Chronic wasting disease (CWD) is a fatal prion disease that affects deer and is spreading rapidly, raising concern about whether it could one day pose a risk to people. So far, CWD has not been shown to infect humans, but the disease is caused by abnormal proteins called prions, and we now know that there are many different types (or strains) of these prions, some of which may adapt more easily to new animal species. In this project, we will study different CWD strains and evaluate whether they can spread from deer to animals such as pigs, sheep, or cattle. We will also test how CWD prions interact with the human prion protein after adaptation in animals other than cervids. Because not all strains have been studied and new ones continue to emerge, this research aims to identify which forms of CWD could represent future risks, helping to better protect people, especially those who handle or consume animal meat.

After the selection of few prion protein candidates that have shown DN capacity in the test tube, in this project, we intend to test if the production of these DN proteins, could indeed delay or stop disease progression animals. For that, these special proteins will be produced directly in the brains of the animal models, successfully driven by harmless adeno-associated viruses (AAVs), an increasingly popular system for genetic therapies. First, we will determine if these DN proteins are produced in the right amounts and locations in the brain. Once correct production is confirmed, we will evaluate the effectiveness of this treatment in our fast disease-developing animal models by comparing survival rates and disease progression with mice that do not produce these DN proteins.