Transmissible spongiform encephalopathies (TSE), known as prion diseases, have been recognized for nearly 300 years in animals and almost 100 years in humans. Modern studies, including the protein-misfolding cyclic amplification (PMCA), have greatly advanced our understanding of the pathogenesis of prion diseases and facilitated the identification of new prion diseases in animals and humans. In the second edition of Prions and Diseases, more than 60 leading researchers and clinicians worldwide provide an up-to-date discussion of these unique infectious pathogens and their associated diseases. The book provides up-to-date knowledge about the etiology, pathogenesis, classification, histopathological, and clinical aspects of the full range of animal and human prion diseases. As a result, the book contains by far the most authoritative views about the past, present, and future of prions and prion diseases. The new second edition covers such important emerging topics such as inherited human prion disease, stem-cell models in prion research, human prion disease surveillance, and gene therapy strategies.

Most of the world's experts on prions met for a workshop in Erice in August 1996. The aim of the workshop was to discuss the fundamentals of the science ofprions. It was for tunate that so many could be present given the pressure that they were under because of the data presented in March 1996, indicating that Bovine Spongiform Encephalopathy, BSE or Mad Cow Disease, had penetrated the species barrier and was beginning to cause a new dis ease in humans--the new variant ofCreutzfeldt-Jakob Disease, nvCJD. This important and urgent subject became an additional major topic at the workshop. This is a book containing most of the talks plus the abstracts of those unable to find time to write up their talks. Almost all papers were written in the spring and summer of 1997 and contain material added after the workshop; thus Bob Will's paper on the new variant of cm contains data up to July 1997 and four contributions arrived in October 1997. In addition to the talks given at our workshop, there was a special joint session with the Planetary Emergencies Workshop where many distinguished scientists, including three Nobel laureates, discussed major issues affecting our planet. Six talks were given by us to this other workshop, five about prions, BSE, and nvCJD, and one on the broader issue of new epi demics by Luc Montagnier. Although most of the talks concerned research issues, there were a few special talks.

Prions and Neurodegenerative Diseases, Volume 172, in the Progress in Molecular Biology and Translational Science series, provides the most topical, informative and exciting monographs available on a wide variety of research topics. The series includes in-depth knowledge on the molecular biological aspects of organismal physiology, with this release including chapters on Cell-free amplification of prions: where do we stand? Transgenic mouse models for the study of prion diseases and much more. - Includes comprehensive coverage of molecular biology - Presents ample use of tables, diagrams, schemata and color figures to enhance the reader's ability to rapidly grasp the information provided - Contains contributions from renowned experts in the field - Includes comprehensive coverage of molecular biology - Presents ample use of tables, diagrams, schemata and color figures to enhance the reader's ability to rapidly grasp the information provided - Contains contributions from renowned experts in the field

This is the first text to examine the connection between virology and human disease. It is also the first book to integrate basic virology with pathophysiological conditions. By contrast, most virology textbooks focus on the molecular biology involved without adequate reference to physiology. Viruses and Human Disease is four-color throughout and contains clearly labeled figures and tables.Key Features*Provides a concise overview of animal viruses, emphasizing those causing diseases in humans;* Integrates discussion of molecular biology, epidemiology, and the history of human viruses;* Presents treatment of prions, gene therapy, and vaccine development;* Illustrated in color by an expert virologist;* Includes world maps depicting the current distribution of existing and newly emerging viruses

The alarm sounded by Canada's confirmed case of bovine spongiform encephalopathy (BSE) has reaffirmed the exigency of establishing improved safeguards and more aggressive surveillance protocols in North America and around the world. Research converging on the probable causative agent--prion proteins--calls for intensive assessment of the headway gained in tracing prions, testing for transmissible neurodegenerative diseases, and developing methods for cornering the epidemic. With an illustrious panel of 36 international contributors, this timely book marshals techniques for prion protein assay and diagnosis of transmissible spongiform encephalopathies (TSEs).

Basic Neurochemistry: Molecular, Cellular and Medical Aspects, a comprehensive text on neurochemistry, is now updated and revised in its Seventh Edition. This well-established text has been recognized worldwide as a resource for postgraduate trainees and teachers in neurology, psychiatry, and basic neuroscience, as well as for graduate and postgraduate students and instructors in the neurosciences. It is an excellent source of information on basic biochemical processes in brain function and disease for qualifying examinations and continuing medical education. - Completely updated with 60% new authors and material, and entirely new chapters - Over 400 fully revised figures in splendid color

Internationally recognized investigators review the latest developments in, and novel approaches to, understanding the prion protein and prion diseases at the molecular level. Utilizing a variety of cutting-edge techniques, these distinguished scientists seek to define the normal function of a prion protein, to detect and measure the early immune response to prion disease, and to discover possible therapeutic targets. They also use transgenic mice and new electrophysiological investigations to elucidate the pathogenetic mechanisms involved in prion diseases. State-of-the-art and richly insightful, Molecular Pathology of the Prions captures for basic and clinical neuropathologists the latest developments and approaches to understanding the pathogenesis of prion diseases, and by analogy suggests possible research techniques for the more common proteinopthies, such as Alzheimer's and Parkinson's diseases.

A historical exploration of scientific disputes on the causation of so-called ‘prion diseases’, this fascinating book covers diseases including Scrapie, Creutzfeldt-Jakob Disease (CJD) and Bovine Spongiform Encephalopathy (BSE). Firstly tracing the twentieth-century history of disease research and biomedicine, the text then focuses on the relations between scientific practice and wider social transformations, before finally building upon the sociologically informed methodological framework. Incisive and thought-provoking, The Social Construction of Disease provides a valuable contribution to that well-established tradition of social history of science, which refers primarily to the theoretical works of the sociology of scientific knowledge.

Research indicates that most neurodegenerative diseases, systemic amyloidoses and many others, arise from the misfolding and aggregation of an underlying protein. This is the first book to discuss significant achievements in protein structure-function relationships in biochemistry, molecular biology and molecular medicine. The authors summarize recent progress in the understanding of the relationships between protein misfolding, aggregation and development of protein deposition disorders.