Details:

Alternative Title:
Emerg Infect Dis
Publisher's site:
http://dx.doi.org/10.3201/eid2012.140548
Personal Author:
Notari, Silvio ; Xiao, Xiangzhu ; Espinosa, Juan Carlos ; Cohen, Yvonne ; Qing, Liuting ; ... More +
Notari, Silvio ; Xiao, Xiangzhu ; Espinosa, Juan Carlos ; Cohen, Yvonne ; Qing, Liuting ; Aguilar-Calvo, Patricia ; Kofskey, Diane ; Cali, Ignazio ; Cracco, Laura ; Kong, Qingzhong ; Torres, Juan Maria ; Zou, Wenquan ; Gambetti, Pierluigi Less -
Description:
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 (PrP(C)). 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 PrP(C) is not an efficient conversion substrate (or mouse brain not a favorable environment) and therefore cannot sustain replication beyond the first passage.
Subject:
[+]
Humans
Prions And Related Diseases
Research
Transgenic Mice
Transmissibility
Transmission Characteristics Of Variably Protease-Sensitive Prionopathy
VPSPr

Source:
Emerg Infect Dis. 20(12):2006-2014.
Document Type:
Journal Article
Collection(s):
Emerging Infectious Diseases
Main Document Checksum:
[+]
urn:sha256:7c751852863196a86e331f47582878ad0c415116cdda5cf91631e33535b60753
File Type:

Supporting Files

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