Prion research stepped up as fear grows of deer disease

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As concern mounts in the western United States about a relative of mad cow disease found among deer and elk, federal agencies are boosting research into this family of fatal neurodegenerative conditions.

Awareness of chronic wasting disease (CWD) has risen in the past few years. The disease, present in the Rocky Mountain states since the 1960s, has recently been found in the midwest (see Nature 416, 569–570; 200210.1038/416569a). Officials are also concerned that mad cow disease might enter the United States.

CWD, mad-cow disease and the human form, Creutzfeldt–Jakob disease (CJD), are all thought to be caused by infectious prions — misshapen forms of a protein that can convert the normal version to their own rogue shape.

There is no evidence that CWD has caused human disease, but this remains a possibility as 60% of people in the affected region eat venison or elk. Fears about CWD have been fuelled by reports of venison-eaters who have died with neurological symptoms, although none was found to have had a prion disease.

The Department of Defense is leading the push for more research with its National Prion Research Program. The Institute of Medicine held a meeting in Washington on 12–13 September to advise on priorities for the first grants, worth $42.5 million.

High on the agenda was the development of better diagnostic tests that could reveal infection before symptoms emerge. Cases of prion disease are currently confirmed by taking biopsies of tonsils or brain tissue.

The Department of Defense also wants to award grants to increase the size of the small US prion-research community. Researchers are put off by biosafety requirements and may be fearful of working with prions, says Pierluigi Gambetti of Case Western Reserve University in Cleveland, Ohio. “The reality is that you have to pay people more to work in your laboratories,” he says.

Gambetti heads the National Prion Disease Pathology Surveillance Center, which examines cases of neurological disease and maintains CJD tissue samples. Its budget is to rise fourfold to $1 million next year.

In addition, the National Institutes of Health wants to create a repository for reagents used in prion research. It is also considering creating a central repository for antibodies, which are necessary for the development of diagnostic tests.

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Dalton, R., Check, E. Prion research stepped up as fear grows of deer disease. Nature 419, 236 (2002) doi:10.1038/419236a

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