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- Title
Assessing Prion Infectivity of Human Urine in Sporadic Creutzfeldt-Jakob Disease.
- Authors
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
- Abstract
Prion diseases are neurodegenerative conditions associated with a misfolded and infectious protein, scrapie prion protein (PrP[sup Sc]). PrP[sup Sc] propagates prion diseases within and between species and thus poses risks to public health. Prion infectivity or PrP[sup 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.
- Subjects
PRION diseases; CREUTZFELDT-Jakob disease; URINALYSIS; NEURODEGENERATION; PROTEINS; EMERGING infectious diseases
- Publication
Emerging Infectious Diseases, 2012, Vol 18, Issue 1, p21
- ISSN
1080-6040
- Publication type
Article
- DOI
10.3201/eid1801.110589