Prion Diseases

1. Front Matter

   Pages i-xv

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2. The Paradox of Prion Disease

   • Rosalind M. Ridley, Harry F. Baker

   Pages 1-13

3. Human Spongiform Encephalopathy

   • Rajith de Silva

   Pages 15-33

4. Neuropathological Diagnosis of Human Prion Disease

   • James W. Ironside

   Pages 35-57

5. Neuropathological Diagnosis of Human Prion Disease

   • Jeanne E. Bell

   Pages 59-83

6. The Diagnosis of Bovine Spongiform Encephalopathy and Scrapie by the Detection of Fibrils and the Abnormal Protein lsoform

   • Michael J. Stack, Paula Keyes, Anthony C. Scott

   Pages 85-103

7. Exposure to, and Inactivation of, the Unconventional Agents that Cause Transmissible Degenerative Encephalopathies

   • David M. Taylor

   Pages 105-118

8. Surveillance of Prion Diseases in Humans

   • Robert G. Will

   Pages 119-137

9. Environmental Causes of Human Spongiform Encephalopathy

   • Paul Brown

   Pages 139-154

10. Bovine Spongiform Encephalopathy

    • John W. Wilesmith

    Pages 155-173

11. Handling the BSE Epidemic in Great Britain

    • David A. J. Tyrrell, Kevin C. Taylor

    Pages 175-198

12. Special Problems of Genetic Counseling in Adult-Onset Diseases

    • Jonathon R. Gray, Jo R. Soldan, Peter S. Harper

    Pages 199-210

13. Genotyping and Susceptibility of Sheep to Scrapie

    • Nora Hunter

    Pages 211-221

14. Strain Typing Studies of Scrapie and BSE

    • Moira E. Bruce

    Pages 223-236

15. PrP-Deficient Mice in the Study of Transmissible Spongiform Encephalopathies

    • Jean C. Manson

    Pages 237-249

16. Transgenic Approaches to Prion “Species-Barrier” Effects

    • David Westaway

    Pages 251-263

17. Methods for Studying Prion Protein Amyloid

    • Fabrizio Tagliavini, Frances Prelli, Giorgio Giaccone, Gianluigi Forloni, Mario Salmona, Pedro Piccardo et al.

    Pages 265-283

18. Methods for Studying Prion Protein (PrP) Metabolism and the Formation of Protease-Resistant PrP in Cell Culture and Cell-Free Systems

    • Byron Caughey, David A. Kocisko, Suzette A. Priola, Gregory J. Raymond, Richard E. Race, Richard A. Bessen et al.

    Pages 285-299

19. Immunohistochemistry of Resinated Tissues for Light and Electron Microscopy

    • Martin Jeffrey, Caroline M. Goodsir

    Pages 301-312

20. Back Matter

    Pages 313-317

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Harry Baker and Rosalind Ridley have done an admirable job in assem­ bling this collection of articles that describe the methodology frequently used to study a group of CNS illnesses often referred to as the "prion diseases." Research on prions and the disorders that they cause has progressed relatively rapidly over the last decade since the discovery of the prion protein (PrP) that allowed the application of modem molecular biological and genetic tools. The power of these techniques is awesome and their use in deciphering the once mysterious prion diseases has brought a wealth of new information. Although prions are unprecedented pathogens, appearing to consist only of PrPSc molecules, the diseases that they cause are no less remarkable. The prion diseases in animals include scrapie of sheep and goats as well as "mad cow" disease or bovine spongiform encephalopathy (BSE). In the United King­ dom, the epidemic of BSE has heightened public awareness of this previously obscure group of diseases such that any work in the field is likely to stir up interest in the media and become a subject of public debate. It has been diffi­ cult for British investigators to work on prion diseases without being involved in these controversies. As such, several chapters have been included that deal with political and social issues surrounding prion diseases. The human prion diseases present an equally fascinating saga in which these CNS degenerations present as genetic, sporadic, and infectious illnesses.

Prion Diseases is a timely publication...Scientists already established in the subject as well as those embarking on such research for the first time will find the book useful. I also recommend it as a well structured text..-British Medical Journal

• Cambridge University, Cambridge, UK

  Harry F. Baker, Rosalind M. Ridley

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