Neuropathological investigation about prion protein deposition of the olfactory bulb and the olfactory mucosa in Creutzfeldt-Jakob disease

• Project/Area Number: 15K08369
• Research Category: Grant-in-Aid for Scientific Research (C)
• Allocation Type: Multi-year Fund
• Section: 一般
• Research Field: Human pathology
• Research Institution: Aichi Medical University
• Principal Investigator: Iwasaki Yasushi 愛知医科大学, 付置研究所, 准教授 (60378172)
• Project Period (FY): 2015-04-01 – 2018-03-31
• Project Status: Completed (Fiscal Year 2017)
• Budget Amount: ¥4,940,000 (Direct Cost: ¥3,800,000、Indirect Cost: ¥1,140,000); Fiscal Year 2017: ¥1,690,000 (Direct Cost: ¥1,300,000、Indirect Cost: ¥390,000); Fiscal Year 2016: ¥1,430,000 (Direct Cost: ¥1,100,000、Indirect Cost: ¥330,000); Fiscal Year 2015: ¥1,820,000 (Direct Cost: ¥1,400,000、Indirect Cost: ¥420,000)
• Keywords: クロイツフェルト・ヤコブ病 / プリオン病 / プリオン蛋白 / 免疫染色 / ウエスタンブロット / 嗅球 / 嗅粘膜 / 嗅索 / 神経病理 / 異常プリオン蛋白 / 病理解剖

Outline of Final Research Achievements

We examined pathologic involvement and prion protein (PrP) deposition of the olfactory bulb and the olfactory mucosa in 11 autopsied cases of MM1-type sporadic Creutzfeldt-Jakob disease (CJD). In the olfactory bulb, spongiform change was not so apparent and the number of neurons was preserved. PrP immunostaining revealed synaptic-type PrP deposition, particularly in the glomerulus, granular cell layer, mitral cell layer, and anterior olfactory nucleus. Consistent PrP deposition from the early disease stage to the prolonged disease stage was recognized. The olfactory mucosa also showed PrP immunoreactivity. These pathologic findings indicated the resistance of these regions to the progression of prion disease pathology. Western blot analysis of the olfactory bulb showed type 1 PrP band pattern in five examined cases.

