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Is thalamic CJD a sporadic form of fatal familial insomnia?

Research Project

Project/Area Number 26670435
Research Category

Grant-in-Aid for Challenging Exploratory Research

Allocation TypeMulti-year Fund
Research Field Neurology
Research InstitutionTohoku University

Principal Investigator

Kitamoto Tetsuyuki  東北大学, 医学(系)研究科(研究院), 教授 (20192560)

Project Period (FY) 2014-04-01 – 2016-03-31
Project Status Completed (Fiscal Year 2015)
Budget Amount *help
¥3,640,000 (Direct Cost: ¥2,800,000、Indirect Cost: ¥840,000)
Fiscal Year 2015: ¥1,430,000 (Direct Cost: ¥1,100,000、Indirect Cost: ¥330,000)
Fiscal Year 2014: ¥2,210,000 (Direct Cost: ¥1,700,000、Indirect Cost: ¥510,000)
Keywordsプリオン / FF1 / ノックインマウス / 感染実験 / FFI
Outline of Final Research Achievements

The purpose of this study is to clarify the same infectivity between thalamic form of Creutzfeldt-Jakob disease and fatal familial insomnia. For this purpose, we did the transmission experiments with humanized prion protein knock-in mouse. Thalamic form prions showed a unique infectivity, and this infectivity was quite different from the MM1, MV2, VV2, or MM2C prions. The result of Ki-178N mice transmission experiment, which is now on-going study, will lead to the conclusion of this study.
In addition to the transmission experiments, we were looking for the cases with MM1 and MM2T prions. We found 3 cases with MM1+2T showing neuronal loss of inferior olivary nucleus. We also have a planning to transmit these MM1+2T cases using humanized knock-in mouse.

Report

(3 results)
  • 2015 Annual Research Report   Final Research Report ( PDF )
  • 2014 Research-status Report
  • Research Products

    (20 results)

All 2016 2015 2014

All Journal Article (20 results) (of which Int'l Joint Research: 6 results,  Peer Reviewed: 20 results,  Open Access: 4 results,  Acknowledgement Compliant: 3 results)

  • [Journal Article] Efficient propagation of variant Creutzfeldt-Jakob disease prion protein using the cell-protein misfolding cyclic amplification technique with samples containing plasma and heparin.2016

    • Author(s)
      Oshita M, Yokoyama T, Takei Y, Takeuchi A, Ironside JW, Kitamoto T, Morita M.
    • Journal Title

      Transfusion.

      Volume: 56(1) Issue: 1 Pages: 223-30

    • DOI

      10.1111/trf.13279

    • Related Report
      2015 Annual Research Report
    • Peer Reviewed / Int'l Joint Research
  • [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] Distinctive properties of plaque-type dura mater graft-associated Creutzfeldt-Jakob disease in cell-protein misfolding cyclic amplification2016

    • Author(s)
      Atsuko Takeuchi, Atsushi Kobayashi, Piero Parchi, Masahito Yamada, Masanori Morita, Shusei Uno and Tetsuyuki Kitamoto
    • Journal Title

      Laboratory Investigation

      Volume: in press Issue: 5 Pages: 581-7

    • DOI

      10.1038/labinvest.2016.27

    • Related Report
      2015 Annual Research Report
    • Peer Reviewed / Int'l Joint Research / Acknowledgement Compliant
  • [Journal Article] Neuropathological and biochemical criteria to identify acquired Creutzfeldt-Jakob disease among presumed sporadic cases.2015

    • Author(s)
      Kobayashi A, Parchi P, Yamada M, Mohri S, Kitamoto T.
    • Journal Title

      Neuropathology.

      Volume: - Issue: 3 Pages: 305-310

    • DOI

      10.1111/neup.12270

    • NAID

      120006219897

    • Related Report
      2015 Annual Research Report
    • Peer Reviewed / Int'l Joint Research
  • [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] 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
      2015 Annual Research Report
    • Peer Reviewed
  • [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
    • Peer Reviewed / Int'l Joint Research
  • [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 Research-status Report
    • Peer Reviewed
  • [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
      2014 Research-status Report
    • Peer Reviewed / Acknowledgement Compliant
  • [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 Research-status Report
    • Peer Reviewed
  • [Journal Article] Molecular barriers to zoonotic transmission of prions2014

    • Author(s)
      Barria MA, Balachandran A, Morita M, Kitamoto T, Barron R, Manson J, Knight R, Ironside JW, Head MW.
    • Journal Title

      Emerg Infect Dis

      Volume: Jan;20(1) Issue: 1 Pages: 88-97

    • DOI

      10.3201/eid2001.130858

    • Related Report
      2014 Research-status Report
    • Peer Reviewed
  • [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 Research-status Report
    • Peer Reviewed / Open Access
  • [Journal Article] Comparison of the clinical course of Japanese MM1-type sporadic Creutzfeldt-Jakob disease between subacute spongiform encephalopathy and panencephalopathic-type.2014

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

      Clin Neurol Neurosurg.

      Volume: 121 Pages: 59-63

    • DOI

      10.1016/j.clineuro.2014.03.024

    • Related Report
      2014 Research-status Report
    • Peer Reviewed
  • [Journal Article] Relation between clinical findings and progression of cerebral cortical pathology in MM1-type sporadic Creutzfeldt-Jakob disease: Proposed staging of cerebral cortical pathology.2014

    • Author(s)
      Iwasaki Y, Tatsumi S, Mimuro M, Kitamoto T, Hashizume Y, Yoshida M
    • Journal Title

      J Neurol Sci.

      Volume: 341(1-2) Issue: 1-2 Pages: 97-104

    • DOI

      10.1016/j.jns.2014.04.011

    • Related Report
      2014 Research-status 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 Research-status Report
    • Peer Reviewed / Open Access / Acknowledgement Compliant
  • [Journal Article] Creutzfeldt-Jakob Disease with a Codon 210 Mutation: First Pathological Observation in a Japanese Patient2014

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

      Internal Medicine

      Volume: 53 Issue: 5 Pages: 483-487

    • DOI

      10.2169/internalmedicine.53.0916

    • NAID

      130003392674

    • ISSN
      0918-2918, 1349-7235
    • Related Report
      2014 Research-status Report
    • Peer Reviewed
  • [Journal Article] Extensive cortical spongiform changes with cerebellar small amyloid plaques: The clinicopathological case of MV2K+C subtype in Creutzfeldt-Jakob disease.2014

    • Author(s)
      Araki K, Nakano Y, Kobayashi A, Matsudaira T, Sugiura A, Takao M, Kitamoto T, Murayama S, Obi T
    • Journal Title

      Neuropathology

      Volume: 34(6) Issue: 6 Pages: 541-6

    • DOI

      10.1111/neup.12133

    • Related Report
      2014 Research-status Report
    • Peer Reviewed
  • [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 Research-status Report
    • Peer Reviewed

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Published: 2014-04-04   Modified: 2017-05-10  

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