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Study to establish the new classification of thalamic Creutzfeldt-Jakob disease

Research Project

Project/Area Number 19K22588
Research Category

Grant-in-Aid for Challenging Research (Exploratory)

Allocation TypeMulti-year Fund
Review Section Medium-sized Section 52:General internal medicine and related fields
Research InstitutionTohoku University

Principal Investigator

Kitamoto Tetsuyuki  東北大学, 医学系研究科, 教授 (20192560)

Project Period (FY) 2019-06-28 – 2021-03-31
Project Status Completed (Fiscal Year 2020)
Budget Amount *help
¥6,500,000 (Direct Cost: ¥5,000,000、Indirect Cost: ¥1,500,000)
Fiscal Year 2020: ¥3,250,000 (Direct Cost: ¥2,500,000、Indirect Cost: ¥750,000)
Fiscal Year 2019: ¥3,250,000 (Direct Cost: ¥2,500,000、Indirect Cost: ¥750,000)
Keywordsプリオン / 視床型CJD / FFI / 2つのプリオン / 新分類 / 視床型CJD / FFI
Outline of Research at the Start

視床型CJDは、従来から特有の感染性を示し、1つのプリオンと考えられてきた。我々は、視床型CJDの家族性と考えられている致死性家族性不眠症(FFI)のわが国での1つの家系で、全く異なる臨床像を呈し、ウエスタンでは区別できない親子例が存在することを報告した。最近、この2例の感染実験が終了し、全く異なる感染性を示す、つまり2つのプリオンが存在することを明らかとした。わが国の視床型CJDは、このFFI家系に見られた2つの病型のうち、それぞれ別々に持つもの、同時に2つの病型が混在するものに分かれることが病理解析で明らかとなった。本研究は、この感染性に基づいた新しい分類法を提案するものである。

Outline of Final Research Achievements

We have demonstrated that two phenotypes of patients with fatal familial insomnia mutation have a distinct infectivity each other. We also found these prions in patients with thalamic form of Creutzfeldt-Jakob diseases. We proposed to name a new prion as M2C(sv) which represents type 2 PrPres and spongiform encephalopathy (small vacuoles, sv) with codon 129 Met. A newly classified M2C(sv) prion has a distinct infectivity and should be distinguished from M2T prion.

Academic Significance and Societal Importance of the Research Achievements

従来、FFIの中には、不眠症を示す典型例と認知症で発病するCJDのような非典型例が混在することが知られていたが、我々は両者のプリオンそのものが異なることを世界で初めて証明した。プリオンが異なるとは、当然感染性も異なるので、それぞれのヒトへの感染力を個別に評価しなければならない。

Report

(3 results)
  • 2020 Annual Research Report   Final Research Report ( PDF )
  • 2019 Research-status Report
  • Research Products

    (23 results)

All 2021 2020 2019

All Journal Article (23 results) (of which Int'l Joint Research: 6 results,  Peer Reviewed: 23 results,  Open Access: 9 results)

  • [Journal Article] Detection of cutaneous prion protein deposits could help diagnose GPI‐anchorless prion disease with neuropathy2021

    • Author(s)
      Honda Hiroyuki、Matsuzono Kosuke、Satoh Kota、Fujisawa Masayoshi、Suzuki Satoshi O.、Furuyama Chiaki、Kitamoto Tetsuyuki、Fujimoto Shigeru、Abe Koji、Iwaki Toru
    • Journal Title

      European Journal of Neurology

      Volume: - Issue: 6 Pages: 2133-2137

    • DOI

      10.1111/ene.14720

    • Related Report
      2020 Annual Research Report
    • Peer Reviewed
  • [Journal Article] System degeneration in an MM1-type sporadic Creutzfeldt-Jakob disease case with an unusually prolonged akinetic mutism state2021

    • Author(s)
      Iwasaki Yasushi、Mori Keiko、Ito Masumi、Kawai Yoshinari、Akagi Akio、Riku Yuichi、Miyahara Hiroaki、Kobayashi Atsushi、Kitamoto Tetsuyuki、Yoshida Mari
    • Journal Title

      Prion

      Volume: 15 Issue: 1 Pages: 12-20

    • DOI

      10.1080/19336896.2020.1868931

    • Related Report
      2020 Annual Research Report
    • Peer Reviewed / Open Access
  • [Journal Article] Genetic Creutzfeldt-Jakob disease‐M232R with the cooccurrence of multiple prion strains, M1+ M2C + M2T: Report of an autopsy case2021

    • Author(s)
      Shintaku Masayuki、Nakamura Takeshi、Kaneda Daita、Shinde Akiyo、Kusaka Hirofumi、Takeuchi Atsuko、Kitamoto Tetsuyuki
    • Journal Title

