Prions are infectious proteins which cause increasing numbers of fatal neurodegenerative diseases in humans and animals. During prion replication, a normal host encoded protein misfolds into an aggregated conformation, which is capable of inducing further conformational change. The resulting process is therefore unique in biology since infectious prion proteins do not require the involvement of informational nucleic acids during their exponential replication.
As the causative agent of transmissible spongiform encephalopathies (TSEs), the unprecedented replicative properties of prions and unpredictable epidemiology of prion diseases, have fatal effects around the world. The transmission of bovine spongiform encephalopathy, known as mad cow disease, through the food chain to humans raised significant concerns about the risks posed to human by other emerging animal prion diseases.
Chronic wasting disease (CWD), a highly contagious and ineradicable prion disease of deer, elk, and related species, is of particular concern. Initially discovered at Colorado State University, CWD has spread across North America, and in recent years, emerged in Asia and Europe.
The unique properties of prions present significant challenges that require facilities with specialized expertise and infrastructure. Researchers at the Prion Research Center (PRC) leverage their renowned expertise in whole animal, transgenic, cell biological, biochemical, molecular genetic, and in vitro approaches to better understand prion pathogenesis, the mechanism of prion propagation, and the molecular events underlying prion species barriers and strain variation.