Rapid and Early Detection of Prions

Transmissible spongiform encephalopathies (TSE’s), 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 of growing concern about 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 specific objectives for Phase II are: 1) Select subclones of cell lines developed in Phase I that rapidly produce high levels of PrPD. 2) Define and characterize the selected subclones growth in culture, cell type, immortalization status, and ability to support CWD prion propagation. 3) Optimize detection method and validate detection of CWD prions in a prototype assay design. We will accomplish these objectives by 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. This cell-based bioassay for CWD will be a substantial commercial product.