Study examines how prion disease adapts to n...( Although scientists believe that mad co...)

Although scientists believe that mad cow disease spread from cattle to people in a few instances in the United Kingdom, they know very little about how that happened. To better understand how diseases like mad cow jump and adapt to a new species, researchers at the National Institute of Allergy and Infectious Diseases (NIAID) examined the process by which a disease called scrapie transfers from hamsters to mice.

"We found that the adaption is a prolonged and subtle process, and the early stages of it are very difficult to detect," says Bruce Chesebro, M.D., senior study author and researcher at NIAID's Rocky Mountain Laboratories (RML) in Montana. The results of his team's work are reported in the current issue of the Journal of Virology.

Scrapie and mad cow are examples of the rare, mysterious and fatal brain diseases known as transmissible spongiform encephalopathies (TSEs). Also known as prion diseases, they include chronic wasting disease in deer and elk and Creutzfeldt-Jakob disease in humans. The hallmark of TSE diseases is misshapen protein molecules that clump together and accumulate in brain tissue. Normal forms of these molecules, called prion protein, reside on the surface of brain cells, although no one knows their proper function. Abnormal prion proteins are the likely cause of the brain damage that occurs in TSE diseases. Scientists believe the misshapen prion proteins somehow induce normal prion proteins to form incorrectly. These abnormal molecules may spread the disease to new individuals. Alternatively, some scientists still believe that the disease may be initiated by a virus.

Dr. Chesebro and his team first inoculated mice with a strain of hamster scrapie, then watched the mice closely over a period of years. This group of mice never became sick, but the scientists found that the hamster scrapie agent persisted in these mice for years at levels too low for standard lab tests to detect. The scientists detected t
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Contact: Jeff Minerd
jminerd@niaid.nih.gov
301-402-1663
NIH/National Institute of Allergy and Infectious Diseases
17-Oct-2001

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