To study the molecular mechanism of prion protein conversion

2012 Fiscal Year Final Research Report

• Project/Area Number: 22249034
• Research Category: Grant-in-Aid for Scientific Research (A)
• Allocation Type: Single-year Grants
• Section: 一般
• Research Field: Neurology
• Research Institution: Tohoku University
• Principal Investigator: KITAMOTO Tetsuyuki 東北大学, 大学院・医学系研究科, 教授 (20192560)
• Project Period (FY): 2010 – 2012
• Keywords: 神経分子病態学

Research Abstract

Overexpression model for Bank vole prion protein (PrP) showed clinical signs of prion disease, spongiform changes and abnormal PrP accumulations in the brain. Therefore, this transgenic mice have a possibility as an animal model for spontaneous prion diseases. We examined the transmission studies with the wild mice and the knock-in mice having bank vole PrP (Ki-Bank) There were no successful transmissions from the transgenic mouse brains. Therefore, it is clear that the PrP conversion is imperfect in this transgenic model. However, it is coming out that this Ki-Bank is a useful animal model for the experimentaltransmission.

Research Products

• [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: (in press)
  • URL: http//www.ncbi.nlm.nih.gov/pu bmed/23595947.
  • Peer Reviewed
• [Journal Article] An autopsied case of Creutzfeldt-Jakob disease with mutation in the prion protein gene codon 232 and type 1+2prion protein (2013)
  • Author(s): Iwasaki Y, Yokoi F, Tatsumi S, Mimuro M, Iwai K, Kitamoto T, Yoshida M
  • Journal Title: Neuropathology Volume: (in press)
  • DOI: doi:10.1111/neup.12013.
  • 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) Pages: e60003
  • DOI: doi:10.1371/joumai.pone.0060003.
  • 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) Pages: e58786
  • DOI: doi:10.1371/joumal.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: 432(1) Pages: 86-91
  • DOI: doi:01.1016/j.bbrc.2013.01.081.
  • Peer Reviewed
• [Journal Article] Creutzfeldt-Jakob disease with the M232R mutation in the prion protein gene in two cases showing differentdisease courses: Aclinicopathological 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
  • Journal Title: J Neurol Sci Volume: 312 Pages: 108-
  • DOI: doi:10.1016/j.jns.2011.08.008.
  • Peer Reviewed
• [Journal Article] Co-occurrence of types 1 and 2 PrPres in sporadic Creutzfeldt-Jakob disease MM1 (2011)
  • Author(s): Kobayashi A, Mizukoshi K, Iwasaki Y, Miyata H, Yoshida Y, Kitamoto T
  • Journal Title: Am J Pathol Volume: 178 Pages: 1309-1315
  • DOI: doi:10.1016/j.ajpath.2010.11.069
  • Peer Reviewed
• [Journal Article] Deduction of the evaluation limit and termination timing ofmulti-round protein misfolding cyclic amplification from a titration curve (2011)
  • Author(s): Takeuchi A, Komiya M, Kitamoto T, Morita M
  • Journal Title: Microbiol Immunol Volume: 55(7) Pages: 502-509
  • DOI: doi:10.1111/j.1348-0421.2011.00340.x.
  • Peer Reviewed
• [Journal Article] Ultrasensitive human prion detection in cerebrospinal fluid by real-time quaking-induced conversion (2011)
  • Author(s): Atarashi R, Satoh K, Sano K, Fuse T, Yamaguchi N, Ishibashi D, Matsubara T, Nakagaki T, Yamanaka H, Shirabe S, Yamada M, Mizusawa H, Kitamoto T, Klug G, McGlade A, Collins SJ, Nishida N
  • Journal Title: Nat Med Volume: 17 Pages: 175-178
  • DOI: doi:10.1038/nm.2294.
  • Peer Reviewed
• [Journal Article] An autopsied case of V180I Creutzfeldt-Jakob disease presenting with panencephalopathic-type pathology and a characteristic prion protein type (2011)
  • Author(s): Iwasaki Y, Mori K, Ito M, Nagaoka M, Ieda T, Kitamoto T, Yoshida M, Hashizume Y
  • Journal Title: Neuropathology Volume: 31 Pages: 540-548
  • DOI: doi:10.1111/j.1440-1789.2010.01192x.
  • Peer Reviewed
• [Journal Article] Heparin enhances the cell-protein misfolding cyclic amplification efficiency of variant Creutzfeldt-Jakob disease (2011)
  • Author(s): Yokoyama T, Takeuchi A, Yamamoto M, Kitamoto T, Ironside JW, Morita M
  • Journal Title: Neurosci Lett Volume: 498 Pages: 119-123
