Understanding prion-related diseases
TSEs are caused by prions (PrP). Unlike any other infectious agent, prions are proteins that can result in widespread neurodegeneration, which finally leads to the patient's death. The EU-funded PRP AND NEURODEGENER project set out to increase the scientific community's understanding of all key mechanisms underlying TSE and prion-related pathology. Furthermore, project partners aimed to devise new animal models of these diseases in an effort to better understand molecular-level interactions. The University of Hamburg examined the physiological role of PrP in neuronal cells. Healthy neurons produce PrP as part of their protein machinery, however the physiological role of the PrP protein in healthy brains has not been fully elucidated. Research revealed that PrP is most likely involved in a series of signaling pathways in the brain. The interaction between PrP and neuronal receptor NCAM has been defined as a key one, and is likely the first step in any signaling pathway that PrP is involved in. It was shown that the PrP-NCAM interaction activates at least one further pathway, termed the fyn-kinase pathway. In the absence of PrP in healthy cell lines, fyn activation remained at low levels, however it increased dramatically upon PrP addition. It is still not clear how the total variety of interactions between PrP and NCAM affects cellular mechanisms but it is likely that PrP is involved in a series of protective mechanisms in the brain cell. Scientists are keen to gain further support in order to continue this line of research. These findings might have important impact on the therapy-development process for TSEs.
