Prion diseases, cell biology of prion infection, chronic wasting disease, prion strains
Prion diseases are unique neurodegenrative diseases for several reasons: first, they are caused by fascinating infectious agents called prions, which are infectious proteins that replicate without genetic information such as DNA or RNA; second, they can have 3 different etiologies (sporadic, genetic, infectious) in humans; third, while there are other neurodegenerative diseases associated with protein misfolding (e.g. Alzheimer's disease) also referred to as 'prion-like' disorders, only prion diseases are naturally transmissible from human-to-human, animal-to-animal and even animal-to-human. Most well-known examples are Creutzfeldt-Jakob disease in humans, bovine spongiform encephalopathy (mad cow disease) in cattle and chronic wasting disease (CWD) in cervids. I have been studying prions for more than 20 years and joined UCVM in 2013 as Tier 2 Canada Research Chair in Prion Disease Research. We use cell culture and rodent models or prion disease to understand how prion replication affects neuronal cholesterol metabolism and vesicle trafficking in the endocytic pathway with the goal to identify new drug targets and therapies for prion diseases, which are strictly fatal and not treatable to date. For example, my group has introduce peptide aptamers as a new class of anti-prion agents, which we currently evaluate for their effects on the pathogenesis of Alzheimer's disease. Further, we study the diversity of chronic wasting disease strains and have established new mouse models that allow us to gain understanding of strain selection and adaptation upon natural infection routes. This is highly relevant in order to determine transmission profiles, including the possibility of cervid-to-human or cervid-to-livestock transmission.