Prions, unlike other infectious agents or germs, appear to lack their own genes and consist of aggregated misshapen forms of one of the body’s own proteins, the prion protein or PrP. Despite lacking genes, prions can exist as distinct strains with quite different properties. These unique features have wide implications in biology and evolution, and prions and prion diseases are of intense international research interest. However, in addition to the importance of this basic science, the Unit’s mission is also to tackle key public health issues posed by bovine spongiform encephalopathy (BSE) and variant Creutzfeldt-Jakob disease (vCJD).
It is becoming increasingly clear that other much commoner degenerative brain diseases, such as Alzheimer’s and Parkinson’s disease, also involve accumulation of rogue or abnormal forms of one or more of the body’s proteins resulting in damage to brain cells. Prion diseases, in addition to their intrinsic interest and importance, can be considered a paradigm for these other “protein misfolding” diseases.