Evaluation of the public health risk of atypical and emerging prions.

The present project aims to clarify the pathogenesis and the transmissibility of atypical and emerging prion disease and identify potential biomarkers for early diagnosis.
Our final goal is to provide to risk assessment and risk management authorities the necessary elements for an in-depth assessment of the public health risk associated with atypical prions (namely BSE-L, BSE-H and atypical scrapie) and the, recently emerged in Europe cervid prion disease CWD. We also aim to generate tools for pre-clinical early detection in human patients, should these agents cross the barrier to the human species.
This goal will be achieved through the implementation of the following objectives:
- Objective 1: To establish the susceptibility of transgenic mice over-expressing human PrP to atypical prions (bovine and ovine) and CWD as compared to classical BSE.
- Objective 2: To establish the relative capacity of human PrP to propagate (in vitro conversion test) atypical prions (bovine and ovine) and CWD.
- Objective 3: To evaluate any possible alteration of atypical and CWD prions zoonotic potential by passage through an intermediate species (i.e. atypical scrapie has been bovinized, atypical bovine prions have been ovinized, and CWD has been bovinized and ovinized).
- Objective 4: To establish new biological fluid biomarkers for the early detection of prion infection.