Rapid and Early Detection of Prions

Transmissible spongiform encephalopathies (TSEs), or prion diseases, are caused by a unique transmissible agent hypothesized to be a misfolded form (PrPD) of a normal host protein (PrPC). We will use chronic wasting disease (CWD) as a model prion agent because there is an urgent need for increased research on this agent, our company is positioned to capitalize on an existing customer base, and CWD has significant biosafety advantages compared to other prion agents. The objectives of this proposal are to 1) establish a library of cervid cell culture lines and 2) demonstrate prion replication in one or more of these lines. We will accomplish these objectives by utilizing a strategy of selecting cell lines based solely upon their ability to support prion replication quickly and sensitively. This assay will be used for antemortem diagnosis of prion disease as well as a detection method for prion contamination in blood, food, pharmaceutical supplies, and the environment. The development of a sensitive cell-based bioassay for CWD will be a substantial commercial product. More significantly, the method demonstrated in this proposal will be quickly adapted for use with other prion agents and will drastically broaden the potential market for such an assay.