Mechanism of Prion Neurotoxicity

(University of Zurich, July 31, 2013)

Prion proteins are the infectious pathogens that cause Mad Cow Disease and Creutzfeldt-Jakob disease. They occur when a normal prion protein becomes deformed and clumped. Neuropathologists from the University of Zurich and University Hospital Zurich have found that it is the flexible tail of the prion protein that triggers cell death. Under normal conditions, this tail is very important in order to maintain the functioning of nerve cells. In the case of a prion infection, the pathogenic prion protein interacts with a portion of the globular part of the protein and - like a switch - cause a lethal mechanism is triggered that can lead to very fast cell death, mediated by the flexible tail.