Protective effect of cellular prions
Under the auspices of the LIFE QUALITY programme, the PRP AND NEURODEGENER project focused on the mechanisms leading from PrP infection to neurodegeneration. Of particular interest was the role of naturally-occurring PrP (PrPC), which is expressed in healthy, non-infected neuronal cells. In its natural form PrP has been implicated in a variety of cellular mechanisms and it is likely involved in protecting cellular components against oxidative stress. Oxidative stress has been linked to neurodegeneration and therefore, the mutated, virulent form of PrP could interfere with the natural function of PrP, namely to protect from oxidative stress. Studies on a number of cell lines showed that PrP expression led to an expression of antioxidant enzymes and finally overall increased resistance to oxidative stresses. Furthermore, the protective effect of PrPC was demonstrated in the cases of metal-induced cellular toxicity. PrPC expression in A74 cells led to a marked protective effect against copper and zinc toxicity. However, the same effect was not observed in the case of manganese toxicity, a fact which has not been explained. Combining such findings with further research observations could help to fully elucidate PrP's cellular function thus paving the way for new treatment against TSEs.
