Recovery of small infectious PrP(res) aggregates from prion-infected cultured cells

J Biol Chem. 2011 Mar 11;286(10):8141-8148. doi: 10.1074/jbc.M110.165233. Epub 2011 Jan 6.

Abstract

Prion diseases are characterized by deposits of abnormal conformers of the PrP protein. Although large aggregates of proteinase K-resistant PrP (PrP(res)) are infectious, the precise relationships between aggregation state and infectivity remain to be established. In this study, we have fractionated detergent lysates from prion-infected cultured cells by differential ultracentrifugation and ultrafiltration and have characterized a previously unnoticed PrP species. This abnormal form is resistant to proteinase K digestion but, in contrast to typical aggregated PrP(res), remains in the soluble fraction at intermediate centrifugal forces and is not retained by filters of 300-kDa cutoff. Cell-based assay and inoculation to animals demonstrate that these entities are infectious. The finding that cell-derived small infectious PrP(res) aggregates can be recovered in the absence of strong in vitro denaturating treatments now gives a biological basis for investigating the role of small PrP aggregates in the pathogenicity and/or the multiplication cycle of prions.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Animals
  • Cell Line
  • Humans
  • Mice
  • Mice, Transgenic
  • PrPSc Proteins* / chemistry
  • PrPSc Proteins* / genetics
  • PrPSc Proteins* / isolation & purification
  • PrPSc Proteins* / metabolism
  • PrPSc Proteins* / pathogenicity
  • Prion Diseases / genetics
  • Prion Diseases / metabolism*
  • Sheep

Substances

  • PrPSc Proteins