      Neuropathology

      Volume: - Issue: 3 Pages: 40-56

    • DOI

      10.1111/neup.12722

    • Related Report
      2020 Annual Research Report
    • Peer Reviewed
  • [Journal Article] A case of M232R genetic Creutzfeldt-Jakob disease with Lewy bodies2020

    • Author(s)
      Akagi Akio、Iwasaki Yasushi、Hashimoto Rina、Aiba Ikuko、Inukai Akira、Mimuro Maya、Riku Yuichi、Miyahara Hiroaki、Kitamoto Tetsuyuki、Yoshida Mari
    • Journal Title

      Journal of the Neurological Sciences

      Volume: 409 Pages: 116605-116605

    • DOI

      10.1016/j.jns.2019.116605

    • Related Report
      2020 Annual Research Report 2019 Research-status Report
    • Peer Reviewed
  • [Journal Article] Eliminating transmissibility of bovine spongiform encephalopathy by dry-heat treatment2020

    • Author(s)
      Matsuura Yuichi、Ishikawa Yukiko、Murayama Yuichi、Yokoyama Takashi、Somerville Robert A.、Kitamoto Tetsuyuki、Mohri Shirou
    • Journal Title

      Journal of General Virology

      Volume: 101 Issue: 1 Pages: 136-142

    • DOI

      10.1099/jgv.0.001335

    • Related Report
      2020 Annual Research Report 2019 Research-status Report
    • Peer Reviewed / Int'l Joint Research
  • [Journal Article] Diffusion-weighted magnetic resonance imaging in dura mater graft-associated Creutzfeldt-Jakob disease2020

    • Author(s)
      Sakai Kenji、Hamaguchi Tsuyoshi、Sanjo Nobuo、Murai Hiroyuki、Iwasaki Yasushi、Hamano Tadanori、Honma Mari、Noguchi-Shinohara Moeko、Nozaki Ichiro、Nakamura Yosikazu、Kitamoto Tetsuyuki、Harada Masafumi、Mizusawa Hidehiro、Yamada Masahito
    • Journal Title

      Journal of the Neurological Sciences

      Volume: 418 Pages: 117094-117094

    • DOI

      10.1016/j.jns.2020.117094

    • Related Report
      2020 Annual Research Report
    • Peer Reviewed
  • [Journal Article] Prion Gene PRNP Y162X Truncation Mutation Can Induce a Refractory Esophageal Achalasia2020

    • Author(s)
      Matsuzono Kosuke、Kim Younhee、Honda Hiroyuki、Anan Yuhei、Tsunoda Masato、Amano Yusuke、Fukusima Noriyoshi、Iwaki Toru、Kitamoto Tetsuyuki、Fujimoto Shigeru
    • Journal Title

      American Journal of Gastroenterology

      Volume: Publish Ahead of Print Issue: 6 Pages: 1350-1351

    • DOI

      10.14309/ajg.0000000000001044

    • Related Report
      2020 Annual Research Report
    • Peer Reviewed / Open Access
  • [Journal Article] Clinicopathological findings of a long-term survivor of V180I genetic Creutzfeldt-Jakob disease2020

    • Author(s)
      Hayashi Yuichi、Iwasaki Yasushi、Waza Masahiro、Kato Shinei、Akagi Akio、Kimura Akio、Inuzuka Takashi、Satoh Katsuya、Kitamoto Tetsuyuki、Yoshida Mari、Shimohata Takayoshi
    • Journal Title

      Prion

      Volume: 14 Issue: 1 Pages: 109-117

    • DOI

      10.1080/19336896.2020.1739603

    • Related Report
      2020 Annual Research Report
    • Peer Reviewed / Open Access
  • [Journal Article] Characterization of Sporadic Creutzfeldt-Jakob Disease and History of Neurosurgery to Identify Potential Iatrogenic Cases2020

    • Author(s)
      Hamaguchi Tsuyoshi、Sakai Kenji、Kobayashi Atsushi、Kitamoto Tetsuyuki、Ae Ryusuke、Nakamura Yosikazu、Sanjo Nobuo、Arai Kimihito、Koide Mizuho、Katada Fumiaki、Harada Masafumi、Murai Hiroyuki、Murayama Shigeo、Tsukamoto Tadashi、Mizusawa Hidehiro、Yamada Masahito
    • Journal Title

      Emerging Infectious Diseases

      Volume: 26 Issue: 6 Pages: 1140-1146

    • DOI

      10.3201/eid2606.181969

    • Related Report
      2020 Annual Research Report
    • Peer Reviewed / Open Access
  • [Journal Article] Correlating diffusion-weighted MRI intensity with type 2 pathology in mixed MM-type sporadic Creutzfeldt-Jakob disease.2020

    • Author(s)
      Ikeda T, Iwasaki Y, Sakurai K, Akagi A, Riku Y, Mimuro M, Miyahara H, Kitamoto T, Matsukawa N, Yoshida M.
    • Journal Title

      J Neurol Sci.

