After initial skepticism, it is now generally accepted that prions exist, that they differ from all other pathogens, and that they are infectious in several species. Prions are implicated in spongiform encephalopathies such as kuru, Creutzfeldt-Jakob disease and fatal familial insomnia in humans, scrapie in sheep and a bovine encephalopathy that may be transmissible to humans. Driven by prions' novelty and by concern about their public health effects, awareness of these pathogens has grown enormously, prompting an immense amount of sometimes conflicting research. This text aims to present an authoritative account of prion biology and disease, offering scientists, educators and students a source of reliable reference material.

Preface Prion Biology 1 An Introduction to Prion Biology and Diseases 1 2 Development of the Prion Concept 67 3 Bioassays of Prions 113 4 Transmission and Replication of Prions 147 5 Structural Studies of Prion Proteins 191 Fungal Prions 6 Prions of Fungi: [URE3], [PSI], and [Het-s] Discovered as Heritable Traits 229 Transgenetics and Cell Biology of Prions 7 Knockouts, Transgenics, and Transplants in Prion Research 273 8 Transgenetic Investigations of the Species Barrier and Prion Strains 307 9 Cell Biology of Prions 349 Prion Diseases in Animals 10 Scrapie, Chronic Wasting Disease, and Transmissible Mink Encephalopathy 393 11 Bovine Spongiform Encephalopathy and Related Diseases 431 Human Prion Diseases 12 Infectious and Sporadic Prion Diseases 465 13 Inherited Prion Diseases 509 14 Neuropathology of Prion Diseases 585 Methodology and Future Studies 15 Some Strategies and Methods for the Study of Prions 653 16 Antibodies as Tools to Probe Prion Protein Biology 717 17 Biosafety Issues in Prion Diseases 743 Index 779