Neuropathology of Transmissible Spongiform Encephalopathies (Prion Diseases) -- Central Pathogenesis of Prion Diseases -- Hereditary Prion Protein Amyloidoses -- Mouse Behavioural Studies and What They Can Teach Us about Prion Diseases -- Electrophysiological Approaches to the Study of Prion Diseases -- Prion Protein, Prion Protein-Like Protein, and Neurodegeneration -- Oxidative Stress and Mitochondrial Dysfunction in Neurodegeneration of Transmissible Spongiform Encephalopathies (TSEs) -- Mechanisms of Prion Toxicity and Their Relationship to Prion Infectivity -- A Stone Guest on the Brain: Death as a Prion -- Molecular Mechanisms Mediating Neuronal Cell Death in Experimental Models of Prion Diseases, in vitro -- Processing and Mis-Processing of the Prion Protein: Insights into the Pathogenesis of Familial Prion Disorders -- Signaling Pathways Controling Prion Neurotoxicity: Role of Endoplasmic Reticulum Stress-Mediated Apoptosis -- Cell Culture Models to Unravel Prion Protein Function and Aberrancies in TSE -- Insights into the Cellular Trafficking of Prion Proteins -- The Molecular Basis of Prion Protein-Mediated Neuronal Damage -- Conclusion: Intervention, the Final Frontier. This volume provides an in-depth overview from world experts on prion disease. These fatal diseases include Creutzfeldt-Jakob disease, chronic wasting disease in deer, scrapie of sheep, bovine spongiform encephalopathy (mad cow's disease) of cattle and related diseases. Understanding what causes neuronal death in these diseases is essential to both preventing and curing them. The most recent advances in understanding neuronal death in prion diseases are presented. Prion diseases serve as an experimental model for all neurodegenerative conditions. This book will provide understanding of neurodegeneration and provide an up-to-date record of the state of the art for other specialists and non-specialists in related fields.