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Publication : High titers of transmissible spongiform encephalopathy infectivity associated with extremely low levels of PrPSc in vivo.

First Author  Barron RM Year  2007
Journal  J Biol Chem Volume  282
Issue  49 Pages  35878-86
PubMed ID  17923484 Mgi Jnum  J:129030
Mgi Id  MGI:3768510 Doi  10.1074/jbc.M704329200
Citation  Barron RM, et al. (2007) High titers of transmissible spongiform encephalopathy infectivity associated with extremely low levels of PrPSc in vivo. J Biol Chem 282(49):35878-86
abstractText  Diagnosis of transmissible spongiform encephalopathy (TSE) disease in humans and ruminants relies on the detection in post-mortem brain tissue of the protease-resistant form of the host glycoprotein PrP. The presence of this abnormal isoform (PrP(Sc)) in tissues is taken as indicative of the presence of TSE infectivity. Here we demonstrate conclusively that high titers of TSE infectivity can be present in brain tissue of animals that show clinical and vacuolar signs of TSE disease but contain low or undetectable levels of PrP(Sc). This work questions the correlation between PrP(Sc) level and the titer of infectivity and shows that tissues containing little or no proteinase K-resistant PrP can be infectious and harbor high titers of TSE infectivity. Reliance on protease-resistant PrP(Sc) as a sole measure of infectivity may therefore in some instances significantly underestimate biological properties of diagnostic samples, thereby undermining efforts to contain and eradicate TSEs.
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