PrP-deficient mice are resistant to scrapie.

Details

Serval ID
serval:BIB_C8A41C1328DC
Type
Article: article from journal or magazin.
Collection
Publications
Title
PrP-deficient mice are resistant to scrapie.
Journal
Annals of the New York Academy of Sciences
Author(s)
Weissmann C., Büeler H., Fischer M., Sailer A., Aguzzi A., Aguet M.
ISSN
0077-8923 (Print)
ISSN-L
0077-8923
Publication state
Published
Issued date
1994
Volume
724
Pages
235-240
Language
english
Abstract
Prusiner proposed that the infectious agent of scrapie, 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 16 months while wild type controls all died within 6 months. Propagation of infectivity in the PrP null mice, if any, was less than 10(-5) that in wild type animals. 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/metabolism, Brain/microbiology, Cricetinae, Genetic Complementation Test, Immunity, Innate, Mesocricetus, Mice, Mice, Mutant Strains, Prions/genetics, Scrapie/genetics, Scrapie/immunology, Spleen/metabolism, Spleen/microbiology
Pubmed
Web of science
Create date
28/01/2008 12:36
Last modification date
20/08/2019 16:43
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