| Issue |
Vet. Res.
Volume 39, Number 4, July-August 2008
Prion diseases in animals
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|---|---|---|
| Number of page(s) | 13 | |
| DOI | https://doi.org/10.1051/vetres:2008008 | |
| Published online | 15 February 2008 | |
| How to cite this article | Vet. Res. (2008) 39:32 | |
DOI: 10.1051/vetres:2008008
Review
Rodent models for prion diseases
Martin H. Groschup and Anne BuschmannFriedrich-Loeffler-Institut (FLI), Institute for Novel and Emerging Infectious Diseases, Südufer 10, 17493 Greifswald - Insel Riems, Germany
Received 2 September 2007; accepted 15 January 2008; published online 15 February 2008
Abstract - Until today most prion strains can only be propagated
and the infectivity content assayed by experimentally challenging
conventional or transgenic animals. Robust cell culture systems are not
available for any of the natural and only for a few of the experimental
prion strains. Moreover, the pathogenesis of different transmissible
spongiform encephalopathies (TSE) can be analysed systematically by
using experimentally infected animals. While, in the beginning, animals
belonging to the natural host species were used, more and more rodent model
species have been established, mostly due to practical reasons. Nowadays,
most of these experiments are performed using highly susceptible transgenic
mouse lines expressing cellular prion proteins, PrP, from a variety of species
like cattle, sheep, goat, cervidae, elk, hamster, mouse, mink, pig, and man.
In addition, transgenic mice carrying specific mutations or polymorphisms
have helped to understand the molecular pathomechanisms of prion diseases.
Transgenic mouse models have been utilised to investigate the physiological
role of PrP
, molecular aspects of species barrier effects, the cell
specificity of the prion propagation, the role of the PrP glycosylation, the
mechanisms of the prion spread, the neuropathological roles of PrP and
of its abnormal isoform PrP
(D for disease) as well as the function of
PrP Doppel. Transgenic mouse models have also been used for mapping of PrP regions
involved in or required for the PrP conversion and prion replication as well
as for modelling of familial forms of human prion diseases.
Key words: prion / scrapie / BSE / transgenic mouse
Corresponding author: martin.groschup@fli.bund.de
© INRA, EDP Sciences 2008