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Content archived on 2024-06-10

Prion diseases: Mechanisms of transmission and identification of ta rgets for potential therapeutics

Objective



Considerable advances have made recently in understanding the molecular biology of prion propagation, although many issues remain unresolved. New variant CJD appears to represent transmission of BSE to humans, and it remains to be seen if many more cases will arise. Because of the long incubation period of these diseases the peak of any such epidemic may be as long as 10-15 years ahead. Therapeutic targets are becoming apparent and the development of rational therapeutics appears increasingly realistic. This proposal aims to bring together a number of groups with different but complementary expertise in this area to explore a number of key areas which will underpin the development and assessment of therapeutics. We will attempt to modify PrPC expression and availability, study the effects of PrP polymorphism on PrPSc formation and develop peptides which bind avidly to PrPC and PrPSc, and which may affect PrPSc formation. Cell culture and transgenic models have already been developed and will be further characterised and the range of strain diversity in human prion diseases will be fully determined. As any useful therapy will act at or before onset of the earliest neuropathological changes, these early events in the brain will be characterised and cell populations at particular risk identified. It is anticipated that such studies, in addition to advancing our understanding of prion biology and assessing therapeutic strategies, will help understand fundamental mechanisms of neurodegeneration that may be relevant to other, commoner neurodegenerative diseases.

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Coordinator

IMPERIAL COLLEGE OF SCIENCE, TECHNOLOGY AND MEDICINE
EU contribution
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Address
Norfolk Place Imperial College School of Medicine
W2 1PG LONDON
United Kingdom

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Total cost

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Participants (9)

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