Prions in Humans and Animals (eBook)

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Beat Hörnlimann, SVISS Consulting, Steinhausen, Switzerland; Detlev Riesner, Heinrich-Heine-Universität, Düsseldorf, Germany; Hans Kretzschmar, Ludwig-Maximilians-Universität, München, Germany.

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

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Reviews of the German previous edition of “Prionen und Prionen Krankheiten”:

"The book provides a thorough overview of current prion research. It contains numerous instructive figures and tables. Expert readers also find information that is otherwise difficult to obtain. Another highlight of the book is the practical advice provided, for example the list of chemical disinfection methods that have proven effective in prion decontamination. The book is an invaluable and comprehensive source of information which can either be read through from cover to cover, or used as a reference book.
Prof Dr. Wolfgang Schlote in: Wissenschaftsmagazin Frankfurt, 4/2002

"A very interesting book with a huge amount of information."
Berliner Heilpraktiker Nachrichten, October/November 2001

"The present book is, in my opinion, the best German-language book on prions and prion diseases currently available. [...] The book is written in a way that it can be understood by a wide range of readers. It is both a textbook as well as a scientific reference book. Unfortunately, space limitations don’t allow me to discuss the book in greater detail. Nevertheless, I can recommend it wholeheartedly to all people interested in prion diseases – physicians, veterinarians and nutrition scientists, as well as the general public.
Prof. Dr. Norbert Nowotny in: Ernährung, Vienna, June 2002

Rezensionen der deutschen Auflage:

"Das Buch gibt durchweg den aktuellen Stand der Forschung wieder. Es ist mit zahlreichen sehr instruktiven Abbildungen und Tabellen versehen. Auch der kundige Leser findet Angaben, die sonst kaum oder nur mit Mühe zu finden sind. Ein weiterer Pluspunkt des Buches ist, dass es auch praktische Hinweise enthält, wie beispielsweise eine Liste der wirksamen chemischen Desinfektionsverfahren bei Prionenkrankheiten. das Buch stellt eine unschätzbare und umfassende Informationsquelle das; man kann es sowohl als 'Lesebuch' wie auch als Nachschlagewerk benutzen; es ist ohne Einschränkung zu empfehlen."
Prof Dr. Wolfgang Schlote in: Wissenschaftsmagazin Frankfurt, 4/2002

"Ein hochinteressantes Buch mit einer Fülle an Informationen."
Berliner Heilpraktiker Nachrichten, Oktober/November 2001

"Das vorliegende Werk stellt meiner Ansicht nach das derzeit beste deutschsprachige Buch über Prionen und Prionkrankheiten dar.[...] Das Buch ist in einer Weise verfasst, die für einen sehr weiten Leserkreis verständlich ist. Es ist sowohl Lehrbuch als auch wissenschaftliches Nachschlagewerk. Es ist an dieser Stelle leider nicht möglich, näher auf das Buch einzugehen, ich empfehle es jedoch wärmstens allen an Prionenkrankheiten Interessierten, Ärzten, Tierärzten und Ernährungswissenschaftlern genauso wie Politikern, Journalisten und interessierten Laien."
Prof. Dr. Norbert Nowotny in: Ernährung, Wien, Juni 2002