Assessing Prion Infectivity of Human Urine in Sporadic Creutzfeldt-Jakob Disease
Silvio Notari1, Liuting Qing1, Maurizio Pocchiari, Ayuna Dagdanova, Kristin Hatcher, Arend Dogterom, Jose F. Groisman, Ib Bo Lumholtz, Maria Puopolo, Corinne Lasmezas, Shu G. Chen, Qingzhong Kong, and Pierluigi Gambetti
Author affiliations: Case Western Reserve University, Cleveland, Ohio, USA (S. Notari, L. Qing, A. Dagdanova, K. Hatcher, S.G. Chen, Q. Kong, P. Gambetti); Istituto Superiore di Sanità, Rome, Italy (M. Pocchiari, M. Puopolo); Ferring Pharmaceuticals, Hvidore, Denmark (A. Dogterom); Instituto Massone, Buenos Aires, Argentina (J.F. Groisman); BL Consult ApS, Copenhagen, Denmark (I.B. Lumholtz); The Scripps Research Institute, Jupiter, Florida, USA (C. Lasmezas)
Figure 2. Immunochemical and histopathologic study of humanized transgenic (Tg) mice inoculated with sporadic Creutzfeldt-Jakob disease MM1 (sCJDMM1) microsomal fraction (MF). A) Immunoblot of proteinase K (PK)–resistant scrapie prion protein (PrPSc) from brains of 10 Tg40 mice inoculated with MF from a patient with sCJDMM1. The inoculum sCJDMM1 MF is shown as control in the first lane. Histologic (B) and immunohistochemical (C) studies show widespread spongiform degeneration and punctate PrPSc immunostaining of the cerebral cortex from the inoculated mice. Monclonal antibody 3F4 was used for all immunostaining. Scale bar in B = 100 μm. Scale bar in C = 50 μm.
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