Variable Protease-Sensitive Prionopathy Transmission to Bank Voles.

ABSTRACT: Variably protease-sensitive prionopathy (VPSPr), a recently described human sporadic prion disease, features a protease-resistant, disease-related prion protein (resPrP^D) displaying 5 fragments reminiscent of Gerstmann-Sträussler-Scheinker disease. Experimental VPSPr transmission to human PrP-expressing transgenic mice, although replication of the VPSPr resPrP^D profile succeeded, has been incomplete because of second passage failure. We bioassayed VPSPr in bank voles, which are susceptible to human prion strains. Transmission was complete; first-passage attack rates were 5%-35%, and second-passage rates reached 100% and survival times were 50% shorter. We observed 3 distinct phenotypes and resPrP^D profiles; 2 imitated sporadic Creutzfeldt-Jakob disease resP

SUBMITTER: Nonno R 

PROVIDER: S-EPMC6302590 | biostudies-literature | 2019 Jan

REPOSITORIES: biostudies-literature