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Prions and Diseases

  • Book
  • © 2013

Overview

  • 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

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Keywords

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. As a result, the book contains by far the most authoritative views about the past, present, and future of prions and prion diseases.

Editors and Affiliations

  • Department of Pathology Room 403, Case Western Reserve University School o, Cleveland, USA

    Wen-Quan Zou

  • Department of Neuropathology Room 419, Case Western Reserve University School o, Cleveland, USA

    Pierluigi Gambetti

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