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