Replication of prions in differentiated muscle cells

Abstract

We have demonstrated that prions accumulate to high levels in nonproliferative C2C12 myotubes. C2C12 cells replicate as myoblasts but can be differentiated into myotubes. Earlier studies indicated that C2C12 myoblasts are not competent for prion replication.1 We confirmed that observation anddemonstrated, for the first time, that while replicative myoblasts do not accumulate PrPSc, differentiated postmitotic myotube cultures replicate prions robustly. Here we extend our observations and describe the implication and utility of this system for replicating prions.