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Test could improve detection of prion disease in humans

and his colleagues extracted brain tissue from 28 people who had died of CJD. They tested these samples using CDI, which uses highly specific antibodies that bind to all disease-causing prions in the brain. They also used immunohistochemistry (IHC) to measure only the prion proteins that are resistant to an enzyme called protease. Protease-resistant prions are abnormal and usually infectious, meaning they can cause CJD and other neurodegenerative diseases. CDI detected abnormal prions in all of the sampled brain regions. In contrast, the researchers found that IHC detected abnormal prions in less than 25 percent of the sampled brain regions. The findings, according to the researchers, suggest that CDI could be used to establish or rule out the diagnosis of CJD with greater accuracy than IHC, particularly when a small number of samples are available. Prusiner and colleagues are exploring the possibility of using CDI in living tissue, like blood or muscle, to detect and diagnose prion diseases, such as CJD or bovine spongiform encephalopathy (BSE, mad cow disease) while people or animals are still alive.

"This research not only is an important advance for the detection and diagnosis of prion diseases, but, with the identification of protease-sensitive infectious prions, will lead to a better understanding of the underlying disease processes," said Andrew Monjan, Ph.D., Chief of the NIA's Neurobiology of Aging Branch.

Prusiner received the 1997 Nobel Prize in physiology or medicine for his discovery of prions. Unlike viruses, bacteria, fungi, and parasites, prions contain no DNA or RNA. Instead, prions are an altered type of protein normally found within cells in humans and other organisms. These abnormal prion proteins appear to convert other, normal prions to an abnormal shape. Many scientists now believe this conversion process leads to several dementing diseases in humans, including CJD. Similar diseases in animals include bovine spongiform encephalopat
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14-Feb-2005


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