Establishment of a diagnosis of hereditary prion disease and the development of new classification and analysis

• Project/Area Number: 24591268
• Research Category: Grant-in-Aid for Scientific Research (C)
• Allocation Type: Multi-year Fund
• Section: 一般
• Research Field: Neurology
• Research Institution: Nagasaki University
• Principal Investigator: SATOH Katsuya 長崎大学, 医歯薬学総合研究科(医学系), 准教授 (70398147)
• Project Period (FY): 2012-04-01 – 2015-03-31
• Project Status: Completed (Fiscal Year 2014)
• Budget Amount: ¥5,460,000 (Direct Cost: ¥4,200,000、Indirect Cost: ¥1,260,000); Fiscal Year 2014: ¥1,560,000 (Direct Cost: ¥1,200,000、Indirect Cost: ¥360,000); 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: プリオン病 / 遺伝性 / 遺伝性プリオン病 / 臨床症状 / 髄液検査 / 髄液

Outline of Final Research Achievements

We succeed to amplify a very small amount of abnormal prion protein in in vitro as an epoch-making method of CSF as a diagnostic stool in human prion disease and reported the Real-time QUIC method and got a high evaluation. I collected CSF samples in cooperation with prion disease surveillance Committee.
I analyzed the specimen of all country prion disease cases using the RT-QUIC method which we developed and elevated on biochemical markers which I reported conventionally and aimed at the establishment of the diagnostic method of genetic prion disease and was able to succeed. Furthermore, I aimed at the development of a new therrpy for genetic prion disease disease and discovered the drug that it was with a therapeutic drug candidate now. I am screening it.

