Description
Book SynopsisTransmissible 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.
Table of ContentsPart I: HISTORY
Chapter 1 Transmissible Spongiform Encephalopathy: from its beginnings to Daniel Carleton Gajdusek
Paul Brown
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
Part IV: ENVIRONMENT AND TRANSMISSION OF PRIONS
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
Chapter 19 Genetic Creutzfeldt-Jakob and Gerstmann-Sträussler-Scheinker Diseases James A. Mastrianni
Chapter 20 Glycoform-selective Prions in Sporadic and Inherited Variably Protease-sensitive Prionopathies
Zerui Wang, Jue Yuan, Tricia Gilliland, Maria Gerasimenko, Syed Zahid Ali Shah, Wen-Quan Zou
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
Part VII: ANIMAL PRION DISEASES
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
Chapter 27 Research models for studying chronic wasting disease
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 Amyloids Subject to Multiple Anti-prion Systems
Reed B. Wickner, Herman K. Edskes, Moonil Son, Songsong Wu and Madaleine Niznikiewicz
Part IX: DIAGNOSIS AND HUMAN PRION SURVEILLANCE
Chapter 30 Real-time quaking-induced conversion (QuIC) assays for the detection and diagnosis of
human prion diseases
Christina D. Orrù, Onyekachi Isiofia, Andrew G. Hughson and Byron Caughey
Chapter 31 Protein Misfolding Cyclic Amplification
Fabio Moda, Sandra Pritzkow, and Claudio Soto
Chapter 32 Skin Misfolded Proteins as Biomarkers for Diagnosis of Prion and Prion-like Diseases
Zerui Wang, Johnny Dang, and Wen-Quan Zou
Chapter 33 Diagnosis of Prion Disease: Conventional Approaches
Inga Zerr and Peter Hermann
Chapter 34 Human Prion Disease Surveillance
Brian S. Appleby, Lawrence B. Schonberger, Ermias D. Belay
Part X: TREATMENT
Chapter 35 Overview on Treatment of Prion Diseases and Decontamination of Prions
Richard Knight
Chapter 36 Gene Therapy Strategies for Prophylactic and Therapeutic Treatments of Human Prion Diseases
Manuel Camacho and Qingzhong Kong
Chapter 37 Immunomodulation
Thomas Wisniewski and Fernando Goñi
