| First Author | Herbst A | Year | 2013 |
| Journal | PLoS Pathog | Volume | 9 |
| Issue | 11 | Pages | e1003755 |
| PubMed ID | 24244171 | Mgi Jnum | J:246992 |
| Mgi Id | MGI:5919388 | Doi | 10.1371/journal.ppat.1003755 |
| Citation | Herbst A, et al. (2013) Infectious prions accumulate to high levels in non proliferative C2C12 myotubes. PLoS Pathog 9(11):e1003755 |
| abstractText | 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. |