      Volume: 408 Pages: 116515-116515

    • DOI

      10.1016/j.jns.2019.116515

    • Related Report
      2020 Annual Research Report 2019 Research-status Report
    • Peer Reviewed
  • [Journal Article] A patient with spastic paralysis finally diagnosed as V180I genetic Creutzfeldt-Jakob disease 9 years after onset2020

    • Author(s)
      Nomura Taichi、Iwata Ikuko、Naganuma Ryoji、Matsushima Masaaki、Satoh Katsuya、Kitamoto Tetsuyuki、Yabe Ichiro
    • Journal Title

      Prion

      Volume: 14 Issue: 1 Pages: 226-231

    • DOI

      10.1080/19336896.2020.1823179

    • Related Report
      2020 Annual Research Report
    • Peer Reviewed / Open Access
  • [Journal Article] MM2 cortical form of sporadic Creutzfeldt-Jakob disease without progressive dementia and akinetic mutism: A case deviating from current diagnostic criteria2020

    • Author(s)
      Takahashi-Iwata Ikuko、Yabe Ichiro、Kudo Akihiko、Eguchi Katsuya、Wakita Masahiro、Shirai Shinichi、Matsushima Masaaki、Toyoshima Takanobu、Chiba Susumu、Tanikawa Satoshi、Tanaka Shinya、Satoh Katsuya、Kitamoto Tetsuyuki、Sasaki Hidenao
    • Journal Title

      Journal of the Neurological Sciences

      Volume: 412 Pages: 116759-116759

    • DOI

      10.1016/j.jns.2020.116759

    • NAID

      120007036450

    • Related Report
      2020 Annual Research Report
    • Peer Reviewed / Open Access
  • [Journal Article] α2,3 linkage of sialic acid to a GPI anchor and an unpredicted GPI attachment site in human prion protein2020

    • Author(s)
      Kobayashi Atsushi、Hirata Tetsuya、Nishikaze Takashi、Ninomiya Akinori、Maki Yuta、Takada Yoko、Kitamoto Tetsuyuki、Kinoshita Taroh
    • Journal Title

      Journal of Biological Chemistry

      Volume: 295 Issue: 22 Pages: 7789-7798

    • DOI

      10.1074/jbc.ra120.013444

    • Related Report
      2020 Annual Research Report
    • Peer Reviewed / Open Access
  • [Journal Article] Co-existence of PrPD types 1 and 2 in sporadic Creutzfeldt-Jakob disease of the VV subgroup: phenotypic and prion protein characteristics2020

    • Author(s)
      Cali Ignazio、Puoti Gianfranco、Smucny Jason、Curtiss Paul Michael、Cracco Laura、Kitamoto Tetsuyuki、Occhipinti Rossana、Cohen Mark Lloyd、Appleby Brian Stephen、Gambetti Pierluigi
    • Journal Title

      Scientific Reports

      Volume: 10 Issue: 1 Pages: 1503-1503

    • DOI

      10.1038/s41598-020-58446-0

    • Related Report
      2020 Annual Research Report
    • Peer Reviewed / Int'l Joint Research
  • [Journal Article] Identification of intracerebral hemorrhage in the early-phase of MM1+2C-type sporadic Creutzfeldt-Jakob disease: A case report2020

    • Author(s)
      Akagi A, Iwasaki Y, Yamamoto A, Matsuura H, Ikeda T, Mimuro M, Riku Y, Miyahara H, Kitamoto T, Yoshida M.
    • Journal Title

      Neuropathology

      Volume: 40(4) Issue: 4 Pages: 399-406

    • DOI

      10.1111/neup.12658

    • Related Report
      2020 Annual Research Report
    • Peer Reviewed
  • [Journal Article] Autopsy case of MV2K‐type sporadic Creutzfeldt‐Jakob disease with spongiform changes of the cerebral cortex2019

    • Author(s)
      Iwasaki Yasushi、Hiraga Keita、Ito Shota、Ando Tetsuo、Akagi Akio、Riku Yuichi、Mimuro Maya、Miyahara Hiroaki、Kobayashi Atsushi、Kitamoto Tetsuyuki、Yoshida Mari
    • Journal Title

      Neuropathology

      Volume: 39 Issue: 6 Pages: 452-460

    • DOI

      10.1111/neup.12595

    • Related Report
      2019 Research-status Report
    • Peer Reviewed
  • [Journal Article] Age at onset in genetic prion disease and the design of preventive clinical trials2019

    • Author(s)
      Minikel Eric Vallabh、Kitamoto Tetsuyuki、Mead Simon、et al(32人,18番目)
    • Journal Title

