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IMMUNOPRION — Result In Brief

Project ID: 23144
Funded under: FP6-FOOD
Country: France

Delineating the effect of prions on the immune system

Since the outbreak of bovine spongiform encephalopathy (BSE) in cattle in the late 1980s, considerable progress has been made regarding the biology and transmission of the prion infectious agent. A European initiative extended this knowledge by investigating the ability of prions to cross the species boundary and get transmitted through the food chain.
Delineating the effect of prions on the immune system
Prion diseases or transmissible spongiform encephalopathies (TSEs) are a group of neurodegenerative disorders that affect both humans and animals. They are characterised by long incubation periods, neuronal loss, and a failure to mount an inflammatory response against the infectious agent.

Despite extensive studying of prions – the causative agents of TSEs – there are many issues which remain unresolved regarding the diversity of prion strains, their capacity to cross the species boundary and the associated host immune responses. The site of the infectious protein (PrPSc) deposition can vary among different TSEs, increasing the chances of being passed on to the food chain.

The EU-funded ′Immunological and structural studies of prion diversity′ (Immunoprion) project wished to investigate fundamental features of TSEs that could aid the development of related diagnostics tools. The first project objective was to provide a better definition of prion strains based on the structural characteristics of the PrPSc. For this purpose, scientists extensively characterised in vitro synthesised PrPSc complexes and analysed their three-dimensional configuration by high-resolution cryo-electron tomography.

In order to examine the influence of chronic inflammation on prion transmission, team members used dextran sodium sulfate to induce colitis in animals. Results showed a block in prion progression during inflammation, concomitant with neuronal damage. This was consistent with other work, supporting the idea that the fate of the disease is determined during the early days of infection.

By isolating immune cells from these animals, researchers would be able to determine the interaction between prion strains and the immune system.

Collectively, the findings of the Immunoprion study strengthen the hypothesis that the immune system plays a crucial role in prion transmission and especially in crossing the species barrier. This insight could lead to the development of new and innovative tools to further study and detect prions.

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