2015 Fiscal Year Final Research Report
Is thalamic CJD a sporadic form of fatal familial insomnia?
| Project/Area Number |
26670435
|
|---|
| Research Category |
Grant-in-Aid for Challenging Exploratory Research
|
| Allocation Type | Multi-year Fund |
|---|
| Research Field |
Neurology
|
|---|
| Research Institution | Tohoku University |
|---|
Principal Investigator |
|
|---|
| Project Period (FY) |
2014-04-01 – 2016-03-31
|
| Keywords | プリオン / FF1 / ノックインマウス / 感染実験 |
|---|
| 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.
|
| Free Research Field |
脳神経科学
|
