Study of the pathomechanism in the prion infection

2008 Fiscal Year Final Research Report

• Project/Area Number: 18209031
• 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 Tohoku University, 大学院・医学系研究科, 教授 (20192560)
• Project Period (FY): 2006 – 2008
• Keywords: 遺伝子 / 感染症 / 神経科学 / 痴呆 / プリオン

Research Abstract

プリオン感染に関してその感染源を特定できる方法として、トレースバック現象を見いだし報告した。また、このトレースバック現象を利用して、従来感染源が不明であった硬膜移植後CJD with plaques(dCJD/PLと略す)の感染源が孤発性CJDのVV2であることを証明し、またMV2も感染源になりうることを学術論文として報告した。dCJD/PLの感染源を特定したのは世界初である。

Research Products

• [Journal Article] The number of octapeptide repeat affects the expression and conversion of prion protein (2009)
  • Author(s): Hiraga C, Kobayashi A, Kitamoto T
  • Journal Title: Biochem Biophys Res Commun (in press)
  • Peer Reviewed
• [Journal Article] Human PrP219K is converted to PrPSc but shows heterozygous inhibition in vCJD infection (2009)
  • Author(s): Hizume M, Kobayashi A, Teruya K, Ohashi H, Ironside JW, Mohri S, Kitamoto T
  • Journal Title: J Biol Chem 284 Pages: 3603-3609
  • Peer Reviewed
• [Journal Article] Yamada M Medical procedures and risk for sporadic Creutzfeldt-Jakob disease, Japan, 1999-2008 (2009)
  • Author(s): Hamaguchi T, Noguchi-Shinohara M, Nozaki I, Nakamura Y, Sato T, Kitamoto T, Mizusawa H
  • Journal Title: Emerg Infect Dis. 15 Pages: 265-271
  • Peer Reviewed
• [Journal Article] Heterozygous inhibition in prion infection The stone fence model (2009)
  • Author(s): Kobayashi A, Hizume M, Teruya K, Mohri S, Kitamoto T
  • Journal Title: Prion 3 Pages: 1-4
  • Peer Reviewed
• [Journal Article] Thr but Asn of the N-glycosylation sites of PrP is indispensable for its misfolding (2008)
  • Author(s): Ikeda s, kobayashi A, Kitamoto T
  • Journal Title: Biochem Biophys Res Commun 369(4) Pages: 1195-1198
  • Peer Reviewed
• [Journal Article] Clinicopathologic characteristics of five autopsied cases of dura mater-associated Creutzfeldt-Jakob disease (2008)
  • Author(s): Iwasaki Y, Mimuro M, Yoshida M, Hashizume Y, Kitamoto T, Sobue G
  • Journal Title: Neuropathology 28(1) Pages: 51-61
  • Peer Reviewed
• [Journal Article] MM1-type sporadic Creutzfeldt-Jakob disease with unusually prolonged disease duration presenting with panencephalopathic-type pathology (2008)
  • Author(s): Hoshino A, Iwasaki Y, Izumi M, Kimura S, Ibi T, Kitamoto T, Yoshida M, Hashizume Y, Sahashi K
  • Journal Title: Neuropathology 28(3) Pages: 326-332
  • Peer Reviewed
• [Journal Article] MM2-cortical-type sporadic Creutzfeldt-Jakob disease with early stage cerebral cortical pathology presenting with a rapidly progressive clinical course (2008)
  • Author(s): Niimi Y, Iwasaki Y, Umemura T, Tranaka F, Yoshida M, Hashizume Y, Kitamoto T, Hirayama M, Sobue G
  • Journal Title: Neuropathology 28(6) Pages: 645-651
  • Peer Reviewed
• [Journal Article] Plaque-type deposition of prion protein in the damaged white matter of sporadic Creutzfeldt-Jakob disease MM1 patients (2008)
  • Author(s): Kobayashi A, Arima K, Ogawa M, Murata M, Fukuda T, Kitamoto T
  • Journal Title: Acta Neuropathol 116(5) Pages: 561-566
  • Peer Reviewed
• [Journal Article] Thr but Asn of the N-glycosylation sites of PrP is dispensable for its misfolding (2008)
  • Author(s): Ikeda S, Kobayashi A, Kitamoto T
  • Journal Title: Biochem Biophys Res Commun 369 Pages: 1195-1198
  • Peer Reviewed
• [Journal Article] Two different clinical phenotypes of Creutzfeldt-Jakob disease with a M232R substitution (2007)
