The biology and molecular biology of scrapie-like diseases

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Abstract
The transmissible spongiform encephalopathies (TSE’s) are degenerative diseases of the central nervous system which naturally affect man (Creutzfeldt-Jakob disease [CJD], Gerstmann-Straussler syndrome [GSS], kuru), sheep and goats (scrapie), cattle (bovine spongiform encephalopathy [BSE]), mink (transmissible mink encephalopathy), mule deer, elk and antelope (chronic wasting disease). Spongiform encephalopathies have also been diagnosed in captive species of zoo antelope and in domestic cats. Much has been written about these maladies in the wake of the BSE outbreak, the tragic cases of CJD in recipients of cadaver-derived human growth hormone, sex hormones or dura mater and this has stimulated a continuing public health debate about the transmissibility, prevalence and clinical variability of scrapie, CJD and related (“prion”) diseases. Prions (Weissmann, Liautard, this volume) and the human (Kretzschmar, this volume) and cattle (Wilesmith, Marsh, this volume) diseases are described in more detail elsewhere. This article presents a brief overview of the biology and molecular cell biology of scrapie and rodent models of these diseases.