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Molecular basis of neurodegeneration in transmissible spongiform encephalopathies (prp and neurodegeneration)

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New data on prion role

Prions (PrP), the causative agents for all transmissible spongiform encephalopathies (TSEs), are also expressed in healthy neuronal cells.


The mutated, virulent version of prions causes extensive brain damage through nerve cell death (neurodegeneration). However, little is known about the physiological role of prions in healthy cells. The EU-funded PRP NEURODEGENER project dealt with expanding our knowledge on prion function, as a potential first step towards the discovery and development of innovative anti-TSE therapies. A number of studies were conducted on trying to pinpoint PrP's role. Project partner, Centre National de Recherche Scientifique, through a series of genetic expression studies showed that PrP could be likely involved in ion homeostasis in neuronal cells. Specifically, copper ion levels were stabilised in the presence of PrP in cells. Copper ion levels are particularly sensitive to oxidative stress, which means that they are likely to fluctuate in such conditions. It is therefore likely that PrP takes on a dual protective role, against oxidative stress and towards copper ion homeostasis. These data could form the basis for a new approach towards therapy discovery and development in this area. Researchers are in a position to form collaborations and exchange technical know-how with interested parties in the field of medical and neuronal research.

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