New data on prion-related brain disease
EU-funded project PRP AND NEURODEGENER sought to define the molecular basis on which neurodegeneration is triggered in mammalian neuronal cells. The causative agents of TSEs are protein-like components called prion proteins (PrP). PrP can be found in healthy brain cells but its mutated form can lead to TSEs. There is however, a variety of interpretations relating to the pathway leading to neurodegeneration. Project partners University of Hamburg worked on the definition of a specific pathogenic model of neurodegeneration in TSE. Researchers showed that PrP interactions with a recently identified binding partner in neuronal cells could trigger a series of events eventually leading to pathology. Hamburg University identified NCAM (neural cell adhesion molecule) as a binding partner to PrP, and both of these have been implicated in the past in neuronal abnormalities. It has been speculated that the PrP-NCAM interaction is somehow altered in the presence of the pathogenic form of PrP. Reduced PrP-NCAM association could also be caused by PrP antibodies which result in extensive neuronal cell death. Researchers are keen to explore this line of research with other interested parties either in the academic or private sector. The study data could play a key role in determining the interplay of different pathways at the molecular level that result in neurodegeneration in TSE. Such findings can be used to develop effective strategies toward therapeutic intervention for TSE in a number of species.
