Prions in Humans and Animals

Published by De Gruyter 2006

Prions in Humans and Animals

Edited by: Beat Hörnlimann , Detlev Riesner and Hans A. Kretzschmar

https://doi.org/10.1515/9783110200171

Overview
Contents

About this book

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This comprehensive work, aimed at both students and researchers alike, systematically covers all aspects of prion diseases (transmissible spongiform encephalopathies), from their history, microbiology and pathology to their transmissibility and prevention. The book describes diseases such as Creutzfeldt-Jakob disease, kuru, mad cow disease (BSE), chronic wasting disease and scrapie, highlighting their biochemical, molecular biological, genetic, and clinical aspects. A detailed presentation of the impact of prion diseases in fields such as pharmaceutics, blood products, disinfection, surgical instruments and epidemiology concludes with a discussion of preventive measures.

A renowned editorial team, representing the fields of medicine, veterinary medicine and molecular biology, brought together 80 internationally respected authors for this translation and new edition of the successful German publication, not only from relevant research fields, but also from industry and public health institutions. The book includes chapters by, among many other notable scientists, William J. Hadlow, who discovered the relationship between the human and animal forms of prion diseases and Michael P. Alpers, with 45 years of experience in Papua New Guinea investigating the first known human epidemic form, kuru, transmitted by endocannibalism. Further contributions from Gerald A. H. Wells, a veterinary pathologist who described BSE and recognised its similarity to scrapie, thus recording the first cases in 1986 of the most important animal epidemic of modern times, and Robert G. Will, a medical neurologist and epidemiologist who discovered the emergence of the variant form of Creutzfeldt-Jakob disease in 1996, underscore the strength of this author team.

Carefully edited with numerous illustrations, this work offers a systematic approach committed to a clear presentation of the current knowledge of prion diseases. It aims to inspire and stimulate interdisciplinary cooperation, innovative research ideas and effective prevention.

