Transmissible Spongiform Encephalopathies:

1. Front Matter

   Pages I-IX

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2. The Human Spongiform Encephalopathies: Kuru, Creutzfeldt-Jakob Disease, and the Gerstmann-Sträussler-Scheinker Syndrome

   • P. Brown, D. C. Gajdusek

   Pages 1-20

3. Bovine Spongiform Encephalopathy

   • J. W. Wilesmith, G. A. H. Wells

   Pages 21-38

4. Purification of Scrapie Agents: How Far Have We Come?

   • D. C. Bolton, P. E. Bendheim

   Pages 39-55

5. The Scrapie Fibril Protein and Its Cellular Isoform

   • J. Hope, J. Manson

   Pages 57-74

6. Ultrastructural Studies of Prions

   • M. P. McKinley, S. B. Prusiner

   Pages 75-91

7. In Vitro Expression and Biosynthesis of Prion Protein

   • B. Caughey

   Pages 93-107

8. Prion Protein Genes: Evolutionary and Functional Aspects

   • B. Oesch, D. Westaway, S. B. Prusiner

   Pages 109-124

9. Scrapie Strain Variation and Its Implications

   • M. E. Bruce, H. Fraser

   Pages 125-138

10. Evidence for Biological and Structural Diversity Among Scrapie Strains

    • R. J. Kascsak, R. Rubenstein, R. I. Carp

    Pages 139-152

11. Genetics of Prion Diversity and Host Susceptibility

    • G. A. Carlson

    Pages 153-164

12. Natural Transmission and Genetic Control of Susceptibility of Sheep to Scrapie

    • N. Hunter

    Pages 165-180

13. The Scrapie Agent In Vitro

    • R. Race

    Pages 181-193

14. The Scrapie Agent: “A Virus by Any Other Name”

    • R. G. Rohwer

    Pages 195-232

15. Novel Properties and Biology of Scrapie Prions

    • S. B. Prusiner

    Pages 233-257

16. Identifying and Mapping Changes in Gene Expression Involved in the Neuropathology of Scrapie and Alzheimer’s Disease

    • J. Diedrich, S. Wietgrefe, A. Haase, J. Duguid, R. I. Carp

    Pages 259-274

17. Search for a Transmissible Agent in Alzheimer’s Disease: Studies of Human Buffy Coat

    • E. E. Manuelidis, L. Manuelidis

    Pages 275-280

18. Back Matter

    Pages 281-290

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In spite of a long history of intense investigation the transmissible spongiform encephalopathies remain a poorly understood family of neurodegenerative diseases. This group of diseases has been described in a wide variety of animal species and includes kuru, Creutzfeldt-Jakob disease, and Gerstmann-Straussler syndrome in humans, and scrapie, bovine spongiform encephalopathy, and related syndromes in ruminants and rodents. In all cases spongiform degeneration and astrocytosis are seen in specimens of brain and a filterable transmissible agent is present in the brain and some other tissues of affected individuals. However, the precise nature of this agent remains unknown. Agent infectivity, which can so far only be assayed by serial transmission to new individuals, be remarkably resistant to inactivation has been shown to by heat, chemicals, and irradiation. These properties create significant biohazard possibilities during exposure to infected tissues. Transmission between humans was originally reco­ gnized in the unique epidemiology of kuru in New Guinea tribesmen, and concern about transmission from animals to humans has re-emerged as a result of the current epidemic of bovine spongiform encephalopathy in dairy cattle in Great Britain. Although interspecies transmission has often been achieved experimentally, its efficiency is highly variable. There­ fore, the possibility of spread of bovine spongiform encephalopathy from cattle to humans or various animal populations cannot be accurately predicted at this time. This volume presents a comprehensive update of know­ ledge concerning the transmissible spongiform encephalo­ pathies.

• Enzephalopathie
• Neurobiologie
• Neurodegenerative Erkrankungen
• Virologie
• diseases
• gene
• neurobiology
• nucleic acid
• prion protein
• protein
• proteins
• tissue
• virology

• Laboratory of Persistent Viral Diseases Rocky Mountain Laboratories National Institute of Allergy and Infectious Diseases, National Institutes of Health, Hamilton, USA

  Bruce W. Chesebro

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