      Neurology

      Volume: 93 Issue: 2

    • DOI

      10.1212/wnl.0000000000007745

    • Related Report
      2019 Research-status Report
    • Peer Reviewed / Int'l Joint Research
  • [Journal Article] Clinicopathological findings of an MM2-cortical-type sporadic Creutzfeldt-Jakob disease patient with cortical blindness during a course of glaucoma and age-related macular degeneration2019

    • Author(s)
      Hayashi Yuichi、Iwasaki Yasushi、Waza Masahiro、Shibata Hideaki、Akagi Akio、Kimura Akio、Inuzuka Takashi、Satoh Katsuya、Kitamoto Tetsuyuki、Yoshida Mari、Shimohata Takayoshi
    • Journal Title

      Prion

      Volume: 13 Issue: 1 Pages: 124-131

    • DOI

      10.1080/19336896.2019.1631680

    • Related Report
      2019 Research-status Report
    • Peer Reviewed / Open Access
  • [Journal Article] Autopsied case of sporadic Creutzfeldt-Jakob disease pathologically classified as MM1+2C-type2019

    • Author(s)
      Iwasaki Y, Kato H, Ando T, Akagi A, Mimuro M, Miyahara H, Kobayashi A, Kitamoto T, Yoshida M.
    • Journal Title

      Neuropathology

      Volume: 印刷中 Issue: 3 Pages: 240-247

    • DOI

      10.1111/neup.12557

    • Related Report
      2019 Research-status Report
    • Peer Reviewed
  • [Journal Article] The characterization of AD/PART co-pathology in CJD suggests independent pathogenic mechanisms and no cross-seeding between misfolded Aβ and prion proteins2019

    • Author(s)
      Rossi Marcello、Kai Hideaki、Baiardi Simone、Bartoletti-Stella Anna、Carla Benedetta、Zenesini Corrado、Capellari Sabina、Kitamoto Tetsuyuki、Parchi Piero
    • Journal Title

      Acta Neuropathologica Communications

      Volume: 7 Issue: 1 Pages: 53-53

    • DOI

      10.1186/s40478-019-0706-6

    • Related Report
      2019 Research-status Report
    • Peer Reviewed / Open Access / Int'l Joint Research
  • [Journal Article] A novel combination of prion strain co-occurrence in patients with sporadic Creutzfeldt-Jakob disease2019

    • Author(s)
      Kobayashi Atsushi、Iwasaki Yasushi、Takao Masaki、Saito Yuko、Iwaki Toru、Qi Zechen、Torimoto Ryouta、Shimazaki Taishi、Munesue Yoshiko、Isoda Norikazu、Sawa Hirofumi、Aoshima Keisuke、Kimura Takashi、Kondo Hinako、Mohri Shirou、Kitamoto Tetsuyuki
    • Journal Title

      The American Journal of Pathology

      Volume: 印刷中 Issue: 6 Pages: 1276-1283

    • DOI

      10.1016/j.ajpath.2019.02.012

    • NAID

      120006847766

    • Related Report
      2019 Research-status Report
    • Peer Reviewed
  • [Journal Article] Correction to: In Vitro Seeding Activity of Glycoform-Deficient Prions from Variably Protease-Sensitive Prionopathy and Familial CJD Associated with PrPV180I Mutation2019

    • Author(s)
      Wang Z, Yuan J, Shen P, Abskharon R, Lang Y, Dang J, Adornato A, Xu L, Chen J, Feng J, Moudjou M, Kitamoto T, Lee HG, Kim YS, Langeveld J, Appleby B, Ma J, Kong Q, Petersen RB, Zou WQ, Cui L
    • Journal Title

      Mol Neurobiol

      Volume: 56 Issue: 8 Pages: 5470-5470

    • DOI

      10.1007/s12035-019-1508-3

    • Related Report
      2019 Research-status Report
    • Peer Reviewed / Int'l Joint Research
  • [Journal Article] Ganglioside Synthase Knockout Reduces Prion Disease Incubation Time in Mouse Models2019

    • Author(s)
      Kobayashi Atsushi、Qi Zechen、Shimazaki Taishi、Munesue Yoshiko、Miyamoto Tomomi、Isoda Norikazu、Sawa Hirofumi、Aoshima Keisuke、Kimura Takashi、Mohri Shirou、Kitamoto Tetsuyuki、Yamashita Tadashi、Miyoshi Ichiro
    • Journal Title

      The American Journal of Pathology

      Volume: 189 Issue: 3 Pages: 677-686

    • DOI

      10.1016/j.ajpath.2018.11.009

    • NAID

      120006770947

    • Related Report
      2019 Research-status Report
    • Peer Reviewed / Int'l Joint Research

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Published: 2019-07-04   Modified: 2022-01-27  

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