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.