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The author, a 1997 recipient of the Noble Prize in medicine, describes the years he spent researching and demonstrating how the infectious proteins known as prions were responsible for brain diseases and how his theory has now become widely accepted in the science establishment.
Prions are infectious proteins responsible for diseases such as Creutzfeldt-Jakob disease and "mad cow" disease. They are misfolded versions of normal proteins that replicate by converting their normal cellular counterparts into abnormal prion proteins that disrupt cell function and can be transmitted to other cells and individuals. This book examines our under-standing of their structure, biochemistry, and pathophysiology.
This volume is a new edition of the most authoritative book on Prion Biology, first published in 1999 and edited by the Nobel Prize-winning founder of the field. This expanded edition has been completely updated, and includes chapters on therapeutics, and diagnostic methods and approaches.
A series of remarkable discoveries in the past three decades have led to the molecular and genetic characterization of the transmissible pathogen causing scrapie in animals and a quartet of human illnesses: kuru, Jakob-Creutzfeld disease, Gerstmann-Sträussler-Scheinker disease, and fatal familial insomnia. To distinguish this pathogen from viruses and viroids, the term "prion" was introduced to emphasize its proteinaceous and infectious nature. Stanley B. Prusiner, editor of this volume, was awarded the 1997 Nobel Prize in Physiology and Medicine for his pioneering discovery of prions. The book reviews advances in studies of prions, which - as considereable evidence indicates - are novel pathogens composed only of protein.
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In this brilliant and gripping medical detective story. Richard Rhodes follows virus hunters on three continents as they track the emergence of a deadly new brain disease that first kills cannibals in New Guinea, then cattle and young people in Britain and France -- and that has already been traced to food animals in the United States. In a new Afterword for the paperback, Rhodes reports the latest U.S. and worldwide developments of a burgeoning global threat.
A conformational transition of the cellular prion protein (PrPC) into an aberrantly folded isoform designated scrapie prion protein (PrPSc) is the hallmark of a variety of neurodegenerative disorders collectively called prion diseases. They include Creutzfeldt-Jakob disease and Gerstmann-Stäussler-Scheinker syndrome in humans, scrapie in sheep, bovine spongiform encephalopathy (BSE) in cattle and chronic wasting disease (CWD) in free-ranging deer. In contrast to the deadly properties of misfolded PrP, PrPC seems to possess a neuroprotective activity. More-over, animal models indicated that the stress-protective activity of PrPC and the neurotoxic effects of PrPSc are somehow interconnected....
Neurology – as only Harrison’s can cover it Featuring a superb compilation of chapters related to neurology that appear in Harrison’s Principles of Internal Medicine, Eighteenth Edition, this concise, full-color clinical companion delivers the latest knowledge in the field backed by the scientific rigor and authority that have defined Harrison’s. You will find content from renowned editors and contributors in a carry-anywhere presentation that is ideal for the classroom, clinic, ward, or exam/certification preparation. Features Current, complete coverage of clinically important topics in neurology, including Clinical Manifestations of Neurologic Diseases, Diseases of the Nervous Syst...
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.
The Nobel Prizes m natural sciences have achieved the reputation of being the ultimate accolade for scientific achievements. This honk gives a unique insight into the selection of Nobel Prize recipients, in particular the life sciences. The evolving mechanisms of selection of prize recipients are illustrated by reference to archives, which have remained secret for 1) years. Many of the prizes subjected to particular evaluation concern awards given for discoveries in the field of infectious diseases and the interconnected field of genetics. The book illustrates the individuals and environments that are conducive to scientific creativity. Nowhere is this enigmatic activity'-- the mime mover in advancing the human condition highlighted as lucidly as by identification individuals worthy of Nobel Prizes. --Book Jacket.