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.

Author: David R. Brown


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
Mechanisms of prion toxicity and their relationship to prion infectMty
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
Signaling pathways controlling prion neurotoxicity
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