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The study of prion propagation, pathogenesis and intermammalian species barriers in transgenic and other models of prion disease


(1) To further our understanding of the fundamental biology of prion propagation, and of the mechanisms and pathways leading to late onset neurodegeneration.
(2) To study possible therapeutic strategies for the human prion diseases.
(3) To estimate the efficacy of the species barriers limiting transmission of BSE and scrapie to humans to address the risk to public health within the EU posed by BSE and other animal prion diseases.
(4) to establish the full phenotypic range and transmission characteristics of the human prion diseases.

Prion diseases are transmissible neurodegenerative diseases of humans and animals. They have become an intense area of biomedical research activity for two reasons: Firstly, the transmissible agent or prion appears to be devoid of nucleic acid and consists essentially of a modified form of a host derived protein. Secondly, the epidemic of an apparently new animal prion disease, BSE, in the UK has raised legitimate public concerns of a risk to human health from eating infected tissues. Such concerns have had a major impact on the UK agricultural industry, and cases of BSE are now being reported throughout the EU. In addition, there have been concerns over the safety of pharmaceutical products derived from human or bovine tissues. We will develop and evaluate transgenic mouse, cell culture and in vitro models which, as well as allowing an experimental estimation of the risks of transmission of BSE to humans and a complete assessment of the transmission characteristics and phenotypic range of the human prion diseases, should provide the tools to study the mechanisms and pathways of prion neurodegeneration. These models will be used to evaluate therapeutic strategies and for testing of novel therapeutics. This application links with both current EC Concerted Actions in this area, studying the epidemiology and the neuropathology of human prion diseases to both provide a comprehensive range of tissues for study and to allow all the activities in this proposal to be placed within an epidemiological context within the EU. The prion diseases are now the best understood neurodegenerative conditions and advances made in this area are already providing insights into processes of neurodegeneration which may well be of much wider application. The recent identification of prion-like mechanisms in yeast suggests that the novel biology involved may not be restricted to this group of diseases but be of wider pathobiological importance. The development of radical treatments for prion related neurodegeneration may provide a key paradigm for studying the pathways of late onset neurodegeneration and the ability of the brain to recover function following therapeutic intervention.

Funding Scheme

CSC - Cost-sharing contracts


St Mary's Hospital Medical School
Norfolk Place
W2 1PG London
United Kingdom

Participants (6)

Aristotle University of Thessaloniki

TK54 006 Thessaloniki
Bundesforschungsanstalt für Viruskrankheiten der Tiere
72076 Tubingen
4,Kellnerweg 4
37077 Göttingen
Georg-August-Universität Göttingen
37073 Gottingen
Institute of Psychiatry
United Kingdom
De Crespigny Park Camberwell
SE5 8AF London
Universität Wien
18-20,Neues Akh O4j
1097 Vienna