Scrapie strains maintain biological phenotypes on propagation in a cell line in culture

EMBO J. 2001 Jul 2;20(13):3351-8. doi: 10.1093/emboj/20.13.3351.

Abstract

Bovine spongiform encephalopathy (BSE) and its human equivalent, variant Creutzfeldt-Jakob disease (vCJD), are caused by the same strain of infectious agent, which is similar to, but distinct from, >20 strains of their sheep scrapie homologue. A better understanding of the molecular strain determinants could be obtained from cells in monoculture than from whole animal studies where different cell targeting is commonly a strain-related feature. Although a few cell types can be infected with different strains, the phenotypes of the emergent strains have not been studied. We have cured the scrapie-infected, clonal SMB cell line with pentosan sulfate, stably re-infected it with a different strain of scrapie and shown that biological properties and prion protein profiles characteristic of each original strain are propagated faithfully in this single non-neuronal cell type. These findings attest to the fact that scrapie strain determinants are stable and host-independent in isolated cells.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Animals
  • Brain / metabolism
  • Cattle
  • Cell Line
  • Creutzfeldt-Jakob Syndrome / etiology
  • Encephalopathy, Bovine Spongiform / etiology
  • Humans
  • Mice
  • Phenotype
  • PrPSc Proteins / biosynthesis
  • PrPSc Proteins / genetics*
  • Protein Isoforms / biosynthesis
  • Protein Isoforms / genetics
  • Protein Isoforms / physiology
  • Scrapie / etiology*
  • Scrapie / physiopathology

Substances

  • PrPSc Proteins
  • Protein Isoforms