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A mouse prion protein transgene rescues mice deficient for the prion protein gene from purkinje cell degeneration and demyelination

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.J.; Prusiner, S.B.; Katamine, S.

Laboratory Investigation; a Journal of Technical Methods and Pathology 79(6): 689-697

1999

ISSN/ISBN: 0023-6837
PMID: 10378511
Accession: 010070785
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Disruption of both alleles of the prion protein gene, Prnp, renders mice resistant to prions; in a Prnp o/o line reported by some of us, mice progressively developed ataxia and Purkinje cell loss. Here we report torpedo-like axonal swellings associated with residual Purkinje cells in Prnp o/o mice, and we demonstrate abnormal myelination in the spinal cord and peripheral nerves in mice from two independently established Prnp o/o lines. Mice were successfully rescued from both demyelination and Purkinje cell degeneration by introduction of a transgene encoding wild-type mouse cellular prion protein. These findings suggest that cellular prion protein expression may be necessary to maintain the integrity of the nervous system.

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