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 Alzheimer's 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.