Backmatter

Chapters in this book

1. Frontmatter I
2. Table of Contents XV
3. Abbreviations XXV
4. Topic I: History
5. 1. Historical Introduction 3
6. 2. History of Kuru Research 28
7. 3. History of Prion Research 44
8. Topic II: Molecular Biology and Genetics
9. 4. The Physical Nature of the Prion 59
10. 5. Folding of the Recombinant Prion Protein 69
11. 6. Structural Studies of Prion Proteins 79
12. 7. Function of Cellular Prion Protein (PrPC) in Copper Homeostasis and Redox Signaling at the Synapse 95
13. 8. The Scarpie Isoform of the Prion Protein PrPSe Compared to the Celluar Isoform PrPC 104
14. 9. The Phylogeny of Mammalian and Nonmammalian Prion Proteins 119
15. 10. Knockouts and Transgenic Mice in Prion Research 134
16. 11. Transplantation as a Tool in Prion Research 160
17. 12. Prion Strains 166
18. Topic III: Portraits of Prion Diseases
19. 13. Portrait of Kuru 187
20. 14. Portrait of Creutzfeldt-Jakob Disease 195
21. 15. Portrait of Variant Creutzfeldt-Jakob Disease 204
22. 16. Portrait of Gerstmann-Sträussler-Scheinker Disease 210
23. 17. Portrait of Fatal Familial Insomnia and Sporadic Fatal Insomnia 216
24. 18. Portrait of Scrapie in Sheep and Goat 222
25. 19. Portrait of Bovine Spongiform Encephalopathy in Cattle and Other Ungulates 233
26. 20. Portrait of Prion Diseases in Zoo Animals 250
27. 21. Portrait of Chronic Wasting Disease in Deer Species 257
28. 22. Portrait of Transmissible Mink Encephalopathy 265
29. 23. Portrait of Transmissible Feline Spongiform Encephalopathy 271
30. 24. Portrait of Experimental BSE in Pigs 275
31. 25. Portrait of a Spongiform Encephalopathy in Birds and the Transmissibility of Mammalian Prior Diseases to Birds 279
32. Topic IV: Pathology
33. 26. Pathology and Genetics of Human Prion Diseases 287
34. 27. The Pathology of Prion Diseases in Animals 315
35. 28. Pathophysiology of Prion Diseases Following Peripheral Infection 328
36. Topic V: Surveillance, Clinical Aspects and Diagnostics
37. 29. Introduction of Surveillance for Human Prion Diseases 339
38. 30. Clinical Findings in Human Prion Diseases 347
39. 31. Methods for the Clinical Diagnosis of Human Prion Deseases 363
40. 32. Introduction to Surveillance for Animal Prion Diseases 382
41. 33. Clinical Findings in Bovine Spongiform Encephalopathy 389
42. 34. Clinical Findings in Scrapie 398
43. 35. Diagnosis of Bovine Spongiform Encephalopathy by Immunological Methods 408
44. Topic VI: Epidemiology
45. 36. Epidemiology and Risk Factors of Creutzfeldt-Jakob Disease 423
46. 37. Creutzfeldt-Jakob Disease in Germany 433
47. 38. The Epidemiology of Kuru 440
48. 39. The Course of the BSE Epidemic - Retrospective Epidemiological Considerations 449
49. 40. The Causes of the BSE Epidemic 464
50. Topic VII: Transmissibility
51. 41. The Experimental Transmissibility of Prions and Infectivity Distribution in the Body 473
52. 42. Iatrogenic and "Natural" Transmissibility of Prion Diseases 483
53. Topic VIII: Agent Inactivation
54. 43. Inactivation in Practice - Risk Assessment and Validation for Food Gelatin 499
55. 44. Chemical Disinfection and Inactivation of Prions 504
56. 45. Thermal Inactivation of Prions 515
57. Topic IX: Prevention
58. 46. Prevention of Prion Diseases in the Production of Medicinal Products, Medical Devices, and Cosmetics 529
59. 47. Prevention of the Transmission of Prion Diseases in Healthcare Settings 546
60. 48. Precautionary Measures for Autopsies Performed in Cases of Suspected Prion Disease 561
61. 49. Prevention of Prion Diseases in Research Laboratories 565
62. Topic X: Risk Assessment
63. 50. Evidence for a Link between Variant Creutzfeldt-Jakob Disease and Bovine Spongiform Encephalopathy 573
64. 51. Risk Assessment of Transmitting Prion Diseases through Blood, Cornea, and Dura Mater 579
65. 52. BSE Risk Assessment and Minimization 601
66. 53. BSE Control - Internationally Recommended Approaches 620
67. 54. Atypical Scrapie-Nor98 630
68. 55. Scrapie Control - Internationally Recommended Approaches 635
69. 56. The PrP Genotype as a Marker for Scrapie Susceptibility in Sheep 640
70. 57. Scrapie control at the National level: The Norwegian Example 648
71. Backmatter 655