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University Of California-San Francisco Researchers Report Test That Detects Prion Diseases, Illuminates Novel,,Findings About Infectious Prions

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"Until now, we believed that once formed in the brain, prions could not be degraded. We now understand that it is the rate at which prions are degraded that explains the differences in the time that it takes a prion strain to cause disease," said Cohen. "Since the body can begin to clear the proteinaceous mess from the brain, treatments are being developed to assist this process."

"The only conclusion," Cohen said, "counterintuitive as it is, can be that the rate-limiting step in prion replication has little to do with PrPsc.

Instead, Cohen and Prusiner suggested, it must have to do with an earlier stage in the development of PrPSc, when normal PrPC protein binds to an as-yet-elusive "protein X." Protein X is believed to act as a molecular chaperone, moving the normal protein out to the dance floor where it presumably is handed off to its deadly suitor.

Needless to say, the researchers are turning their attention to this earlier stage in the conversion cascade, before the protease-resistant fraction is formed.

"While we still can't visualize protein X, we need to see if we can figure out its role," said Safar. The researchers' challenge, which molecular biologist face every day in their explorations, will be developing still more clever techniques that will reveal to them what they can't actually see, in this case the machinations of a deadly protein.

The University of California has filed a patent on the full technology platform for the immunoassay. Centeon Inc. holds a license granting them exclusive rights to the immunoassay technology.

Other co-authors of the UCSF study included Holger Wille, PhD, Vincenza Itri, BS, Darlene Groth, BS, Hana Serban, MS, and Marilyn Torchia, DVM. The study was supported by grants from the National Institutes of Health, as well as well as by gifts from the Leila G. and Harold Mathers Foundation, Sherman Fairchild Foundation and
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Contact: Jennifer O'Brien
jobrien@itsa.ucsf.edu
(415) 476-2557
University of California - San Francisco
28-Sep-1998


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