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See Inside January 2008

Progress against Prions

Ideas for treating the human form of mad cow disease begin to emerge

More than 200 cases of variant Creutzfeldt-Jakob disease, the human form of mad cow, have occurred worldwide since the 1990s. No accepted treatment exists for the devastatingly fatal disease or any of the others caused by infection with the malformed, malignant protein particles called prions. Giovanna R. Mallucci and her co-workers at the Institute of Neurology in London have performed an experiment in mice that could lay the groundwork for an eventual cure. Researchers genetically engineered mice to produce the protein PrP for only the first nine weeks after birth. PrP misfolds in the presence of prions, which causes it to produce more prions.

When both the altered mice and a normal group were injected with prions causing the illness called scrapie, both groups experienced a cognitive decline at first. The normal subjects continued to deteriorate. But by 12 weeks, the engineered group, without an ongoing supply of PrP, had recovered memory and normal behavior patterns. The absence of the natural protein did not appear to have an adverse effect on the restored rodents. If this approach proves itself further, it might lead to drugs or gene therapies that diminish PrP.

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