NC1024: Domestic Surveillance, Diagnosis, And Therapy Of Transmissible Spongiform Encephalopathies (NC505)
Statement of Issues and Justification
Transmissible spongiform encephalopathies represent a family of emerging, potentially zoonotic diseases affecting US Agriculture and Wildlife management. While most of these disorders are species-specific, they have the potential to cause massive economic losses due to general concern over the potential consequences of a zoonotic spread. This was clearly demonstrated when Bovine Spongiform Encephalopathy was found to cause a novel disease in humans. Although at least one of these diseases (Scrapie) has been recognized for more than two centuries, it has only recently been demonstrated that the infectious agent is a new, little understood variant of a normal cellular protein known as a prion. Most of these prion diseases appear to be species specific and the general nature of the disease pathogenesis appears to be highly conserved across species. While the United States has in the past had incidences of prion diseases affecting sheep, elk, deer, and mink, the zoonotic bovine form (BSE) was only recently identified within the North American cattle herd. Due to the devastating effect of BSE on the European beef and cattle industries, a strong network of prion researchers currently exists within the European Community. Until recently most research in the United States was confined to TSEs affecting individual species, with studies conducted by individual investigators focused on specifically defined problems. These individual research projects focused primarily on molecular, cellular, physiologic or epidemiologic problems, with limited integration. It is the broad, long-term goal of this proposal to form a co-operative of basic and applied researchers focused on animal prion diseases that will rapidly and markedly expand the collaboration and focus of US-based TSE research. The goal of this proposal is to develop and maintain that activity and provide immediate support in the form of new research towards understanding, controlling, and responding to prion diseases such as BSE.
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