1;Frontmatter;1 2;Table of Contents;15 3;Abbreviations;25 4;1. Historical Introduction;31 5;2. History of Kuru Research;56 6;3. History of Prion Research;72 7;4. The Physical Nature of the Prion;87 8;5. Folding of the Recombinant Prion Protein;97 9;6. Structural Studies of Prion Proteins;107 10;7. Function of Cellular Prion Protein (PrPC) in Copper Homeostasis and Redox Signaling at the Synapse;123 11;8. The Scarpie Isoform of the Prion Protein PrPSe Compared to the Celluar Isoform PrPC;132 12;9. The Phylogeny of Mammalian and Nonmammalian Prion Proteins;147 13;10. Knockouts and Transgenic Mice in Prion Research ;162 14;11. Transplantation as a Tool in Prion Research;188 15;12. Prion Strains;194 16;13. Portrait of Kuru;215 17;14. Portrait of Creutzfeldt-Jakob Disease;223 18;15. Portrait of Variant Creutzfeldt-Jakob Disease ;232 19;16. Portrait of Gerstmann-Sträussler-Scheinker Disease;238 20;17. Portrait of Fatal Familial Insomnia and Sporadic Fatal Insomnia;244 21;18. Portrait of Scrapie in Sheep and Goat;250 22;19. Portrait of Bovine Spongiform Encephalopathy in Cattle and Other Ungulates;261 23;20. Portrait of Prion Diseases in Zoo Animals;278 24;21. Portrait of Chronic Wasting Disease in Deer Species;285 25;22. Portrait of Transmissible Mink Encephalopathy;293 26;23. Portrait of Transmissible Feline Spongiform Encephalopathy;299 27;24. Portrait of Experimental BSE in Pigs;303 28;25. Portrait of a Spongiform Encephalopathy in Birds and the Transmissibility of Mammalian Prior Diseases to Birds;307 29;26. Pathology and Genetics of Human Prion Diseases;315 30;27. The Pathology of Prion Diseases in Animals;343 31;28. Pathophysiology of Prion Diseases Following Peripheral Infection;356 32;29. Introduction of Surveillance for Human Prion Diseases;367 33;30. Clinical Findings in Human Prion Diseases;375 34;31. Methods for the Clinical Diagnosis of Human Prion Deseases ;391 35;32. Introduction to Surveillance for Animal Prion Diseases;410 36;33. Clinical Findings in Bovine Spongiform Encephalopathy;417 37;34. Clinical Findings in Scrapie;426 38;35. Diagnosis of Bovine Spongiform Encephalopathy by Immunological Methods;436 39;36. Epidemiology and Risk Factors of Creutzfeldt-Jakob Disease;451 40;37. Creutzfeldt-Jakob Disease in Germany;461 41;38. The Epidemiology of Kuru;468 42;39. The Course of the BSE Epidemic - Retrospective Epidemiological Considerations;477 43;40. The Causes of the BSE Epidemic;492 44;41. The Experimental Transmissibility of Prions and Infectivity Distribution in the Body;501 45;42. Iatrogenic and "Natural" Transmissibility of Prion Diseases;511 46;43. Inactivation in Practice - Risk Assessment and Validation for Food Gelatin;527 47;44. Chemical Disinfection and Inactivation of Prions;532 48;45. Thermal Inactivation of Prions;543 49;46. Prevention of Prion Diseases in the Production of Medicinal Products, Medical Devices, and Cosmetics;557 50;47. Prevention of the Transmission of Prion Diseases in Healthcare Settings;574 51;48. Precautionary Measures for Autopsies Performed in Cases of Suspected Prion Disease;589 52;49. Prevention of Prion Diseases in Research Laboratories;593 53;50. Evidence for a Link between Variant Creutzfeldt-Jakob Disease and Bovine Spongiform Encephalopathy;601 54;51. Risk Assessment of Transmitting Prion Diseases through Blood, Cornea, and Dura Mater ;607 55;52. BSE Risk Assessment and Minimization;629 56;53. BSE Control - Internationally Recommended Approaches;648 57;54. Atypical Scrapie-Nor98;658 58;55. Scrapie Control - Internationally Recommended Approaches ;663 59;56. The PrP Genotype as a Marker for Scrapie Susceptibility in Sheep;668 60;57. Scrapie control at the National level: The Norwegian Example;676