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To identify and differentiate typical and atypical case samples of CWD and Scrapie by characterizing the biologic phenotype and pathologic profile of these agents when administered to susceptible lines of transgenic and inbred mice.
APPROACH: Tissue samples from deer, elk, sheep and goats with Transmissible Spongiform Encephalopathy (TSE) will be administered to mice by intracerebral injection. Multiple tissue types will be included, such as samples of brain, lymph node, blood, urine, feces, antler velvet and muscle. Mouse models used as recipient hosts will include both preexisting and recently created transgenic and inbred mouse lines. Recipient mouse phenotype will be evaluated by measuring clinical response, population disease rate, incubation time, and pathologic profile within the central nervous system (CNS). Pathologic profile of CNS lesion foci is assessed by evaluating anatomic localization, spongiform change, astrocytic gliosis, and deposition of protease resistant prion protein. BSL-1; 9-4-06. Documents SCA with U. of WA.