Prions and Diseases : Volume 2, Animals, Humans and the Environment

Authored by a team of leading researchers and clinicians
Updated knowledge and concepts on pathogenic prion proteins and prion diseases
Present state-of the art findings and hypotheses beyond prions and prion diseases

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.


Chapter 1 Bovine Spongiform Encephalopathy
Gianluigi Zanusso, Salvatore Monaco

Chapter 2 Classical and Atypical Scrapie in Sheep and Goats
Christine Fast, Martin H. Groschup

Chapter 3 Chronic wasting disease and the development of research models
Glenn Telling

Chapter 4 Sporadic Human Prion Disease
Pierluigi Gambetti

Chapter 5 Environmentally-acquired Transmissible Spongiform Encephalopathy
Paul Brown

Chapter 6 Prions in the Environment
Shannon L. Bartelt-Hunt, Jason C. Bartz, Samuel E. Saunders

Chapter 7 The Spectrum of Tau Pathology in Human Prion Disease
Gabor G. Kovacs, Herbert Budka

Chapter 8 Risk of Transmission of Creutzfeldt-Jakob Disease by Blood Transfusion
Alexander H. Peden, Mark W. Head, James W. Ironside

Chapter 9 Species Barriers in Prion Disease
Suzette A. Priola

Chapter 10 Transgenic Mice Modelling
Abigail B. Diack, Rona Wilson, Enrico Cancellotti, Barry Bradford, Matthew Bishop, Jean C. Manson

Chapter 11 Prion Transmission Studies in Transgenic Mice
Qingzhong Kong

Chapter 12 Alternative Models of Prion Diseases
Pedro Fernandez-Funez, Edward Málaga-Trillo, Diego E. Rincon-Limas

Chapter 13 Diagnosis of Prion Disease: Conventional Approaches
Inga Zerr, Joanna Gawinecka, Katharina Stoeck, Maren Breithaupt

Chapter 14 Quaking-induced Conversion (QuIC) Assays for the Detection and Diagnosis of Prion Diseases
Jason M. Wilham, Christina D. Orrù, Sarah Vascellari, Andrew G. Hughson, Byron Caughey

Chapter 15 The Tyr-Tyr-Arg Prion-Specific Epitope: Update and Context
T. Dean Airey, and Neil R. Cashman

Chapter 16 Overview on Treatment of Prion Diseases and Decontamination of Prions
Richard Knight

Chapter 17 Immunomodulation
Thomas Wisniewski, Fernando Goñi

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