Prion diseases belong to a group of fatal infectious diseases with no effective therapies available. Throughout
the last 35 years, less than 50 different drugs have been tested in different experimental animal models without hopeful results.
An important limitation when searching for new drugs is the existence of appropriate models of the disease. The
three different possible origins of prion diseases require the existence of different animal models for testing anti-prion
compounds. Wild type, over-expressing transgenic mice and other more sophisticated animal models have been used to
evaluate a diversity of compounds which some of them were previously tested in different in vitro experimental models.
The complexity of prion diseases will require more pre-screening studies, reliable sporadic (or spontaneous) animal models
and accurate chemical modifications of the selected compounds before having an effective therapy against human
This review is intended to put on display the more relevant animal models that have been used in the search of new antiprion
therapies and describe some possible procedures when handling chemical compounds presumed to have anti-prion
activity prior to testing them in animal models.
Keywords: Anti-prion drug, Model, Prion diseases, TSE, Transgenic mouse, PMCA, Cell-free system, Cell culture.
Rights & PermissionsPrintExport