Research Products

• [Journal Article] Pathological progression of genetic Creutzfeldt-Jakob disease with a PrP V180I mutation. (2018)
  • Author(s): Akagi A, Iwasaki Y, Mimuro M, Kitamoto T, Yamada M, Yoshida M.
  • Journal Title: Prion Volume: 12 Issue: 1 Pages: 54-62
  • DOI: 10.1080/19336896.2017.1414130
  • Peer Reviewed / Int'l Joint Research
• [Journal Article] Prion-Like Seeding of Misfolded α-Synuclein in the Brains of Dementia with Lewy Body Patients in RT-QUIC (2017)
  • Author(s): Sano Kazunori、Atarashi Ryuichiro、Satoh Katsuya、Ishibashi Daisuke、Nakagaki Takehiro、Iwasaki Yasushi、Yoshida Mari、Murayama Shigeo、Mishima Kenichi、Nishida Noriyuki
  • Journal Title: Molecular Neurobiology Volume: - Issue: 5 Pages: 3916-3930
  • DOI: 10.1007/s12035-017-0624-1
  • Peer Reviewed / Open Access
• [Journal Article] Creutzfeldt-Jakob disease (2017)
  • Author(s): Iwasaki Y
  • Journal Title: Neuropathology Volume: 37 Issue: 2 Pages: 174-188
  • DOI: 10.1111/neup.12355
  • NAID: 130005279092
  • Peer Reviewed / Acknowledgement Compliant
• [Journal Article] An autopsied case of MV2K + C-type sporadic Creutzfeldt-Jakob disease presenting with widespread cerebral cortical involvement and Kuru plaques. (2017)
  • Author(s): Iwasaki Y, Saito Y, Aiba I, Kobayashi A, Mimuro M, Kitamoto T, Yoshida M.
  • Journal Title: Neuropathology Volume: 37 Issue: 3 Pages: 241-248
  • DOI: 10.1111/neup.12350
  • Peer Reviewed
• [Journal Article] Gastrostomy in patients with prion disease. (2017)
  • Author(s): Iwasaki Y, Mori K, Ito M, Kawai Y, Hoshino K, Kawabata Y, Mimuro M, Yoshida M.
  • Journal Title: Prion Volume: 11 Issue: 3 Pages: 186-194
  • DOI: 10.1080/19336896.2017.1306164
  • Peer Reviewed
• [Journal Article] MM1-type sporadic Creutzfeldt-Jakob disease with 1-month total disease duration and early pathologic indicators (2017)
  • Author(s): Iwasaki Y, Kato H, Ando T, Mimuro M, Kitamoto T, Yoshida M
  • Journal Title: Neuropathology Volume: 印刷中 Issue: 5 Pages: 420-425
  • DOI: 10.1111/neup.12379
  • Peer Reviewed / Acknowledgement Compliant
• [Journal Article] An autopsy case of Creutzfeldt-Jakob disease with a prion protein gene codon 180 mutation presenting with pathological laughing and an exaggerated startle reaction. (2017)
  • Author(s): Iwasaki Y, Mori K, Ito M, Akagi A, Mimuro M, Kitamoto T, Yoshida M.
  • Journal Title: Neuropathology Volume: 37 Issue: 6 Pages: 575-581
  • DOI: 10.1111/neup.12399
  • Peer Reviewed
• [Journal Article] An autopsied case of MM1 + MM2-cortical with thalamic-type sporadic Creutzfeldt-Jakob disease presenting with hyperintensities on diffusion-weighted MRI before clinical onset. (2016)
  • Author(s): Iwasaki Y, Mori K, Ito M, Mimuro M, Kitamoto T, Yoshida M.
  • Journal Title: Neuropathology. Volume: 37(1) Issue: 1 Pages: 78-85
  • DOI: 10.1111/neup.12327
  • Peer Reviewed
• [Journal Article] プリオン病と自律神経障害 (2016)
  • Author(s): 岩崎 靖
  • Journal Title: 自律神経 Volume: 53 Pages: 152-157
  • Peer Reviewed / Acknowledgement Compliant
• [Journal Article] クロイツフェルト・ヤコブ病 (2016)
  • Author(s): 岩崎 靖
  • Journal Title: 難病と在宅ケア Volume: 22 Pages: 29-33
  • Acknowledgement Compliant
• [Journal Article] Creutzfeldt-Jakob病 (2016)
  • Author(s): 岩崎 靖
  • Journal Title: 神経治療 Volume: 33 Pages: 333-337
  • NAID: 130005279092
  • Acknowledgement Compliant
• [Journal Article] Significant association of cadaveric dura mater grafting with subpial Aβ deposition and meningeal amyloid angiopathy. (2016)
  • Author(s): Hamaguchi T, Taniguchi Y, Sakai K, Kitamoto T, Takao M, Murayama S, Iwasaki Y, Yoshida M, Shimizu H, Kakita A, Takahashi H, Suzuki H, Naiki H, Sanjo N, Mizusawa H, Yamada M.
  • Journal Title: Acta Neuropathol. Volume: 132(2) Issue: 2 Pages: 313-5
  • DOI: 10.1007/s00401-016-1588-3
  • Peer Reviewed / Open Access
• [Journal Article] An autopsy-verified case of FTLD-TDP type A with upper motor neuron-predominant motor neuron disease mimicking MM2-thalamic-type sporadic Creutzfeldt-Jakob disease (2016)
  • Author(s): Hayashi Y, Iwasaki Y, Takekoshi A, Yoshikura N, Asano T, Mimuro M, Kimura A, Satoh K, Kitamoto T, Yoshida M, Inuzuka T
  • Journal Title: Prion Volume: 10 Pages: 492-501
  • Peer Reviewed / Acknowledgement Compliant
• [Journal Article] Prion-Seeding Activity Is widely Distributed in Tissues of Sporadic Creutzfeldt-Jakob Disease Patients. (2016)
  • Author(s): Takatsuki H, Fuse T, Nakagaki T, Mori T, Mihara B, Takao M, Iwasaki Y, Yoshida M, Murayama S, Atarashi R, Nishida N, Satoh K.
