Mice devoid of PrP are resistant to scrapie.

Details

Serval ID
serval:BIB_E198D39C6504
Type
Article: article from journal or magazin.
Collection
Publications
Title
Mice devoid of PrP are resistant to scrapie.
Journal
Cell
Author(s)
Büeler H., Aguzzi A., Sailer A., Greiner R.A., Autenried P., Aguet M., Weissmann C.
ISSN
0092-8674 (Print)
ISSN-L
0092-8674
Publication state
Published
Issued date
1993
Volume
73
Number
7
Pages
1339-1347
Language
english
Abstract
S.B. Prusiner proposed that the infectious agent of scraple, the prion, is PrPSc, a modified form of the normal host protein PrPC. Prn-p0/0 mice devoid of PrPC showed normal development and behavior. When inoculated with mouse scrapie prions, they remained free of scrapie symptoms for at least 13 months while wild-type controls all died within 6 months. Surprisingly, heterozygous Prn-p0/+ mice also showed enhanced resistance to scrapie. After introduction of Syrian hamster PrP transgenes, Prn-p0/0 mice became highly susceptible to hamster but not to mouse prions. These experiments show that PrPC, possibly at close to normal levels, is required for the usual susceptibility to scrapie and that lack of homology between incoming prions and the host's PrP genes retards disease.
Keywords
Animals, Brain/pathology, Disease Susceptibility, Gene Deletion, Heterozygote, Mice, Mice, Transgenic, PrPSc Proteins, Prions/genetics, Prions/metabolism, Scrapie/etiology, Species Specificity, Spleen/pathology
Pubmed
Web of science
Create date
28/01/2008 12:37
Last modification date
20/08/2019 17:05
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