1998 Fiscal Year Final Research Report Summary
Prion protein-deficient mice : neuronal abnormalities in the cerebellum and spinal cord
| Project/Area Number |
09680752
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| Research Category |
Grant-in-Aid for Scientific Research (C)
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| Allocation Type | Single-year Grants |
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| Section | 一般 |
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| Research Field |
Nerve anatomy/Neuropathology
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| Research Institution | Kansai Medical University |
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Principal Investigator |
SUGIMOTO Tetsuo Kansai Medical University, Faculty of Medicine Professor, 医学部, 教授 (90144352)
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| Project Period (FY) |
1997 – 1998
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| Keywords | Prion protein / Knockout mouse / Purkinje cell / Cerebellum / Spinal cord |
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| Research Abstract |
Disruption of both alleles of the prion protein gene, Prnp, renders mice resistant to prions. In one PrnpO/O line, such older mice developed ataxia and Purkinje cell loss. We found torpedo-like axonal swellings associated with residual Purkinje cells in PrnpO/O mice and demonstrated abnormal myelination in the spinal cord and peripheral nerves in mice from two independently established PrnpOfO lines. Both demyelination and Purkinje-cell degeneration weresuccessfully rescued by introduction of a transgene encoding wild-type mousecellular prion protein (PrPc). Together, these findings provide firm evidence that PrPc is indispensable for neuronal integrity.
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