Our lab studies the molecular mechanisms that underlie prion disease. Prion diseases are similar to other neurodegenerative disorders such as Alzheimer’s, Parkinson’s, and Huntington’s disease in that misfolded proteins play a central role.

Prions are the only protein aggregates that are naturally transmitted as an infectious disease, and most recently, human to human transmission likely occurred by transfusion of prion-contaminated blood. Cases of natural transmission can occur through ingestion of contaminated food followed by prion spread to the CNS through nerves. Our current research focuses on understanding how prions are initially taken up by cells and transported to the brain.

Prion infections have the potential to spread from animals to humans and have caused deaths as well as enormous economic losses in the UK. Chronic wasting disease (CWD) is an emerging prion disease in deer and elk, transmitted at a rate unparalleled by any other prion disease, and thus may present a risk of infecting humans, our food animals, and other wildlife. We are investigating the underlying mechanisms for when and how prions can infect a new host.

Our long term goal is to prevent prion aggregation and ultimately design treatments to arrest disease progression. We develop cell culture systems, biochemistry tools, and transgenic mouse models, together with our collaborators in protein chemistry, structural biology, and biophysics, to study the pathogenesis of a diverse array of infectious prions.