Prion Disease Research

Prion (pronounced “pree-on”) diseases are rare neurodegenerative diseases affecting about one in a million people worldwide.

Despite this low prevalence, prion diseases constitute an important disease class as they represent the only known neurodegenerative disease which, in addition to sporadic and familial modes of manifestation (approximately 10% of all cases), can spread horizontally among humans or livestock through an infectious mode of propagation.  Prions are also unique amongst infectious pathogens in their ability to direct disease replication without the apparent need for nucleic acids.

Prion diseases in humans are known as Creutzfeldt-Jakob Disease (CJD). Rarer forms of manifestation are known as Gerstmann-Sträussler-Scheinker Syndrome (GSS) and Fatal Familial Insomnia (FFI). The better known prion diseases in livestock are “Scrapie” in sheep and goats, Bovine Spongiform Encephalopathy (BSE) in cattle and Chronic Wasting Disease (CWD) in elk and mule deer.

In the past decade, public awareness of these diseases has grown in parallel with the occurrence of cases of BSE (also known as “Mad Cow Disease”) and the realization that this disease can in rare instances be transmitted to humans through the consumption of contaminated meat and give rise to a variant form of Creutzfeldt-Jakob disease.

In the past few years it has become apparent that the most common dementias, including Alzheimer’s and Parkinson’s disease, spread within the brains of afflicted individuals by a mechanism that was first established to exist in Prion diseases. As a result, insights gained from the study of Prion diseases and the scientific methods used to investigate these relatively rare diseases have become critical for understanding the most common dementias.