1998 Fiscal Year Final Research Report Summary
MECHANISMS OF NEUTONAL CELL DEATH IN PRION PROTEIN-LESS MICE
| 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)
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| Project Period (FY) |
1997 – 1998
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| Keywords | PRION PROTEIN / TARGETED MYTAGENESIS / PURKINJE CELLS / NEURODEGENERATION / DEMYELINATION / 軸索 |
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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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[Publications] NISIDA,N., KATAMINE,S., SHIGEMATSU,K., NAKATANI,A., SAKAMOTO,N., HASEGAWA,.S., NAKAOKE,R., ATARASHI,R., KATAOKA,Y., and MIYAMOTO,T.: "Prion protein is necessary for latent learning and long-term memory retention"Cell. Mol. Neurobiol.. 17. 537-545 (1997)
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「研究成果報告書概要(欧文)」より
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[Publications] KATAMINE,S., NISHIDA,N., SUGIMOTO,T., NODA,T., SAKAGUCHI,S., SHIGEMATSU,K., KATAOKA,Y., NAKATANI,A., HASEGAWA,S., MORIUCHI,R., and MMIYAMIOTO,T.: "Impaired motor coordination in mice lacking prion protein"Cell. Mol. Neurobiol. 18. 731-742 (1998)
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「研究成果報告書概要(欧文)」より
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[Publications] SATOH,J., YUKITAKE,M., KUROHARA,K., NISHIDA,N., KATAMINE,S., MIYAMOTO,T., and KURODA,Y.: "Cultured skin fibroblasts isolated from mice devoid of the prion protein gene express major heat shock proteins in response to heat stress"Exp. Neurol.. 151. 105-115 (1998)
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「研究成果報告書概要(欧文)」より
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[Publications] NISHIDA,N., Tremblay,P., SUGIMOTO,T., SHIGEMATSU,K., SHIRABE,S., Petromilli C., Erpel S.P., NAKAOKE,R., ATARASHI,R., HOUTANI,T., Torchia,M., SAKAGUCHI,S., DeArmond,S.B., KATAMINE,S.: "A mouse prion protein (PrP) transgene rescues Purkinje cell degeneration and demyelination in mice deficient for PrP"(submitted).
Description
「研究成果報告書概要(欧文)」より
