30
    • Related Report
      2014 Annual Research Report
    • Invited
  • [Presentation] Prion-like properties of pathological proteins from diseased brains.2014

    • Author(s)
      Hasegawa M
    • Organizer
      Brain Conference 2014, Symposium 4「Pathological propagation in neurodegenerative diseases」
    • Place of Presentation
      Seoul, Korea
    • Year and Date
      2014-11-06
    • Related Report
      2014 Annual Research Report
    • Invited
  • [Presentation] αシヌクレイン.2014

    • Author(s)
      長谷川成人
    • Organizer
      第8回パーキンソン病・運動障害疾患コングレス2014
    • Place of Presentation
      京都オークラホテル(京都府・京都市)
    • Year and Date
      2014-10-04
    • Related Report
      2014 Annual Research Report
    • Invited
  • [Presentation] Prion-like features in alpha-synucleinopathies.2014

    • Author(s)
      Hasegawa M
    • Organizer
      XVIIIth International Congress of Neuropathology 2014. W3: Prion-like mechanisms in neurodegenerative disorders.
    • Place of Presentation
      Rio de Janeiro, Brazil
    • Year and Date
      2014-09-15
    • Related Report
      2014 Annual Research Report
    • Invited
  • [Presentation] Pathological alpha-synuclein spreading explains the disease progression.2014

    • Author(s)
      Hasegawa M
    • Organizer
      Neuroscience 2014. Joint Symposium of the Japan Neuroscience Society and the Korean Society of Brain and Neuroscience: Molecular Mechanisms underlying Parkinson Disease.
    • Place of Presentation
      パシフィコ横浜(神奈川県・横浜市)
    • Year and Date
      2014-09-12
    • Related Report
      2014 Annual Research Report
    • Invited
  • [Presentation] 神経変性疾患における細胞内異常タンパク質の伝播.2014

    • Author(s)
      長谷川成人
    • Organizer
      第2回 アミロイドーシス研究会 学術集会
    • Place of Presentation
      KKRホテル東京(東京都・千代田区)
    • Year and Date
      2014-08-22
    • Related Report
      2014 Annual Research Report
    • Invited
  • [Remarks] 認知症の発症と進行機序の解明

    • URL

      http://www.igakuken.or.jp/project/detail/dementia.html

    • Related Report
      2018 Annual Research Report
  • [Remarks] 認知症プロジェクト

    • URL

      http://www.igakuken.or.jp/dementia/

    • Related Report
      2018 Annual Research Report
  • [Remarks] パーキンソン病やレビー小体型認知症のマーモセットモデルの開発に成功

    • URL

      http://www.igakuken.or.jp/topics/2017/0202.html

    • Related Report
      2016 Annual Research Report
  • [Remarks] リソソームにおけるプログラニュリンの機能的役割を解明

    • URL

      http://www.igakuken.or.jp/topics/2017/0110.html

    • Related Report
      2016 Annual Research Report
  • [Remarks] ALS患者脳中に異常蓄積したTDP-43を質量分析器を使いタンパク質化学的に解析した。

    • URL

      http://www.igakuken.or.jp/topics/2016/0316.html

    • Related Report
      2016 Annual Research Report
  • [Remarks] 「Acta Neuropathologica」に研究成果が発表されました。

    • URL

      http://www.igakuken.or.jp/topics/2015/0414.html

    • Related Report
      2014 Annual Research Report
  • [Remarks] 「Human Molecular Genetics」に研究成果が発表されました。

    • URL

      http://www.igakuken.or.jp/topics/2014/0405.html

    • Related Report
      2014 Annual Research Report

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Published: 2014-11-20   Modified: 2022-07-19  

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