Prion function in neuronal cells
Neurodegeneration describes the large scale demise of neuronal cells in brain tissue. TSEs are characterised by increased levels of neurodegeneration eventually leading to the patient's death. Within the framework of the EU-funded PRP AND NEURODEGENER project, researchers set out to shed light on the links between PrP and neurodegeneration. PrP in its natural form is produced by brain cells, however, its physiological role is still not fully explained. A number of studies have implicated PrP in cellular defence mechanisms against oxidative stress. Project partner, Centre National de Recherche Scientifique concentrated on PrP role's in anti-oxidative stress protection. Furthermore emphasis was placed not only on PrP as a whole, but also on specific protein domains. Study data indicated that cells overexpressing PrP to higher-than-normal levels seemed better equipped against the toxicity and potential DNA damage induced by oxidative stress. In addition to its antioxidant properties, PrPs were found to be involved in controlling copper ion levels in brain tissue. Copper ion balance in cells is known to be disturbed in conditions of oxidative stress, thus linking PrP's apparently dual set of roles. These findings are early-stage indications that PrP's function could be multifaceted and indeed crucial for the well-being of neuronal cells. The pathogenic form of PrP is likely to interfere with this function thus enabling the onset of TSE pathology. However, further research is needed before solid conclusion can be drawn and such findings can be capitalised on, in the form of new therapies and preventative vaccines.
