Making sense of prion biology
The EU-funded PRP AND NEURODEGENER project concentrated partner efforts on the pathology surrounding Transmissible spongiform encephalopathies (TSEs). TSEs comprise a number of terminal brain diseases characterised by extensive neurodegeneration. The causative agent of TSEs, the prion protein (PrP), is still poorly understood, however, in spite of the seriousness of the conditions. PrP forms part of the proteins produced by neuronal cells under normal conditions. However, its physiological role is still not clear. Project partners worked on expanding the available knowledge-base and providing concrete evidence on the role of PrP in the brain. These findings could facilitate the development of novel therapies to combat TSEs. PrP appears to be anchored in the surface of cells and has been implicated in a number of cellular functions during the course of the study. Its interactions with a "well known" molecule in the central nervous system, NCAM (neural cell adhesion molecule) received much attention within the framework of PRP AND NEURODEGENER. It was established the PrP interacts with NCAM in a variety of ways. These interactions were proven to be crucial for the stabilisation of NCAM onto specialised cellular areas, known as "lipid rafts". As part of NCAM-dependant signalling pathways, PrP was shown to play an important role in the distribution and structural support of NCAM onto lipid rafts. In enriching the available knowledge surrounding PrP and TSE-related neurodegeneration, scientists are improving the medium-term prospects for the development of new therapies based on appropriate molecular targets.
