Understanding the role of prions
TSEs are a group of terminal brain infectious diseases affecting a number of mammalian species, including humans. They are caused by prions; these causative agents are the mutated forms of the 'normal' prions, produced by healthy neuronal cells. The role of prions in cells, however, is not fully elucidated, leaving a big gap in our understanding of the molecular mechanisms involved in prion pathology. The EU-funded PRP AND NEURODEGENER aimed to arrive at clear definitions of prion cellular function and thus pave the way for a better understanding of TSEs. Initial studies had implicated prions in the cellular maintenance of ion balance and as part of the protective tools against oxidative stress. Project partners set out to test both these theories. Research at the Joseph Fourier University focused on examining prion role in copper ion homeostasis. A number of genetically modified (transgenic) mice populations were used for this purpose. These mice were genetically altered to carry specific genetic characteristics such as overexpression of prions. The studies showed that in mice with prion-overexpressing cells, the levels of free copper are drastically reduced, owing to the prion-copper ion interactions. Continuing this line of research is likely to yield further information on the function of prions and the events that follow infection.