  • DOI: doi:10.1016/j.neulet.2011.04.072.
  • Peer Reviewed
• [Journal Article] Slow-progressive ataxia with amethionine-to-arginine point mutation in codon 232 in the prion protein gene (PRNP) (2011)
  • Author(s): Nishimoto Y, Ito D, Suzuki S, Shimizu T, Kitamoto T, Suzuki N
  • Journal Title: Clin Neurol Neurosurg Volume: 113 Pages: 696-698
  • DOI: doi:10.1016/j.clineuro.2011.04.009.
  • Peer Reviewed
• [Journal Article] Serial diffusion-weighted MRI and SPECT findings in a Creutzfeldt-Jakob disease patient with V180I mutation (2011)
  • Author(s): Kono S, Manabe Y, Fujii D, Sakai Y, Narai H, Omori N, Kitamoto T, Abe K
  • Journal Title: J Neurol Sci Volume: 301 Pages: 100-103
  • DOI: doi:10.1016/j.jns.2010.10.032.
  • Peer Reviewed
• [Journal Article] Survival to akinetic mutism state in Japanese cases of MM1-type sporadic Creutzfeldt-Jakob disease is similar to Caucasians (2011)
  • Author(s): Iwasaki Y, Mimuro M, Yoshida M, Kitamoto T, Hashizume Y
  • Journal Title: Eur J Neurol Volume: 18 Pages: 999-1002
  • DOI: doi:10.1111/j.1468-1331.2010.03185.x
  • Peer Reviewed
• [Journal Article] An autopsy case of MM2-cortical+thalamic-type sporadic Creutzfeldt-Jakob disease (2011)
  • Author(s): Saito Y, Iwasaki Y, Aiba I, Kitamoto T, Yoshida M, Hashizume Y
  • Journal Title: Neuropathology Volume: 31 Pages: 523-530
  • DOI: doi:10.1111/j.1440-1789.2010.01181.x.
  • Peer Reviewed
• [Journal Article] Less protease-resistant PrP in a patient with sporadic CJD treated with intraventricular pentosan polysulphate (2010)
  • Author(s): Terada T, Tsuboi Y, Obi T, Doh-Ura K, Murayama S, Kitamoto T, Yamada T, Mizoguchi K
  • Journal Title: Acta Neurol Scand Volume: 121 Pages: 127-130
  • DOI: doi:10.1111/j.1600-0404.2009.01272.x.
  • Peer Reviewed
• [Journal Article] Experimental verification of a traceback phenomenon in prion infection (2010)
  • Author(s): Kobayashi A, Sakuma N, Matsuura Y, Mohri S, Aguzzi A, Kitamoto T
  • Journal Title: J. Virol Volume: 84 Pages: 3230-3238
  • DOI: doi:10.1128/JVI.02387-09.
  • Peer Reviewed
• [Journal Article] Amino acid conditions near the GPI anchor attachment site of prion protein for the conversion and the GPI anchoring (2010)
  • Author(s): Hizume M, Kobayashi A, Mizusawa H, Kitamoto T
  • Journal Title: Biochem. Biophys. Res. Commun Volume: 391(4) Pages: 1681-1686
  • DOI: doi:10.1016/j.bbrc.2009.12.128.
  • Peer Reviewed
• [Journal Article] Discordant clinicopathologic phenotypes in a Japanese kindred of fatal familial insomnia (2010)
  • Author(s): Saitoh Y, Ogawa M, Naito Y, Komatsuzaki Y, Tagaya H, Arima K, Tamaoka A, Kitamoto T, Murata M
  • Journal Title: Neurology Volume: 74 Pages: 86-89
  • DOI: doi:10.1212/WNL.0b013e3181c7da09.
  • Peer Reviewed
• [Journal Article] Age-dependent increase inlysosome-associated membrane protein1 and early-onset behavioral deficits in APPSL transgenic mouse model of Alzheimer's disease (2010)
  • Author(s): Hashimoto T, Ogino K, Shin RW, Kitamoto T, Kikuchi T, Shimizu N
  • Journal Title: Neurosci Lett Volume: 469 Pages: 273-277
  • DOI: doi:10.1016/j.neulet.2009.12.015.
  • Peer Reviewed
• [Journal Article] A novel anti-prion protein monoclonal antibody and its single-chain fragment variable derivative with ability to inhibit abnormal prion protein accumulation in cultured cells (2010)
  • Author(s): Shimizu Y, Kaku-Ushiki Y, Iwamaru Y, Muramoto T, Kitamoto T, Yokoyama T, Mohri S, Tagawa Y
  • Journal Title: Microbiol Immunol Volume: 54 Pages: 112-121
  • DOI: doi:10.1111/j.1348-0421.2009.00190.x.
  • Peer Reviewed
• [Journal Article] Prospective 10-year surveillance of human prion diseases in Japan (2010)
  • Author(s): Nozaki I, Hamaguchi T, Sanjo N, Noguchi-Shinohara M, Sakai K, Nakamura Y, Sato T, Kitamoto T, Mizusawa H, Moriwaka F, Shiga Y, Kuroiwa Y, Nishizawa M, Kuzuhara S, Inuzuka T, Takeda M, Kuroda S, Abe K, Murai H, Murayama S, Tateishi J, Takumi I, Shirabe S, Harada M, Sadakane A, Yamada M
  • Journal Title: Brain Volume: 133 Pages: 3043-3057
  • DOI: doi:10.1093/brain/awq216.
  • Peer Reviewed