Main description:

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.

Contents:

Part 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-Straussler-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. Orru, 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 Goni