Infectious prions accumulate to high levels in non proliferative C2C12 myotubes

PLoS Pathog. 2013;9(11):e1003755. doi: 10.1371/journal.ppat.1003755. Epub 2013 Nov 7.

Abstract

Prion diseases are driven by the strain-specific, template-dependent transconformation of the normal cellular prion protein (PrP(C)) into a disease specific isoform PrP(Sc). Cell culture models of prion infection generally use replicating cells resulting in lower levels of prion accumulation compared to animals. Using non-replicating cells allows the accumulation of higher levels of PrP(Sc) and, thus, greater amounts of infectivity. Here, we infect non-proliferating muscle fiber myotube cultures prepared from differentiated myoblasts. We demonstrate that prion-infected myotubes generate substantial amounts of PrP(Sc) and that the level of infectivity produced in these post-mitotic cells, 10(5.5) L.D.50/mg of total protein, approaches that observed in vivo. Exposure of the myotubes to different mouse-adapted agents demonstrates strain-specific replication of infectious agents. Mouse-derived myotubes could not be infected with hamster prions suggesting that the species barrier effect is intact. We suggest that non-proliferating myotubes will be a valuable model system for generating infectious prions and for screening compounds for anti-prion activity.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Animals
  • Cell Line
  • Cell Proliferation*
  • Cricetinae
  • Mice
  • Muscle Fibers, Skeletal
  • PrPSc Proteins / metabolism*
  • Species Specificity

Substances

  • PrPSc Proteins

Associated data

  • GEO/GSE44563

Grants and funding

This work was supported by the Alberta Prion Research Institute and PrioNet (http://www.prioninstitute.ca/ and http://www.prionetcanada.ca/). The funders had no role in study design, data collection and analysis, decision to publish, or preparation of the manuscript.