Transmission Characteristics of Variably Protease-Sensitive Prionopathy
| Autor: | Silvio Notari, Xiangzhu Xiao, Juan Carlos Espinosa, Yvonne Cohen, Liuting Qing, Patricia Aguilar-Calvo, Diane Kofskey, Ignazio Cali, Laura Cracco, Qingzhong Kong, Juan Maria Torres, Pierluigi Gambetti |
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| Jazyk: | angličtina |
| Rok vydání: | 2014 |
| Předmět: | |
| Zdroj: | Emerging Infectious Diseases, Vol 20, Iss 12, Pp 2006-2014 (2014) |
| Druh dokumentu: | article |
| ISSN: | 1080-6040 1080-6059 |
| DOI: | 10.3201/eid2012.140548 |
| Popis: | Variably protease-sensitive prionopathy (VPSPr), a recently identified and seemingly sporadic human prion disease, is distinct from Creutzfeldt-Jakob disease (CJD) but shares features of Gerstmann-Sträussler-Scheinker disease (GSS). However, contrary to exclusively inherited GSS, no prion protein (PrP) gene variations have been detected in VPSPr, suggesting that VPSPr might be the long-sought sporadic form of GSS. The VPSPr atypical features raised the issue of transmissibility, a prototypical property of prion diseases. We inoculated VPSPr brain homogenate into transgenic mice expressing various levels of human PrP (PrPC). On first passage, 54% of challenged mice showed histopathologic lesions, and 34% harbored abnormal PrP similar to that of VPSPr. Surprisingly, no prion disease was detected on second passage. We concluded that VPSPr is transmissible; thus, it is an authentic prion disease. However, we speculate that normal human PrPC is not an efficient conversion substrate (or mouse brain not a favorable environment) and therefore cannot sustain replication beyond the first passage. |
| Databáze: | Directory of Open Access Journals |
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