Prions and Diseases

Transmissible spongiform encephalopathies (TSE), known as prion diseases, have been recognized for nearly 300 years in animals and almost 100 years in humans. Modern studies, including the 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 the second edition of Prions and Diseases, more than 60 leading researchers and clinicians worldwide provide an up-to-date discussion of these unique infectious pathogens and their associated diseases. The book provides up-to-date knowledge about the etiology, pathogenesis, classification, histopathological, and clinical aspects of the full range of animal and human prion diseases. As a result, the book contains by far the most authoritative views about the past, present, and future of prions and prion diseases. The new second edition covers such important emerging topics such as inherited human prion disease, stem-cell models in prion research, human prion disease surveillance, and gene therapy strategies.

Part I: HISTORY

Chapter 1   Transmissible Spongiform Encephalopathy: from its beginnings to Daniel Carleton Gajdusek

Part II: GENERAL ASPECTS OF PRIONS

Chapter 2  The Rich Chemistry of the Copper and Zinc Sites in Cellular Prion Protein

                   Glenn L. Millhauser

Chapter 3  Mammalian Prion Structures

                   Byron Caughey, Efrosini Artikis, and Allison Kraus

Chapter 4  Insoluble Cellular Prion Protein and Other Neurodegeneration-related Misfolded Protein

                   Aggregates in the Brain of Asymptomatic Individuals

                   Wen-Quan Zou

Part III: CONVERSION AND STRAIN OF PRIONS

Chapter 5   Prion Conversion and Deformed Templating

                    Ilia V. Baskakov

Chapter 6   Prion Strain Interference    

                    Ronald A. Shikiya and Jason C. Bartz

Chapter 7   Molecular Mechanisms Encoding Strains of Prions and Prion-Like Misfolded Proteins

             Lenka Hromadkova, M. Khursheed Siddiqi, He Liu, and Jiri G. Safar

Chapter 8   Cofactor Involvement in Prion Propagation

                    Surachai Supattapone and Michael B. Miller

Chapter 9    Prion Protein Conversion and Lipids

                     Jiyan Ma and Xiangyi Zhang

Chapter 10  Prions in the Environment

                     Shannon L. Bartelt-Hunt, Jason C. Bartz, and Qi Yuan

Chapter 11 Environmentally Acquired Transmissible Spongiform Encephalopathy

                    Paul Brown

Chapter 12 Risk of Transmission of Creutzfeldt-Jakob Disease by Blood Transfusion

             Alexander H. Peden, Marcelo A. Barria

Chapter 13 Species Barriers in Prion Disease

                    Suzette A. Priola

Part V:  MODELLING OF PRIONS

Chapter 14 Modeling the Cell Biology of Prions

                    Richard Rubenstein, David Doyle and Robert B. Petersen

Chapter 15 Transgenic Mice Modelling in Prion Diseases

                    Barry Bradford, Neil A. Mabbott and Abigail B. Diack

Chapter 16 Stem Cell Models in Prion Research

                    Cathryn Haigh

Chapter 17 Drosophila Models of Prion Diseases

                    Ryan R. Myers and Pedro Fernandez-Funez

 Part VI:    HUMAN PRION DISEASES AND OTHER PATHOLOGIES

Chapter 18 Human Sporadic Prion Diseases

                    Pierluigi Gambetti and Ignazio Cali

                   James A. Mastrianni

Chapter 20 Glycoform-selective Prions in Sporadic and Inherited Variably Protease-sensitive Prionopathies

Chapter 21 The Spectrum of Tau Pathology in Human Prion Disease

                    Gabor G. Kovacs and Herbert Budka

Chapter 22  Prion Protein Complex with mGluR5 Mediates Amyloid-ß Synaptic Loss in Alzheimer’s Disease

                    Graham P. Roseman, Li Fu and Stephen M. Strittmatter

Chapter 23 Prion and Cancers

             Wei Xin

Chapter 24 Protective Role of Cellular Prion Protein in Tissue Ischemic/Reperfusion Injury

                    Zerui Wang and Wen-Quan Zou

Chapter 25  Bovine Spongiform Encephalopathy

                      Gianluigi Zanusso and Salvatore Monaco

Chapter 26  Classical and Atypical Scrapie in Sheep and Goats

                     Christine Fast and Martin H. Groschup

                     Julianna Sun and Glenn Telling

Part VIII: YEAST PRIONS

Chapter 28  Introduction to Yeast and Fungal Prions

                     Reed B. Wickner and Herman K. Edskes

Chapter 29  Yeast Prions Are Folded, In-Register Parallel Am