Is thalamic CJD a sporadic form of fatal familial insomnia?
Project/Area Number |
26670435
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Research Category |
Grant-in-Aid for Challenging Exploratory Research
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Allocation Type | Multi-year Fund |
Research Field |
Neurology
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Research Institution | Tohoku University |
Principal Investigator |
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Project Period (FY) |
2014-04-01 – 2016-03-31
|
Project Status |
Completed (Fiscal Year 2015)
|
Budget Amount *help |
¥3,640,000 (Direct Cost: ¥2,800,000、Indirect Cost: ¥840,000)
Fiscal Year 2015: ¥1,430,000 (Direct Cost: ¥1,100,000、Indirect Cost: ¥330,000)
Fiscal Year 2014: ¥2,210,000 (Direct Cost: ¥1,700,000、Indirect Cost: ¥510,000)
|
Keywords | プリオン / FF1 / ノックインマウス / 感染実験 / FFI |
Outline of Final Research Achievements |
The purpose of this study is to clarify the same infectivity between thalamic form of Creutzfeldt-Jakob disease and fatal familial insomnia. For this purpose, we did the transmission experiments with humanized prion protein knock-in mouse. Thalamic form prions showed a unique infectivity, and this infectivity was quite different from the MM1, MV2, VV2, or MM2C prions. The result of Ki-178N mice transmission experiment, which is now on-going study, will lead to the conclusion of this study. In addition to the transmission experiments, we were looking for the cases with MM1 and MM2T prions. We found 3 cases with MM1+2T showing neuronal loss of inferior olivary nucleus. We also have a planning to transmit these MM1+2T cases using humanized knock-in mouse.
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Report
(3 results)
Research Products
(20 results)
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[Journal Article] Sporadic Creutzfeldt-Jakob Disease MM1+2C and MM1 are Identical in Transmission Properties.2016
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.
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Journal Title
Brain Pathol.
Volume: 26(1)
Pages: 95-101
DOI
Related Report
Peer Reviewed
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[Journal Article] Quantifying prion disease penetrance using large population control cohorts.2016
Author(s)
Minikel EV, Vallabh SM, Lek M, Estrada K, Samocha KE, Sathirapongsasuti JF, McLean CY, Tung JY, Yu LP, Gambetti P, Blevins J, Zhang S, Cohen Y, Chen W, Yamada M, Hamaguchi T, Sanjo N, Mizusawa H, Nakamura Y, Kitamoto T, et al.
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Journal Title
Sci Transl Med.
Volume: 8(322)
Pages: -
DOI
Related Report
Peer Reviewed / Int'l Joint Research
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[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.
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Journal Title
J Neurol Sci.
Volume: 358(1-2)
Pages: 447-52
DOI
Related Report
Peer Reviewed
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[Journal Article] Creutzfeldt-Jakob disease with homozygous M232R mutation: A case report.2015
Author(s)
Kon T, Miki Y, Arai A, Funamizu Y, Ueno T, Haga R, Nishijima H, Suzuki C, Nunomura J, Baba M, Oyama Y, Shiga Y, Kitamoto T, Tomiyama M.
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Journal Title
J Neurol Sci.
Volume: 352(1-2)
Pages: 108-9
DOI
Related Report
Peer Reviewed
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[Journal Article] Clinical features of genetic Creutzfeldt-Jakob disease with V180I mutation in the prion protein gene.2014
Author(s)
Qina T, Sanjo N, Hizume M, Higuma M, Tomita M, Atarashi R, Satoh K, Nozaki I, Hamaguchi T, Nakamura Y, Kobayashi A, Kitamoto T, Murayama S, Murai H, Yamada M, Mizusawa H.
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Journal Title
BMJ Open.
Volume: 4(5)
Pages: e004968
DOI
Related Report
Peer Reviewed
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