Assessing Prion Infectivity of Human Urine in Sporadic Creutzfeldt-Jakob Disease

Notari, Silvio; Qing, Liuting; Pocchiari, Maurizio; Dagdanova, Ayuna; Hatcher, Kristin; Dogterom, Arend; Groisman, Jose F.; Lumholtz, Ib Bo; Puopolo, Maria; Lasmezas, Corinne; Chen, Shu G.; Kong, Qingzhong; Gambetti, Pierluigi

doi:10.3201/eid1801.110589

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Assessing Prion Infectivity of Human Urine in Sporadic Creutzfeldt-Jakob Disease

Published Date:

Jan 2012

Publisher's site:

http://dx.doi.org/10.3201/eid1801.110589 New Site Icon

Source:

Emerg Infect Dis. 18(1):21-28.

[PDF-800.47 KB]

Details:

Alternative Title:

Emerg Infect Dis

Personal Author:

Notari, Silvio ; Qing, Liuting ; Pocchiari, Maurizio ; Dagdanova, Ayuna ; Hatcher, Kristin ; ... More ▼

Description:

Prion diseases are neurodegenerative conditions associated with a misfolded and infectious protein, scrapie prion protein (PrP(Sc)). PrP(Sc) propagate prion diseases within and between species and thus pose risks to public health. Prion infectivity or PrP(Sc) presence has been demonstrated in urine of experimentally infected animals, but there are no recent studies of urine from patients with Creutzfeldt-Jakob disease (CJD). We performed bioassays in transgenic mice expressing human PrP to assess prion infectivity in urine from patients affected by a common subtype of sporadic CJD, sCJDMM1. We tested raw urine and 100-fold concentrated and dialyzed urine and assessed the sensitivity of the bioassay along with the effect of concentration and dialysis on prion infectivity. Intracerebral inoculation of transgenic mice with urine from 3 sCJDMM1 patients failed to demonstrate prion disease transmission, indicating that prion infectivity in urine from sCJDMM1 patients is either not present or is <0.38 infectious units/mL.

Subject:

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Document Type:

Journal Article

Funding:

AG014359/AG/NIA NIH HHS/United States ; NS052319/NS/NINDS NIH HHS/United States ; UR8/CCU515004/PHS HHS/United States ;

Collection(s):

Emerging Infectious Diseases

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