Journal
EMERGING INFECTIOUS DISEASES
Volume 20, Issue 12, Pages 2006-2014Publisher
CENTERS DISEASE CONTROL
DOI: 10.3201/eid2012.140548
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Funding
- National Institutes of Health [AG14359, NS062787]
- Centers for Disease Control and Prevention [UR8/CCU515004]
- Charles S. Britton Fund
- Spanish Ministerio de Ciencia e Innovacion [AGL2012-37988-C04-04]
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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-Straussler-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.
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