  • Author(s): Shiga Y, Satoh K, Kitamoto T, Kanno S, Nakashima I, Sato S, Fujihara K, Takata H, Nobukuni K, Kuroda S, Takano H, Umeda Y, Konno H, Nagasato K, Satoh A, Matsuda Y, Hidaka M, Takahashi H, Sano Y, Kim K, Konishi T, Doh-ura K, Sato T, Sasaki K, Nakamura Y, Yamada M, Mizusawa H, Itoyama Y
  • Journal Title: J Neurol 254(11) Pages: 1509-1517
  • Peer Reviewed
• [Journal Article] Enhanced Aquaporin-4 immunoreactivity in sporadic Creutzfeldt-Jakob disease (2007)
  • Author(s): Iwasaki Y, Mimuro M, Yoshida M, Hashizume Y, Ito M, Kitamoto T, Wakayama Y, Sobue G
  • Journal Title: Neuropathology 27 Pages: 314-323
  • Peer Reviewed
• [Journal Article] Cross-sequence transmission of sporadic Creutzfeldt-Jakob disease creates a new prion strain (2007)
  • Author(s): Kobayashi A, Asano M, Mohri S, Kitamoto T
  • Journal Title: J Biol Chem 282 Pages: 30022-30028
  • Peer Reviewed
• [Journal Article] Clinical features and diagnosis of dura mater graft associatedCreutzfeldt Jakob disease (2007)
  • Author(s): Noguchi-Shinohara M, Hamaguchi T, Kitamoto T, Sato T, Nakamura Y,Mizusawa H, Yamada M
  • Journal Title: Neurology 69(4) Pages: 360-367
  • Peer Reviewed
• [Journal Article] Amyloid precursor protein cytoplasmic domainwithphospho-Thr668accumulates inAlzheimer's disease and its transgenic models : a role tomediate interaction of Abeta and tau (2007)
  • Author(s): Shin RW, Ogino K, Shimabuku A, Taki T, Nakashima H, Ishihara T, KitamotoT
  • Journal Title: Acta Neuropathol(Berl) 113(6) Pages: 627-636
  • Peer Reviewed
• [Journal Article] Ophthalmic surgery in prion diseases (2007)
  • Author(s): Hamaguchi T, Noguchi-Shinohara M, Nakamura Y, Sato T, KitamotoT,Mizusawa H, Yamada M
  • Journal Title: Emerg Infect Dis 13(1) Pages: 162-164
  • Peer Reviewed
• [Journal Article] Autopsy case of sporadic Creutzfeldt-Jakob disease presenting with signs suggestive of brainstem and spinal cord involvement (2006)
  • Author(s): Iwasaki Y, Iijima M, Kimura S, Yoshida M, Hashizume Y, Yamada M, Kitamoto T, sobue G
  • Journal Title: Neuropathology 26(6) Pages: 550-556
  • Peer Reviewed
• [Journal Article] Clinicopathologic characteristics of sporadic Japanese Creutzfeldt-Jakob disease classified according to prion protein gene polymorphism and prion protein type (2006)
  • Author(s): Iwasaki Y, Yoshida M, Hashizume Y, Kitamoto T, Sobue
  • Journal Title: Acta Neuropathol(Berl) 112(5) Pages: 561-571
  • Peer Reviewed
• [Journal Article] MM2-thalamic-type sporadic Creutzfeldt-Jakob disease with widespread neocortical pathology (2006)
  • Author(s): Hirose K, Iwasaki Y, Izumi M, Yoshida M, Hashizume Y, Kitamoto T, Sahashi K
  • Journal Title: Acta Neuropathol(Berl) 112(4) Pages: 503-511
  • Peer Reviewed
• [Journal Article] Severe cortical involvement in MV2 Creutzfeldt-Jakob disease : an autopsy case report (2006)
  • Author(s): Ishihara K, Sugie M, Shiota J, Kawamura M, Kitamoto T, Nakano I
  • Journal Title: Neuropathology 26(5) Pages: 433-437
  • Peer Reviewed
• [Journal Article] The MM2-cortical form of sporadic Creutzfeldt-Jakob disease presenting with visual disturbance (2006)
  • Author(s): Nozaki I, Hamaguchi T, Noguchi-Shimohara M, Ono k, Shirasaki H, Komai K, Kitamoto T, Yamada M
  • Journal Title: Neurology 67(3) Pages: 531-3
  • Peer Reviewed
• [Journal Article] Autopsy case of Creutzfeldt-Jakob disease with Met/Val heterozygosity at codon 129 and type 1 protease-resistant prion protein presenting some florid-type plaques and many Kuru plaques in the cerebellum (2006)
  • Author(s): Kawauchi Y, Kamitani T, Yagishita S, Kitamoto T, Kishida H
  • Journal Title: Neuropathology 26(4) Pages: 313-317
  • Peer Reviewed