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40 reviewsBovine spongiform encephalopathy (BSE) has become the most publicly recognizable example of a group of fatal neurodegenerative diseases caused by proteinaceous infectious particles called prions. Transmission of bovine spongiform encephalopathy to humans in the form of variant Creutzfeldt-Jakob disease (vCJD) has intensified the urgency of understanding and controlling these unusual disorders. The contributors to this volume, all internationally recognized experts in their fields, provide an introduction to prion biology, followed by reviews of the latest information on BSE, vCJD, and chronic wasting disease, an animal prion disease that has recently emerged in North America.