Author / Editor information

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

Reviews

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

Topics

Frontmatter Requires Authentication Unlicensed Licensed Download PDF	I
Table of Contents Requires Authentication Unlicensed Licensed Download PDF	XV
Abbreviations Requires Authentication Unlicensed Licensed Download PDF	XXV
Topic I: History
1. Historical Introduction Beat Hörnlimann, Detlev Riesner, Hans Kretschmar, Robert G. Will, Stuart C. MacDiarmid, Gerald A. H. Wells and Michael P. Alpers Requires Authentication Unlicensed Licensed Download PDF	3
2. History of Kuru Research Beat Hörnlimann Requires Authentication Unlicensed Licensed Download PDF	28
3. History of Prion Research Stanley B. Prusiner Requires Authentication Unlicensed Licensed Download PDF	44
Topic II: Molecular Biology and Genetics
4. The Physical Nature of the Prion Detlev Riesner Requires Authentication Unlicensed Licensed Download PDF	59
5. Folding of the Recombinant Prion Protein Rudi Glockshuber, Jan Stöhr and Detlev Riesner Requires Authentication Unlicensed Licensed Download PDF	69
6. Structural Studies of Prion Proteins Stephan Schwarzinger, Dieter Willhold and Jan Ziegler Requires Authentication Unlicensed Licensed Download PDF	79
7. Function of Cellular Prion Protein (PrPC) in Copper Homeostasis and Redox Signaling at the Synapse Jochen Herms and Hans Kretschmar Requires Authentication Unlicensed Licensed Download PDF	95
8. The Scarpie Isoform of the Prion Protein PrPSe Compared to the Celluar Isoform PrPC Detlev Riesner Requires Authentication Unlicensed Licensed Download PDF	104
9. The Phylogeny of Mammalian and Nonmammalian Prion Proteins Herrmann M. Schätzl Requires Authentication Unlicensed Licensed Download PDF	119
10. Knockouts and Transgenic Mice in Prion Research Eckhard Flechsig, Ivan Hegyi, Alex J. Raeber, Antiono Cozzio, Adriano Aguzzi and Charles Weissmann Requires Authentication Unlicensed Licensed Download PDF	134
11. Transplantation as a Tool in Prion Research Eckhard Flechsig, Ivan Hegyi, Alex J. Raeber, Antiono Cozzio, Adriano Aguzzi and Charles Weissmann Requires Authentication Unlicensed Licensed Download PDF	160
12. Prion Strains Martin H. Groschup, Anja Gretzschel and Thomas Kuczius Requires Authentication Unlicensed Licensed Download PDF	166
Topic III: Portraits of Prion Diseases
13. Portrait of Kuru Beat Hörnlimann and Michael P. Alpers Requires Authentication Unlicensed Licensed Download PDF	187
14. Portrait of Creutzfeldt-Jakob Disease Herbert Budka Requires Authentication Unlicensed Licensed Download PDF	195
15. Portrait of Variant Creutzfeldt-Jakob Disease Robert G. Will and James W. Ironside Requires Authentication Unlicensed Licensed Download PDF	204
16. Portrait of Gerstmann-Sträussler-Scheinker Disease Herbert Budka Requires Authentication Unlicensed Licensed Download PDF	210
17. Portrait of Fatal Familial Insomnia and Sporadic Fatal Insomnia Herbert Budka and Ellen Gelpi Requires Authentication Unlicensed Licensed Download PDF	216
18. Portrait of Scrapie in Sheep and Goat Beat Hörnlimann, Lucien Keulen, Martha J. Ulvund and Ray Bradley Requires Authentication Unlicensed Licensed Download PDF	222
19. Portrait of Bovine Spongiform Encephalopathy in Cattle and Other Ungulates Beat Hörnlimann, Jutta Bachmann and Ray Bradley Requires Authentication Unlicensed Licensed Download PDF	233
20. Portrait of Prion Diseases in Zoo Animals James K. Kirkwood and Andrew A. Cunningham Requires Authentication Unlicensed Licensed Download PDF	250
21. Portrait of Chronic Wasting Disease in Deer Species Elizabeth S. Williams and Michael W. Miller Requires Authentication Unlicensed Licensed Download PDF	257
22. Portrait of Transmissible Mink Encephalopathy William J. Hadlow Requires Authentication Unlicensed Licensed Download PDF	265
23. Portrait of Transmissible Feline Spongiform Encephalopathy Marion Hewicker-Trauwein and Ray Bradley Requires Authentication Unlicensed Licensed Download PDF	271
24. Portrait of Experimental BSE in Pigs Gerald A. H. Wells, Stephen A. C. Hawkins, Joachim Pohlenz and Danny Matthews Requires Authentication Unlicensed Licensed Download PDF	275
25. Portrait of a Spongiform Encephalopathy in Birds and the Transmissibility of Mammalian Prior Diseases to Birds Gerald A. H. Wells, Joachim Pohlenz, Stephen A. C. Hawkins and Danny Matthews Requires Authentication Unlicensed Licensed Download PDF	279
Topic IV: Pathology
26. Pathology and Genetics of Human Prion Diseases Hans Kretzschmar and Piero Parchi Requires Authentication Unlicensed Licensed Download PDF	287
27. The Pathology of Prion Diseases in Animals Gerald A. H. Wells, Stephen J. Ryder and William J. Hadlow Requires Authentication Unlicensed Licensed Download PDF	315
28. Pathophysiology of Prion Diseases Following Peripheral Infection Walter J. Schulz-Schaeffer, Hans Kretzschmar and Michael Beekes Requires Authentication Unlicensed Licensed Download PDF	328
Topic V: Surveillance, Clinical Aspects and Diagnostics
29. Introduction of Surveillance for Human Prion Diseases Beat Hörnlimann, Hans Kretzschmar, Robert G. Will, Otto Windl and Herbert Budka Requires Authentication Unlicensed Licensed Download PDF	339
30. Clinical Findings in Human Prion Diseases Matthias Sturzenegger and Robert G. Will Requires Authentication Unlicensed Licensed Download PDF	347