Research Products

• [Journal Article] Creutzfeldt-Jakob disease with a prion protein gene codon 180 mutation presenting asymmetric cortical high-intensity on magnetic resonance imaging. (2015)
  • Author(s): Amano Y, Kimura N, Hanaoka T, Aso Y, Hirano T, Murai H, Satoh K, Matsubara E.
  • Journal Title: Prion Volume: 2 Pages: 343-343
  • Peer Reviewed / Open Access
• [Journal Article] Persistent prion infection disturbs the function of Oct-1, resulting in the down-regulation of murine interferon regulatory factor-3. (2014)
  • Author(s): Homma T, Ishibashi D, Nakagaki T, Fuse T, Sano K, Satoh K, Atarashi R, Nishida N.
  • Journal Title: Sci Rep Volume: 4 Issue: 1 Pages: 6006-6006
  • DOI: 10.1038/srep06006
  • NAID: 120006986450
  • Peer Reviewed / Open Access
• [Journal Article] Conformational properties of prion strains can be transmitted to recombinant prion protein fibrils in real-time quaking-induced conversion. (2014)
  • Author(s): Sano K, Atarashi R, Ishibashi D, Nakagaki T, Satoh K, Nishida N.
  • Journal Title: J Virol Volume: 88(20) Issue: 20 Pages: 11791-11801
  • DOI: 10.1128/jvi.00585-14
  • NAID: 120006986372
  • Peer Reviewed / Open Access
• [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
  • Peer Reviewed / Open Access
• [Journal Article] Increased expression of p62/SQSTM1 in prion diseases and its association with (2014)
  • Author(s): Homma T, Ishibashi D, Nakagaki T, Satoh K, Sano K, Atarashi R, Nishida N.
  • Journal Title: Sci Rep. Volume: 4 Issue: 1 Pages: 4504-4504
  • DOI: 10.1038/srep04504
  • Peer Reviewed / Open Access
• [Journal Article] Increased expression of p62/SQSTM1 in prion diseases and its association with pathogenic prion protein. (2014)
  • Author(s): Homma T, Ishibashi D, Nakagaki T, Satoh K, Sano K, Atarashi R, Nishida N.
  • Journal Title: Sci Rep Volume: 4 Pages: 4504-4504
  • 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）
  • Peer Reviewed
• [Journal Article] FK506 reduces abnormal prion protein through the activation of autolysosomal degradation and prolongs survival in prion-infected mice. (2013)
  • Author(s): Nakagaki T, Satoh K, Ishibashi D, Fuse T, Sano K, Kamatari YO, Kuwata K, Shigematsu K, Iwamaru Y, Takenouchi T, Kitani H, Nishida N, Atarashi R.
  • Journal Title: Autophagy Volume: 9(9) Pages: 1386-1394
  • Peer Reviewed
• [Journal Article] Efficacy of prosultiamine treatment in patients with human T lymphotropic virus type I-associated myelopathy/tropical spastic paraparesis: results from an open-label clinical trial. (2013)
  • Author(s): Nakamura T, Matsuo T, Fukuda T, Yamato S, Yamaguchi K, Kinoshita I, Matsuzaki T, Nishiura Y, Nagasato K, Narita-Masuda T, Nakamura H, Satoh K, Sasaki H, Sakai H, Kawakami A
  • Journal Title: BMC Med Volume: １１ Pages: 182-182
  • Peer Reviewed
• [Journal Article] Early Detection of Abnormal Prion Protein in Genetic Human Prion Diseases Now Possible Using Real-Time QUIC Assay. (2013)
  • Author(s): Sano K, Satoh K, Atarashi R, Takashima H, Iwasaki Y, Yoshida M, Sanjo N, Murai H, Mizuswawa H, Schmitz M, Zerr I, Kim Y, Nishida N
  • Journal Title: PLoS One Volume: 8(1)
  • Peer Reviewed
• [Journal Article] Intra-arterial cell transplantation provides timing-dependent cell distribution and functional recovery after stroke. (2013)
  • Author(s): Ishizaka S, Horie N, Satoh K, Fukuda Y, Nishida N, Nagata I.
  • Journal Title: Stroke Volume: Mar;44(3) Pages: 720-6
  • Peer Reviewed
• [Journal Article] Rapidly progressive Alzheimer's disease: a multicenter update. (2012)
  • Author(s): Schmidt C, Haïk S, Satoh K, Rábano A, Martinez-Martin P, Roeber S, Brandel JP, Calero-Lara M, de Pedro-Cuesta J, Laplanche JL, Hauw JJ, Kretzschmar H, Zerr I.
  • Journal Title: J Alzheimers Dis Volume: 30(4) Pages: 751-756
  • Peer Reviewed
• [Journal Article] Protective role of interferon regulatory factor 3-mediated signaling against prion infection. (2012)
  • Author(s): Ishibashi D, Atarashi R, Fuse T, Nakagaki T, Yamaguchi N, Satoh K, Honda K, Nishida N.
  • Journal Title: J Virol Volume: 86(9) Pages: 4947-55
  • Peer Reviewed
• [Presentation] 髄液によるプリオン病の診断と鑑別診断 (2013)
  • Author(s): 佐藤克也
  • Organizer: 第54回 日本神経学会学術大会
  • Place of Presentation: 東京(東京フォーラム）
  • Invited
• [Presentation] Sensitivity and specificity of RT-QuiC and novel biomarkers (14-3-3 protein and total tau protein) in CSF of patients with sporadic CJD. (2013)
  • Author(s): Satoh K
  • Organizer: JPND
  • Place of Presentation: スペイン（バルセロナ）
  • Invited
• [Presentation] EARLY DETECTION OF ABNORMAL PRION PROTEIN IN GENETIC HUMAN PRION DISEASES NOW POSSIBLE USING REAL-TIME QUIC(RT-QUIC) ASSAY. (2013)
  • Author(s): Satoh K.
  • Organizer: WORLD CONGRESS OF NEUROLOGY
  • Place of Presentation: オーストラリア（ウィーン）
• [Presentation] プリオン病の新しい診断法 (2013)
  • Author(s): 佐藤克也
  • Organizer: 第32回日本認知症学会
  • Place of Presentation: 長野（キッセイ文化ホール、松本市総合体育館）
  • Invited
• [Presentation] Sensitivity and specificity of RT-QuIC (2012)
  • Author(s): Katsuya Satoh、
  • Organizer: JPND research
  • Place of Presentation: ハノーバー（ドイツ）
  • Invited
• [Presentation] 遺伝性プリオン病患者における髄液中のバイオマーカーの検討 (2012)
  • Author(s): 佐藤 克也
  • Organizer: 第53回日本神経学会学術大会
  • Place of Presentation: 東京
• [Presentation] ヒトプリオン病患者における髄液中の生化学的バイオマーカーと異常プリオン蛋白の検出について (2012)
  • Author(s): 佐藤 克也
  • Organizer: 日本神経感染症学会総会学術集会
  • Place of Presentation: 京都
  • Invited
• [Book] 感染症事典 (2012)
  • Author(s): ウイルス性髄膜炎
  • Publisher: オーム出版
• [Book] 感染症事典 (2012)
  • Author(s): 急性脳炎
  • Publisher: オーム出版
• [Book] 感染症事典 (2012)
  • Author(s): 性器ヘルペス
  • Publisher: オーム出版
• [Book] 感染症事典 (2012)
  • Author(s): クール―
  • Publisher: オーム出版
• [Book] 感染症事典 (2012)
  • Author(s): クロイツフェルト・ヤコブ病
  • Publisher: オーム出版