Prions in humans and animals / edited by Beat Hornlimann ; in collaboration with Detlev Riesner and Hans Kretzschmar.

Format:

Book

Contributor:

Hörnlimann, Beat, 1958-

Riesner, Detlev, 1941-

Kretzschmar, H. A. (Hans A.)

Language:

English

Subjects (All):

Prion diseases.

Physical Description:

1 online resource (714 p.)

Edition:

1st ed.

Place of Publication:

Berlin ; New York : Walter de Gruyter, c2007.

Language Note:

English

Summary:

Dieses umfassende Werk in englischer Sprache richtet sich gleichermaßen an Studenten und Forscher. Es behandelt systematisch alle Aspekte der Prionenkrankheiten, von ihrer Geschichte, Mikrobiologie und Pathologie bis hin zu Übertragungswegen und möglicher Prävention. Das Buch beschreibt Krankheiten wie Creutzfeld-Jakob-Krankheit, Kuru, Rinderwahn (BSE) und Scrapie, unter besonderer Berücksichtigung der biochemischen, molekularbiologischen, genetischen und klinischen Aspekte. Eine genaue Beschreibung der Wirkung von Prionenkrankheiten im Bereich der Pharmazie, bei Blutprodukten, Desinfektion, chirurgischen Instrumenten und der Epidemiologie rundet das Werk mit einer Diskussion über präventive Maßnahmen ab.

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.

Contents:

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

Notes:

Description based upon print version of record.

Includes bibliographical references and indexes.

ISBN:

9786612188091

9781282188099

1282188097

9783110200171

3110200171

OCLC:

712015585