JBC Invitrogen Ultrasensitive Cytokine Assays

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Originally published In Press as doi:10.1074/jbc.C100278200 on June 21, 2001

J. Biol. Chem., Vol. 276, Issue 34, 31479-31482, August 24, 2001
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ACCELERATED PUBLICATION
A Protease-resistant Prion Protein Isoform Is Present in Urine of Animals and Humans Affected with Prion Diseases*

Gideon M. Shaked, Yuval Shaked, Zehavit Kariv-Inbal, Michele Halimi, Inbal Avraham, and Ruth GabizonDagger

From the Department of Neurology, the Agnes Ginges Center for Human Neurogenetics, Hadassah University Hospital, Jerusalem 91120, Israel

Prion protein (PrP)Sc, the only known component of the prion, is present mostly in the brains of animals and humans affected with prion diseases. We now show that a protease-resistant PrP isoform can also be detected in the urine of hamsters, cattle, and humans suffering from transmissible spongiform encephalopathies. Most important, this PrP isoform (UPrPSc) was also found in the urine of hamsters inoculated with prions long before the appearance of clinical signs. Interestingly, intracerebrally inoculation of hamsters with UPrPSc did not cause clinical signs of prion disease even after 270 days, suggesting it differs in its pathogenic properties from brain PrPSc. We propose that the detection of UPrPSc can be used to diagnose humans and animals incubating prion diseases, as well as to increase our understanding on the metabolism of PrPSc in vivo.

* The costs of publication of this article were defrayed in part by the payment of page charges. The article must therefore be hereby marked "advertisement" in accordance with 18 U.S.C. Section 1734 solely to indicate this fact.

Dagger To whom correspondence should be addressed. Fax: 972-2-6429441; E-mail: gabizonr@hadassah.org.il.

Copyright © 2001 by The American Society for Biochemistry and Molecular Biology, Inc.


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