new classification of sporadic CJD with MV2.

• Project/Area Number: 24650185
• Research Category: Grant-in-Aid for Challenging Exploratory Research
• Allocation Type: Single-year Grants
• Research Field: Nerve anatomy/Neuropathology
• Research Institution: Tohoku University
• Principal Investigator: KITAMOTO Tetsuyuki 東北大学, 医学(系)研究科(研究院), 教授 (20192560)
• Project Period (FY): 2012-04-01 – 2014-03-31
• Project Status: Completed (Fiscal Year 2013)
• Budget Amount: ¥3,900,000 (Direct Cost: ¥3,000,000、Indirect Cost: ¥900,000); Fiscal Year 2013: ¥1,560,000 (Direct Cost: ¥1,200,000、Indirect Cost: ¥360,000); Fiscal Year 2012: ¥2,340,000 (Direct Cost: ¥1,800,000、Indirect Cost: ¥540,000)
• Keywords: ﾌﾟﾘｵﾝ / MV2 / 感染性 / ﾉｯｸｲﾝﾏｳｽ / 新分類 / プリオン / クロイツフェルト・ヤコブ病 / 孤発性 / 感染実験 / ウエスタンブロット / ノックインマウス

Research Abstract

There are MV2 cases in sporadic Creuzfeldt-Jakob disease. We established the method to classify these MV2 cases into cases with V2 prions or M2 prions. In Western blot analysis, T-1 antibody revealed Mi PrPres molecule in cases with V2 prions, while this antibody did not detected in cases with M2 prions. In cases with V2 prions, the infectivity shows the similar pattern with VV2 prions. Therefore, we established the practical classification method of MV2 prions.

