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.- Chapter 3: Mammalian Prion Structures.- Chapter 4:
Insoluble Cellular Prion Protein and Other Neurodegeneration-related
Misfolded Protein Aggregates in the Brain of Asymptomatic Individuals.- Part
III: CONVERSION AND STRAIN OF PRIONS.- Chapter 5: Prion Conversion and
Deformed Templating.- Chapter 6: Prion Strain Interference.- Chapter 7:
Molecular Mechanisms Encoding Strains of Prions and Prion-Like Misfolded
Proteins.- Chapter 8: Cofactor Involvement in Prion Propagation.- Chapter 9:
Prion Protein Conversion and Lipids.- Part IV: ENVIRONMENT AND TRANSMISSION
OF PRIONS.- Chapter 10: Prions in the Environment.- Chapter 11:
Environmentally Acquired Transmissible Spongiform Encephalopathy.- Chapter
12: Risk of Transmission of Creutzfeldt-Jakob Disease by Blood
Transfusion.- Chapter 13: Species Barriers in Prion Disease.- Part V:
MODELLING OF PRIONS.- Chapter 14: Modeling the Cell Biology of
Prions.- Chapter 15: Transgenic Mice Modelling in Prion Diseases.- Chapter
16: Stem Cell Models in Prion Research.- Chapter 17: Drosophila Models of
Prion Diseases.- Part VI: HUMAN PRION DISEASES AND OTHER
PATHOLOGIES.- Chapter 18: Human Sporadic Prion Diseases.- Chapter 19 Genetic
Creutzfeldt-Jakob and Gerstmann-Sträussler-Scheinker Diseases.- Chapter 20:
Glycoform-selective Prions in Sporadic and Inherited Variably
Protease-sensitive Prionopathies.- Chapter 21: The Spectrum of Tau Pathology
in Human Prion Disease.- Chapter 22: Prion Protein Complex with mGluR5
Mediates Amyloid-ß Synaptic Loss in Alzheimers Disease.- Chapter 23: Prion
and Cancers.- Chapter 24: Protective Role of Cellular Prion Protein in Tissue
Ischemic/Reperfusion Injury.- Part VII: ANIMAL PRION DISEASES.- Chapter 25:
Bovine Spongiform Encephalopathy.- Chapter 26: Classical and Atypical Scrapie
in Sheep and Goats.- Chapter 27: Research models for studying chronic wasting
disease.- Part VIII: YEAST PRIONS.- Chapter 28: Introduction to Yeast and
Fungal Prions.- Chapter 29: Yeast Prions Are Folded, In-Register Parallel
Amyloids Subject to Multiple Anti-prion Systems.- 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.- Chapter 31: Protein Misfolding Cyclic Amplification.- Chapter 32:
Skin Misfolded Proteins as Biomarkers for Diagnosis of Prion and Prion-like
Diseases.- Chapter 33: Diagnosis of Prion Disease: Conventional
Approaches.- Chapter 34: Human Prion Disease Surveillance.- Part X:
TREATMENT.- Chapter 35: Overview on Treatment of Prion Diseases and
Decontamination of Prions.- Chapter 36: Gene Therapy Strategies for
Prophylactic and Therapeutic Treatments of Human Prion Diseases.- Chapter 37:
Immunomodulation.
Wen-Quan Zou, MD, PhD is Professor of Pathology at Case Western University School of Medicine, and he is Associate Director of the National Prion Disease Pathology Surveillance Center.
Pierluigi Gambetti, MD, is Professor of Pathology and Neurology at Case Western University School of Medicine.
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