Toxic by-product, not rogue proteins, may cause fatal brain wasting.
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References
Mallucci, G. et al. Depleting neuronal PrP in prion infection prevents disease and reverses spongiosis. Science, 302, 871 - 874, (2003).
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University of Zurich
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Clarke, T. Gene tweak halts mad cow disease. Nature (2003). https://doi.org/10.1038/news031027-10
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DOI: https://doi.org/10.1038/news031027-10