31. Methods for the Clinical Diagnosis of Human Prion Deseases Inga Zerr Requires Authentication Unlicensed Licensed Download PDF	363
32. Introduction to Surveillance for Animal Prion Diseases Beat Hörnlimann, Marcus G. Doherr, Danny Matthews and Stuart C. MacDiarmid Requires Authentication Unlicensed Licensed Download PDF	382
33. Clinical Findings in Bovine Spongiform Encephalopathy Ernst Schicker, Ueli Braun, Beat Hörnlimann and Timm Konold Requires Authentication Unlicensed Licensed Download PDF	389
34. Clinical Findings in Scrapie Martha J. Ulvund Requires Authentication Unlicensed Licensed Download PDF	398
35. Diagnosis of Bovine Spongiform Encephalopathy by Immunological Methods Alex J. Raeber, Markus Moser and Bruno Oesch Requires Authentication Unlicensed Licensed Download PDF	408
Topic VI: Epidemiology
36. Epidemiology and Risk Factors of Creutzfeldt-Jakob Disease Inga Zerr and Sigrid Poser Requires Authentication Unlicensed Licensed Download PDF	423
37. Creutzfeldt-Jakob Disease in Germany Inga Zerr, Sigrid Poser and Hans Kretzschmar Requires Authentication Unlicensed Licensed Download PDF	433
38. The Epidemiology of Kuru Michael P. Alpers and Beat Hörnlimann Requires Authentication Unlicensed Licensed Download PDF	440
39. The Course of the BSE Epidemic - Retrospective Epidemiological Considerations Beat Hörnlimann, Judith B. Ryan and Stuart C. MacDiarmid Requires Authentication Unlicensed Licensed Download PDF	449
40. The Causes of the BSE Epidemic Susanne Dahms and Beat Hörnlimann Requires Authentication Unlicensed Licensed Download PDF	464
Topic VII: Transmissibility
41. The Experimental Transmissibility of Prions and Infectivity Distribution in the Body Martin H. Groschup, Markus Geissen and Anne Buschmann Requires Authentication Unlicensed Licensed Download PDF	473
42. Iatrogenic and "Natural" Transmissibility of Prion Diseases Martin H. Groschup, Beat Hörnlimann and Anne Buschmann Requires Authentication Unlicensed Licensed Download PDF	483
Topic VIII: Agent Inactivation
43. Inactivation in Practice - Risk Assessment and Validation for Food Gelatin Stuart C. MacDiarmid Requires Authentication Unlicensed Licensed Download PDF	499
44. Chemical Disinfection and Inactivation of Prions Beat Hörnlimann, Walter J. Schulz-Schaeffer, Klaus Roth, Zheng-Xin Yan, Henrik Müller, Radulf C. Oberthür and Detlev Riesner Requires Authentication Unlicensed Licensed Download PDF	504
45. Thermal Inactivation of Prions Radulf C. Oberthür, Henrik Müller and Detlev Riesner Requires Authentication Unlicensed Licensed Download PDF	515
Topic IX: Prevention
46. Prevention of Prion Diseases in the Production of Medicinal Products, Medical Devices, and Cosmetics Michael Ruffing, Helena Windemann and Jan Schaefer Requires Authentication Unlicensed Licensed Download PDF	529
47. Prevention of the Transmission of Prion Diseases in Healthcare Settings Beat Hörnlimann, Georg Pauli, Karin Lemmer, Michael Beekes and Martin Mielke Requires Authentication Unlicensed Licensed Download PDF	546
48. Precautionary Measures for Autopsies Performed in Cases of Suspected Prion Disease Walter J. Schulz-Schaeffer, Armin Giese and Hans Kretzschmar Requires Authentication Unlicensed Licensed Download PDF	561
49. Prevention of Prion Diseases in Research Laboratories Alex J. Raeber and Adriano Aguzzi Requires Authentication Unlicensed Licensed Download PDF	565
Topic X: Risk Assessment
50. Evidence for a Link between Variant Creutzfeldt-Jakob Disease and Bovine Spongiform Encephalopathy Moira E. Bruce, Robert G. Will, James W. Ironside and Hugh Fraser Requires Authentication Unlicensed Licensed Download PDF	573
51. Risk Assessment of Transmitting Prion Diseases through Blood, Cornea, and Dura Mater Johannes Löwer and Thomas R. Kreil Requires Authentication Unlicensed Licensed Download PDF	579
52. BSE Risk Assessment and Minimization Radulf C. Oberthür, Aline A. Koeijer, Bram E. C. Schreuder and Stuart C. MacDiarmid Requires Authentication Unlicensed Licensed Download PDF	601
53. BSE Control - Internationally Recommended Approaches Stuart C. MacDiarmid, Paul Infanger and Beat Hörnlimann Requires Authentication Unlicensed Licensed Download PDF	620
54. Atypical Scrapie-Nor98 Sylvie L. Benestad and Bjørn Bratberg Requires Authentication Unlicensed Licensed Download PDF	630
55. Scrapie Control - Internationally Recommended Approaches Marcus G. Doherr and Nora Hunter Requires Authentication Unlicensed Licensed Download PDF	635
56. The PrP Genotype as a Marker for Scrapie Susceptibility in Sheep Nora Hunter and Alex Bossers Requires Authentication Unlicensed Licensed Download PDF	640
57. Scrapie control at the National level: The Norwegian Example Kristin Ruud Alvseike, Ingrid Melkild and Kristin Thorud Requires Authentication Unlicensed Licensed Download PDF	648
Backmatter Requires Authentication Unlicensed Licensed Download PDF	655

(Deutsch)

Language: English
Publisher: De Gruyter
Audience: Libraries of university and academic departmants, research centers and ministries
Pages
- Front matter: 27
- Main content: 714
Illustrations
- Illustrations: 162
- Tables: 88
Keywords: BSE; Creutzfeld-Jakob-Disease; Pathology; Prion; Scrapie

eBook

Published: May 8, 2009
ISBN: 9783110200171

Hardcover

Published: December 21, 2006
ISBN: 9783110182750