Research Products

• [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
  • Peer Reviewed
• [Journal Article] Molecular barriers to zoonotic transmission of prions (2014)
  • 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
  • Peer Reviewed
• [Journal Article] Panencephalopathic-type sporadic Creutzfeldt-Jakob disease with circumscribed spongy foci (2014)
  • Author(s): Iwasaki Y, Tatsumi S, Mimuro M, Mori K, Ito M, Kitamoto T, Yoshida M.
  • Journal Title: Clin Neuropathol Volume: Mar-Apr;33(2) Issue: 03 Pages: 160-4
  • DOI: 10.5414/np300684
  • Peer Reviewed
• [Journal Article] Graft-related disease progression in dura mater graft-associated Creutzfeldt-Jakob disease: a cross-sectional study (2013)
  • Author(s): Sakai K, Hamaguchi T, Noguchi-Shinohara M, Nozaki I, Takumi I, Sanjo N, Nakamura Y, Kitamoto T, Saito N, Mizusawa H, Yamada M
  • Journal Title: BMJ Open Volume: 3(8) Issue: 8 Pages: e003400-e003400
  • DOI: 10.1136/bmjopen-2013-003400
  • Peer Reviewed
• [Journal Article] Critical significance of the region between Helix 1 and 2 for efficient dominant-negative inhibition by conversion-incompetent prion protein (2013)
  • Author(s): Taguchi Y, Mistica AM, Kitamoto T, Schatzl HM
  • Journal Title: PLoS Pathog Volume: 9(6) Issue: 6 Pages: e1003466-e1003466
  • DOI: 10.1371/journal.ppat.1003466
  • Peer Reviewed
• [Journal Article] Insight into the frequent occurrence of dura mater graft-associated Creutzfeldt-Jakob disease in Japan (2013)
  • Author(s): Hamaguchi T, Sakai K, Noguchi-Shinohara M, Nozaki I, Takumi I, Sanjo N, Sadakane A, Nakamura Y, Kitamoto T, Saito N, Mizusawa H, Yamada M
  • Journal Title: J Neurol Neurosurg Psychiatry Volume: 84(10) Issue: 10 Pages: 1171-5
  • DOI: 10.1136/jnnp-2012-304850
  • Peer Reviewed
• [Journal Article] Relationships between Clinicopathological Features and Cerebrospinal Fluid Biomarkers in Japanese Patients with Genetic Prion Diseases (2013)
  • Author(s): Higuma M, Sanjo N, Satoh K, Shiga Y, Sakai K, Nozaki I, Hamaguchi T, Nakamura Y, Kitamoto T, Shirabe S, Murayama S, Yamada M, Tateishi J, Mizusawa H
  • Journal Title: PLoS One Volume: 8(3) Issue: 3 Pages: e60003-e60003
  • DOI: 10.1371/journal.pone.0060003
  • NAID: 120006985964
  • Peer Reviewed
• [Journal Article] Glycoform-selective prion formation in sporadic and familial forms of prion disease (2013)
  • Author(s): Xiao X, Yuan J, Haik S, Cali I, Zhan Y, Moudjou M, Li B, Laplanche JL, Laude H, Langeveld J, Gambetti P, Kitamoto T, Kong Q, Brandel JP, Cobb BA, Petersen RB, Zou WQ.
  • Journal Title: PLoS One Volume: 8(3) Issue: 3 Pages: e58786-e58786
  • DOI: 10.1371/journal.pone.0058786
  • Peer Reviewed
• [Journal Article] Quantitative analysis of wet-heat inactivation in bovine spongiform encephalopathy (2013)
  • Author(s): Matsuura Y, Ishikawa Y, Bo X, Murayama Y, Yokoyama T, Somerville RA, Kitamoto T, Mohri S.
  • Journal Title: Biochem Biophys Res Commun Volume: Mar 1;432(1)(Epub 2013 Jan 30) Issue: 1 Pages: 86-91
  • DOI: 10.1016/j.bbrc.2013.01.081
  • Peer Reviewed
• [Journal Article] An autopsied case of Creutzfeldt-Jakob disease with mutation in the prion protein gene codon 232 and type 1+2 prion protein (2013)
  • Author(s): Iwasaki Y, Yokoi F, Tatsumi S, Mimuro M, Iwai K, Kitamoto T, Yoshida M.
  • Journal Title: Neuropathology Volume: Jan 16;33(5) Issue: 5 Pages: 568-75
  • DOI: 10.1111/neup.12013
  • Peer Reviewed
• [Journal Article] Characterization of variant Creutzfeldt-Jakob disease prions in prion protein-humanized mice carrying distinct codon 129 genotypes (2013)
  • Author(s): Takeuchi A, Kobayashi A, Ironside JW, Mohri S, Kitamoto T
  • Journal Title: J Biol Chem Volume: 288(30) Issue: 30 Pages: 21659-66
  • DOI: 10.1074/jbc.m113.470328
  • Peer Reviewed
• [Journal Article] Deciphering the pathogenesis of sporadic Creutzfeldt-Jakob disease with codon 129 M/V and type 2 abnormal prion protein (2013)
  • Author(s): Kobayashi A, Iwasaki Y, Otsuka H, Yamada M, Yoshida M, Matsuura Y, Mohri S, Kitamoto T.
  • Journal Title: Acta Neuropathol Commun Volume: Nov 13;1(1) Issue: 1 Pages: 74-74
  • DOI: 10.1186/2051-5960-1-74
  • Peer Reviewed
• [Journal Article] Glycoform-selective prion formation in sporadic and familial forms of prion disease (2013)
  • Author(s): Xiao X, Yuan J, Haïk S, Cali I, Zhan Y, Moudjou M, Li B, Laplanche JL, Laude H, Langeveld J, Gambetti P, Kitamoto T, Kong Q, Brandel JP, Cobb BA, Petersen RB, Zou WQ
  • Journal Title: PLoS One Volume: -
  • Peer Reviewed
• [Journal Article] Creutzfeldt-Jakob disease with the M232R mutation in the prion protein gene in two cases showing different disease courses: a clinicopathological study (2012)
  • Author(s): Takeda N, Yokota O, Terada S, Haraguchi T, Nobukuni K, Mizuki R, Honda H, Yoshida H, Kishimoto Y, Oshima E, Ishizu H, Katsuya Satoh K, Kitamoto T, Ihara Y, Uchitomi Y.
  • Journal Title: Journal of the neurological Sciences Volume: 312 Issue: 1-2 Pages: 108-116
  • DOI: 10.1016/j.jns.2011.08.008
  • Peer Reviewed
• [Journal Article] Enhanced antigen retrieval of amyloid β immunohistochemistry: re-evaluation of amyloid β pathology in Alzheimer disease and its mouse model (2012)
  • Author(s): Kai H, Shin RW, Ogino K, Hatsuta H, Murayama S, Kitamoto T
  • Journal Title: J Histochem Cytochem Volume: 60 Pages: 761-769
  • Peer Reviewed
• [Journal Article] Creutzfeldt-Jakob disease with the M232R mutation in the prion protein gene in two cases showing different disease courses: A clinicopathological study (2012)
  • Author(s): Takeda N, Yokota O, Terada S, Haraguchi T, Nobukuni K, Mizuki R, Honda H, Yoshida H, Kishimoto Y, Oshima E, Ishizu H, Satoh K, Kitamoto T, Ihara Y, Uchitomi Y
  • Journal Title: J Neurol Sci Volume: 312 Pages: 108-116
  • Peer Reviewed