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Understanding prion strains and species barriers and devising novel diagnostic approaches

Final Report Summary - STRAINBARRIER (Understanding prion strains and species barriers and devising novel diagnostic approaches)

The research consortium STRAINBARRIER studied fundamental and applied aspects of prions strains and their relationship to the species barrier, with the goal of providing better tools and methodologies to diagnose prion strains and to predict their behaviour.

The project aimed to:
1. systematically study field BSE / scrapie strains, their species barriers in transgenic mice expressing sheep, cattle, porcine, murine PrP;
2. evaluate the permeability of the animal-to-human species barrier of these TSE agents;
3. validate the use of a transgenic mice panel for a rapid and highly efficient identification and characterisation of TSE strain biodiversity;
4. study fundamental aspects of strains: PrPSc structure, cell biology and pathogenesis;
5. develop novel diagnostic and research reagents such as strain-dependent antibodies and cell culture systems;
6. interact with national disease control agencies.

In general, the objectives of the STRAINBARRIER project were to:
(i) improve our understanding of the prion strain phenomenon at the structural / molecular, cell biological, and epidemiological levels; and
(ii) to prepare novel reagents, devise assay system, and use the knowledge generated in order to improve our ability to detect prions and diagnose strains.

These goals were largely attained. In addition, a number of unexpected discoveries were made, and the project has led to a better understanding of the biology of prions.

STRAINBARRIER has provided a host of results about prions per se, irrespective of strains. The cell biology of prions appears to have especially benefited from these studies (the first PrPSc-synthetic compartment; tunnelling nanotupes transport prions from cell-to-cell; signalling networks mapped in prion-infected cells, etc). However, other aspects will benefit too: novel expertise was generated as to the detection of PrPSc in brain section by cryo-electron microscopy; a novel insight was attained as to the properties of protease-sensitive PrP (CR4 Requena), etc. This new knowledge will help to advance the science of prions.

STRAINBARRIER has generated a very large body of data as to the behaviour of strains as they cross species (their new incubation time, their strain stability, the biochemical properties of the strains in the two species, etc.). This will help epidemiologists and health authorities to predict possible zoonotics, etc.

Finally, reagents (strain-specific PrPSc-binding peptides, antibodies), powerful cell systems (neurospheres, etc.), and methodologies were developed that will help both authorities and scientists in their endeavour in the field of prions. This will have an important impact on public health, food safety, and prion research.

STRAINBARRIER has resulted in many publications in refereed journals as well as numerous presentations at scientific conferences as well as in the lay media.