Prions in Humans and Animals

Frontmatter --
Table of Contents --
Abbreviations --
Topic I: History --
1. Historical Introduction --
2. History of Kuru Research --
3. History of Prion Research --
Topic II: Molecular Biology and Genetics --
4. The Physical Nature of the Prion --
5. Folding of the Recombinant Prion Protein --
6. Structural Studies of Prion Proteins --
7. Function of Cellular Prion Protein (PrPC) in Copper Homeostasis and Redox Signaling at the Synapse --
8. The Scarpie Isoform of the Prion Protein PrPSe Compared to the Celluar Isoform PrPC --
9. The Phylogeny of Mammalian and Nonmammalian Prion Proteins --
10. Knockouts and Transgenic Mice in Prion Research --
11. Transplantation as a Tool in Prion Research --
12. Prion Strains --
Topic III: Portraits of Prion Diseases --
13. Portrait of Kuru --
14. Portrait of Creutzfeldt-Jakob Disease --
15. Portrait of Variant Creutzfeldt-Jakob Disease --
16. Portrait of Gerstmann-Sträussler-Scheinker Disease --
17. Portrait of Fatal Familial Insomnia and Sporadic Fatal Insomnia --
18. Portrait of Scrapie in Sheep and Goat --
19. Portrait of Bovine Spongiform Encephalopathy in Cattle and Other Ungulates --
20. Portrait of Prion Diseases in Zoo Animals --
21. Portrait of Chronic Wasting Disease in Deer Species --
22. Portrait of Transmissible Mink Encephalopathy --
23. Portrait of Transmissible Feline Spongiform Encephalopathy --
24. Portrait of Experimental BSE in Pigs --
25. Portrait of a Spongiform Encephalopathy in Birds and the Transmissibility of Mammalian Prior Diseases to Birds --
Topic IV: Pathology --
26. Pathology and Genetics of Human Prion Diseases --
27. The Pathology of Prion Diseases in Animals --
28. Pathophysiology of Prion Diseases Following Peripheral Infection --
Topic V: Surveillance, Clinical Aspects and Diagnostics --
29. Introduction of Surveillance for Human Prion Diseases --
30. Clinical Findings in Human Prion Diseases --
31. Methods for the Clinical Diagnosis of Human Prion Deseases --
32. Introduction to Surveillance for Animal Prion Diseases --
33. Clinical Findings in Bovine Spongiform Encephalopathy --
34. Clinical Findings in Scrapie --
35. Diagnosis of Bovine Spongiform Encephalopathy by Immunological Methods --
Topic VI: Epidemiology --
36. Epidemiology and Risk Factors of Creutzfeldt-Jakob Disease --
37. Creutzfeldt-Jakob Disease in Germany --
38. The Epidemiology of Kuru --
39. The Course of the BSE Epidemic --
Retrospective Epidemiological Considerations --
40. The Causes of the BSE Epidemic --
Topic VII: Transmissibility --
41. The Experimental Transmissibility of Prions and Infectivity Distribution in the Body --
42. Iatrogenic and \"Natural\" Transmissibility of Prion Diseases --
Topic VIII: Agent Inactivation --
43. Inactivation in Practice --
Risk Assessment and Validation for Food Gelatin --
44. Chemical Disinfection and Inactivation of Prions --
45. Thermal Inactivation of Prions --
Topic IX: Prevention --
46. Prevention of Prion Diseases in the Production of Medicinal Products, Medical Devices, and Cosmetics --
47. Prevention of the Transmission of Prion Diseases in Healthcare Settings --
48. Precautionary Measures for Autopsies Performed in Cases of Suspected Prion Disease --
49. Prevention of Prion Diseases in Research Laboratories --
Topic X: Risk Assessment --
50. Evidence for a Link between Variant Creutzfeldt-Jakob Disease and Bovine Spongiform Encephalopathy --
51. Risk Assessment of Transmitting Prion Diseases through Blood, Cornea, and Dura Mater --
52. BSE Risk Assessment and Minimization --
53. BSE Control --
Internationally Recommended Approaches --
54. Atypical Scrapie-Nor98 --
55. Scrapie Control --
Internationally Recommended Approaches --
56. The PrP Genotype as a Marker for Scrapie Susceptibility in Sheep --
57. Scrapie control at the National level: The Norwegian Example --
Backmatter.