| Project/Area Number |
09044321
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| Research Category |
Grant-in-Aid for international Scientific Research
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| Allocation Type | Single-year Grants |
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| Section | Joint Research |
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| Research Field |
Neuroscience in general
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| Research Institution | Nagasaki University |
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Principal Investigator |
KATAMINE Shigeru NAGASAKI UNIVERSITY SCHOOL OF MEDICINE, DEPTRTMENT OF BACTERIOLOGY, PROFESSOR, 医学部, 教授 (40161062)
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| Co-Investigator(Kenkyū-buntansha) |
SHIRABE Susumu NAGASAKI UNIVERSITY SCHOOL OF MEDICINE, DEPTRTMENT OF BACTERIOLOGY, LECTURER, 医学部・附属病院, 助手 (40264220)
SHIGEMATSU Kazutio NAGASAKI UNIVERSITY SCHOOL OF MEDICINE, DEPTRTMENT OF BACTERIOLOGY, ASSOCIATE PROFESSOR, 医学部, 助教授 (20154205)
NIWA Msaki NAGASAKI UNIVERSITY SCHOOL OF MEDICINE, DEPARTMENT OF PTHOLOGY, PROFESSOR, 医学部, 教授 (20136641)
KATAOKA Yasufumi NAGASAKI UNIVERSITY SCHOOL OF MEDICINE, DEPTRTMENT OF PHARMACEUTICAL MEDICINE, ASSOCIATE PROFESSOR, 医学部・附属病院, 助教授 (70136513)
SAKAGUCHI Suehiro NAGASAKI UNIVERSITY SCHOOL OF MEDICINE, DEPTRTMENT OF BACTERIOLOGY, LECTURER, 医学部, 助手 (60274635)
DERI Maria.A ハンガリー科学アカデミー, 生物学研究センター, 専任研究員
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| Project Period (FY) |
1997 – 1998
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| Project Status |
Completed (Fiscal Year 1998)
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| Budget Amount *help |
¥6,300,000 (Direct Cost: ¥6,300,000)
Fiscal Year 1998: ¥2,700,000 (Direct Cost: ¥2,700,000)
Fiscal Year 1997: ¥3,600,000 (Direct Cost: ¥3,600,000)
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| Keywords | PRION PROTEIN / TARGETED MYTAGENESIS / PURKINJE CELLS / NEURODEGENERATION / DEMYELINATION / 軸索 / プリオン病 / プリオン / 遺伝子改変動物 / 神経細胞死 / アストロサイト / 脱髄 |
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| Research Abstract |
In prion-infected animals, the normal, cellular prion protein (PrPィイD1cィエD1), the function of which is unknown, is converted into a pathogenic isoform (PrPィイD1scィエD1) by a posttranslational process. Disruption of both Prnp alleles (PrnpィイD10/0ィエD1) renders mice resistant to prions and in one PrnpィイD10/0ィエD1 line reported by some of us, such older mice developed ataxia and Purkinje cell loss suggesting that PrPィイD1cィエD1 might be essential for the long-term survival of some neurons. This phenotype was not reported for two other PrnpィイD10/0ィエD1 lines established independently. We report here torpedo-like axonal swellings associated with residual Purkinje cells in PrnpィイD10/0ィエD1 mice and demonstrate abnormal myelination in the spinal cord and peripheral nervous system (PNS) in mice from two independently established PrnpィイD10/0ィエD1 lines. Both demyelination and Purkinje cell degeneration were rescued by introduction of a transgene encoding wild-type mouse PrPィイD1cィエD1.
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