Prions and Diseases

Volume 2, Animals, Humans and the Environment

  • Wen-Quan Zou
  • Pierluigi Gambetti

Table of contents

  1. Front Matter
    Pages i-ix
  2. Gianluigi Zanusso, Salvatore Monaco
    Pages 1-13
  3. Christine Fast, Martin H. Groschup
    Pages 15-44
  4. Pierluigi Gambetti, Silvio Notari
    Pages 59-72
  5. Shannon L. Bartelt-Hunt, Jason C. Bartz, Samuel E. Saunders
    Pages 89-101
  6. Gabor G. Kovacs, Herbert Budka
    Pages 103-119
  7. Alexander H. Peden, Mark W. Head, James W. Ironside
    Pages 121-138
  8. Suzette A. Priola
    Pages 139-154
  9. Abigail B. Diack, Rona Wilson, Enrico Cancellotti, Barry Bradford, Matthew Bishop, Jean C. Manson
    Pages 155-169
  10. Pedro Fernandez-Funez, Edward Málaga-Trillo, Diego E. Rincon-Limas
    Pages 183-199
  11. Inga Zerr, Joanna Gawinecka, Katharina Stoeck, Maren Breithaupt
    Pages 201-222
  12. Jason M. Wilham, Christina D. Orrù, Sarah Vascellari, Andrew G. Hughson, Byron Caughey
    Pages 223-232
  13. T. Dean Airey, Neil R. Cashman
    Pages 233-255
  14. Thomas Wisniewski, Fernando Goñi
    Pages 269-287
  15. Back Matter
    Pages 289-295

About this book


Transmissible spongiform encephalopathies (TSE), now broadly known as prion diseases, have been recognized for nearly 300 years in animals and almost 100 years in humans. However, the nature of the transmissible agent had largely remained a mystery until Stanley Prusiner discovered the infectious isoform of the prion protein (PrP), named prion or scrapie PrP (PrPSc), in 1982. The subsequent modern studies with protein chemistry and molecular biology in cell culture, transgenic animals, and cell-free systems, including the revolutionary protein-misfolding cyclic amplification (PMCA), have greatly advanced our understanding of the pathogenesis of prion diseases and facilitated the identification of new prion diseases in animals and humans. In Prions and Prion Diseases, more than 60 leading researchers and clinicians worldwide provide an up-to-date development in many aspects of these unique infectious pathogens and their associated diseases.

Volume II features a variety of animal and human prion diseases, including the newly-identified atypical forms of bovine spongiform encephalopathy and scrapie in animals, and variably protease-sensitive prionopathy in humans, prions in the environment, Tau pathology in human prion disease, transmission of the disease by blood transfusion, mammalian and non-mammalian models, conventional and advanced diagnoses, prion-specific antibodies, as well as decontamination of prions and development of therapeutics of prion diseases, such as the application of immunomodulation. This volume provides up-to-date knowledge about the etiology, pathogenesis, classification, histopathological, and clinical aspects of the highly publicized animal and human prion diseases.


Creutzfeldt-Jakob disease Prion protein bovine spongiform encephalopathy chronic wasting disease prion diseases prions scrapie

Editors and affiliations

  • Wen-Quan Zou
    • 1
  • Pierluigi Gambetti
    • 2
  1. 1., Department of PathologyCase Western Reserve University School oClevelandUSA
  2. 2., Department of NeuropathologyCase Western Reserve University School oClevelandUSA

Bibliographic information