  • Journal Title: EBioMedicine. Volume: 12 Pages: 150-155
  • DOI: 10.1016/j.ebiom.2016.08.033
  • NAID: 120006987618
  • Peer Reviewed / Open Access
• [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
  • 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
  • Peer Reviewed / Int'l Joint Research
• [Journal Article] Rapid and Quantitative Assay of Amyloid-Seeding Activity in Human Brains Affected with Prion Diseases. (2015)
  • Author(s): Takatsuki H, Satoh K, Sano K, Fuse T, Nakagaki T, Mori T, Ishibashi D, Mihara B, Takao M, Iwasaki Y, Yoshida M, Atarashi R, Nishida N.
  • Journal Title: PLoS One Volume: 10 Issue: 6 Pages: 0126930-0126930
  • DOI: 10.1371/journal.pone.0126930
  • NAID: 120006986998
  • Peer Reviewed / Open Access / Int'l Joint Research
• [Presentation] Neuropathological investigation of the olfactory bulb and olfactory tract in sporadic Creutzfeldt-Jakob disease. (2017)
  • Author(s): Iwasaki Y, Mimuro M, Yoshida M.
  • Organizer: PRION2017
  • Int'l Joint Research
• [Presentation] Clinicopathology of human prion diseas. (2017)
  • Author(s): Iwasaki Y.
  • Organizer: Asian Pacific Prion Symposium 2017
  • Int'l Joint Research / Invited
• [Presentation] Relationship between clinical findings and progression of cerebral cortical pathology in MM1-type sporadic Creutzfeldt-Jakob disease. (2017)
  • Author(s): Iwasaki Y, Mimuro M, Yoshida M.
  • Organizer: XXIII World Congress of Neurology
  • Int'l Joint Research
• [Presentation] 高度のアミロイドβ沈着を認めた，非プラーク型硬膜移植後クロイツフェルト・ヤコブ病の30歳台女性例 (2016)
  • Author(s): 岩崎 靖，今村一博，岩井克成，小林 靖，三室マヤ，吉田眞理
  • Organizer: 第44回臨床神経病理懇話会
  • Place of Presentation: 大阪
  • Year and Date: 2016-11-19
• [Presentation] プリオン病患者に対する胃瘻造設 (2016)
  • Author(s): 岩崎 靖，森 恵子，伊藤益美，川合圭成，三室マヤ，吉田眞理
  • Organizer: 第34回日本神経治療学会総会
  • Place of Presentation: 米子
  • Year and Date: 2016-11-04
• [Presentation] クロイツフェルト・ヤコブ病における嗅球病変についての神経病理学的検討 (2016)
  • Author(s): 岩崎 靖，三室マヤ，吉田眞理
  • Organizer: 第21回 日本神経感染症学会総会・学術大会
  • Place of Presentation: 金沢
  • Year and Date: 2016-10-22
• [Presentation] V180I遺伝性Creutzfeldt-Jakob病の病理学的検討 (2016)
  • Author(s): 赤木明生，三室マヤ，岩崎 靖，山田正仁，吉田眞理
  • Organizer: 第21回日本神経感染症学会総会・学術大会
  • Place of Presentation: 金沢
  • Year and Date: 2016-10-21
• [Presentation] V180I変異クロイツフェルト・ヤコブ病(CJD)の病理所見 (2016)
  • Author(s): 横井風兒，赤木明生，三室マヤ，岩崎 靖，吉田眞理
  • Organizer: 第57回日本神経病理学会総会学術研究会
  • Place of Presentation: 弘前
  • Year and Date: 2016-06-03
• [Presentation] 孤発性クロイツフェルト・ヤコブ病 (2016)
  • Author(s): 岩崎 靖
  • Organizer: 第57回日本神経病理学会総会学術研究会
  • Place of Presentation: 弘前
  • Year and Date: 2016-06-03
  • Invited
• [Presentation] An autopsy case of MM1-type sporadic Creutzfeldt-Jakob disease with 1-month total disease duration presenting with early disease pathology (2016)
  • Author(s): Iwasaki Y, Kato H, Ando T, Mimuro M, Yoshida M．
  • Organizer: PRION2016
  • Place of Presentation: 東京
  • Year and Date: 2016-05-11
  • Int'l Joint Research
• [Presentation] クロイツフェルト・ヤコブ病 (2015)
  • Author(s): 岩崎 靖
  • Organizer: 第33回日本神経治療学会総会
  • Place of Presentation: 名古屋
  • Year and Date: 2015-11-28
  • Invited
• [Presentation] プリオン病と自律神経障害 (2015)
  • Author(s): 岩崎 靖
  • Organizer: 第68回日本自律神経学会総会
  • Place of Presentation: 名古屋
  • Year and Date: 2015-10-30
  • Invited
• [Presentation] 全経過1ヶ月のMM1型孤発性Creutzfeldt-Jakob病の1剖検例 (2015)
  • Author(s): 岩崎 靖, 加藤博子, 赤木明生, 三室マヤ, 安藤哲朗, 吉田眞理
  • Organizer: 第56回日本神経病理学会学術大会
  • Place of Presentation: 福岡
  • Year and Date: 2015-06-05
• [Presentation] 第11回神経病理コアカリキュラム教育セミナー プリオン病 (2015)
  • Author(s): 岩崎 靖
  • Organizer: 第56回日本神経病理学会学術大会
  • Place of Presentation: 福岡
  • Year and Date: 2015-06-03
• [Presentation] V180I変異Creutzfeldt-Jakob 病の病理学的検討 (2015)
  • Author(s): 赤木明生, 三室マヤ, 岩崎 靖, 酒井素子, 吉田眞理
  • Organizer: 第56回日本神経病理学会学術大会
  • Place of Presentation: 福岡
  • Year and Date: 2015-06-03
• [Presentation] プリオン病患者に対する胃瘻造設 (2015)
  • Author(s): 岩崎 靖, 森 恵子, 伊藤益美, 赤木明生, 三室マヤ, 吉田眞理
  • Organizer: 第56回日本神経学会学術大会
  • Place of Presentation: 新潟
  • Year and Date: 2015-05-22
• [Presentation] Creutzfeldt-Jakob病では発症4ヶ月よりαB- Crystallin陽性神経細胞が出現する (2015)
  • Author(s): 赤木明生, 三室マヤ, 岩崎 靖, 吉田眞理
  • Organizer: 第56回日本神経学会学術大会
  • Place of Presentation: 新潟
  • Year and Date: 2015-05-22
• [Presentation] 当施設におけるプリオン病の剖検検索症例についての検討 (2015)
  • Author(s): 岩崎 靖, 赤木明生, 三室マヤ, 吉田眞理
  • Organizer: 第104回日本病理学会総会
  • Place of Presentation: 名古屋
  • Year and Date: 2015-05-01