The prion protein (PrP) has been shown to bind copper. In the present study we have investigated whether prion disease in a mouse scrapie model resulted in modification of metal concentrations. We found changes in the levels of copper and manganese in the brains of scrapie-infected mice prior to the onset of clinical symptoms. Interestingly, we noted a major increase in blood manganese in the early stages of disease. Analysis of purified PrP from the brains of scrapie-infected mice also showed a reduction in copper binding to the protein and a proportional decrease in antioxidant activity between 30 and 60 days post-inoculation. We postulate that alterations in trace-element metabolism as a result of changes in metal binding to PrP are central to the pathological modifications in prion disease.
Metal imbalance and compromised antioxidant function are early changes in prion disease
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Alana M. THACKRAY, Robert KNIGHT, Stephen J. HASWELL, Raymond BUJDOSO, David R. BROWN; Metal imbalance and compromised antioxidant function are early changes in prion disease. Biochem J 1 March 2002; 362 (2): 253–258. doi: https://doi.org/10.1042/bj3620253
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