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

作者：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^*

来源：Emerging Infectious Diseases, 2012, 18(1): 21-28.

DOI：10.3201/eid1801.110589

Prion diseases are neurodegenerative conditions associated with a misfolded and infectious protein, scrapie prion protein (PrP(Sc)). PrP(Sc) propagates prion diseases within and between species and thus poses 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 %26lt;0.38 infectious units/mL.

• 出版日期2012-1