Novel Reagent Development for Chronic Wasting Disease

Chronic wasting disease (CWD) in elk and deer, and scrapie in sheep are a family of degenerative, central nervous system diseases known as transmissible spongiform encephalopathies (TSEs) or prion diseases. They are often called spongiform because of the post mortem appearance of hole-like structures called vacuoles in the cortex and cerebellum of the brain. Chronic wasting disease is the result of the accumulation in the brain of a normal cell surface-expressed protein, called PrP or PrPsen, that has re-folded (fromnalpha-helical to beta-sheet) and aggregated into an entity, PrPres, that interrupts normal neuronal activity. The accumulation of PrPres is considered to be directly responsible for the onset of the pathology observed with CWD and is also considered the main cause of other TSEs. To date, no suitable antibody reagents are available that can distinguish between the normal and the pathological form of the prion protein in CWD, and there is urgency and a significant requirement in the marketplace for reagents of this nature. Current tests for CWD are lengthy, involve extensive pre-treatment and are only available as "dead animal" tests. An opportunity exists for New England Rare Reagents to produce specific antibodies to CWD PrPres. The benefit from such a reagent will allow for more specific and less labor-intensive testing to be performed. These improvements should make deer and elk testing more available to state facilities attempting to control and eventually eradicate the disease.