Table of Contents

Neuropathology of transmissible spongiform encephalopathies (prion diseases).- Central pathogenesis of prion diseases.- Hereditory 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 protein 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.

About the Author

David Brown has worked in the field of prion disease or TSEs for over ten years. He was born in Australia but spent part of his early child-hood in the United Kingdom. After returning to Australia he completes his schooling in Sydney and attended Sydney University. There he completed a Bachelor of Science degree in biochemistry, a Master of Science degree in neurobiology and a Doctor of Philosophy degree also in Neuroscience. His initial research interests included neuronal growth factors and topographic innervation of toad muscle. Following three years of postdoctoral research, Dr. Brown left Australia in 1994. Since then he has worked in the Albert Einstein College of Medicine in New York, the Department of Neuropathology in Göttingen and the Department of Biochemistry at the University of Cambridge. His interest in prion began during his four years researching in Germany. After returning to work in the United Kingdom to work at Cambridge, Dr. Brown established his own independent research group that quickly gained international recognition. In parallel with research focusing on the function of the prion protein and mechanisms of cell death in neurodegeneration, Dr. Brown’s research has also investigated basic aspects of cellular neurobiology including the nature of the interactions between neurones and glial cells. David Brown is currently a Reader in Biochemistry at the University of Bath and his research continues to reap international recognition and acclaim. He is also a member of the Spongiform Encephalopathy Advisory Committee that advises the UK government on issues to do with BSE and variant CJD.