Advancing Prion Science PDF Download

Author: Institute of Medicine
Publisher: National Academies Press
ISBN: 0309087449
Category : Medical
Languages : en
Pages : 125

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Book Description
In Advancing Prion Science, the Institute of Medicine's Committee on Transmissible Spongiform Encephalopathies Assessment of Relevant Science recommends priorities for research and investment to the Department of Defense's National Prion Research Program (NPRP). Transmissible spongiform encephalopathies (TSEs), also called prion diseases, are invariably fatal neurodegenerative infectious diseases that include bovine spongiform encephalopathy (commonly called mad cow disease), chronic wasting disease, scrapie, and Creutzfeldt-Jakob disease. To develop antemortem diagnostics or therapies for TSEs, the committee concludes that NPRP should invest in basic research specifically to elucidate the structural features of prions, the molecular mechanisms of prion replication, the mechanisms of TSE pathogenesis, and the physiological function of prions' normal cellular isoform. Advancing Prion Science provides the first comprehensive reference on present knowledge about all aspects of TSEs' from basic science to the U.S. research infrastructure, from diagnostics to surveillance, and from prevention to treatment. This report summarizes the progress thus far.

Author: Sylvain Lehmann
Publisher: Birkhäuser
ISBN: 3034879490
Category : Medical
Languages : en
Pages : 232

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Book Description
-There is an acceleration in prion disease research because of the spread of mad cow disease. -This book covers in vitro, cellular, and animal models adapted for the study of TSEs. -Includes bio-saftey procedures.

Author:
Publisher: Elsevier
ISBN: 0444639535
Category : Medical
Languages : en
Pages : 520

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Book Description
Human Prion Diseases, Volume 153 is designed to update the reader on the latest advances and clinical aspects of prion diseases. The book is organized into five sections, including the pathophysiology of prions and a description of animal and human diseases. This is followed by detailed reports on recent advances in diagnosis strategies for the development of novel anti-prion molecules and possible designs of clinical trials in such a rare disease. An introductory chapter gives an extensive historical background of prion research, with a final chapter highlighting recent progress, and more importantly, unsolved problems. - Offers an authoritative overview of prion diseases in humans, detailing the pathogenesis of the disease, clinical investigations, and the diagnosis of both the genetic and acquired forms - Provides clarity and context by presenting prion diseases in relation to other neurodegenerative diseases in humans - Emphasizes the unique properties of prion diseases and consequent problems they can cause, both clinically and in public health terms

Author: Stanley B. Prusiner
Publisher: Yale University Press
ISBN: 0300191146
Category : Science
Languages : en
Pages : 344

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Book Description
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.

Author: Jorg Tatzelt
Publisher:
ISBN: 9780954333522
Category : Prions
Languages : en
Pages : 80

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Book Description
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. In this timely book, leading scientists in the field have come together to highlight the apparently incongruous activities of different PrP conformers. The articles outline current research on celluar pathways implicated in the formation and signaling of neurotoxic and physiological PrP isoforms and delineate future research direction. Topics covered include the physiologcial activity of PrPC and its possible role as a neurotrophic factor, the finding that aberrant PrP conformers can cause neurodegeneration in the absence of infectious prion propagation, the requirement of the GPI anchor of PrPC for the neurotoxic effects of scrapie prions, the pathways implicated in the formation and neurotoxic properties of cytosolically localized PrP, the impact of metal ions on the processing of PrP, and the role of autophagy in the propagation and clearance of PrPSc. The book is fully illustrated and chapters include comprehensive reference sections. Essential reading for scientists involved in prion research.

Author: G. Jolles
Publisher: Academic Press
ISBN:
Category : Medical
Languages : en
Pages : 330

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Book Description
Over the last few years, the considerable progress made in biochemistry, virology, molecular biology and genetics has revealed some of the intimate mechanisms of the neurodegenerative diseases. The present volume is an attempt to review the latest data in the field to illuminate new avenues for future research. This volume gathers together chapters and discussions on the etiology and pathogenesis of the neurodegenerative diseases. Apoptosis of programmed cell death as well as other genetic implications are discussed; special attention is given to the coexistence and interconnection of genetic and environmental factors. There is extensive coverage of prions responsible for bovine spongiform encephalopathy, Cruetzfeld-Jacob disease and kuru. The various aspects of non-conventional transmissible agents are thoroughly reviewed. Further contributions deal with the role of growth factors as well as of free radicals. Consideration is given to the molecular mechanisms of Alzheimer's disease, in particular the role of tau protein. Finally, several pharmacological models now available, which throw light upon aspects of Parkinson's disease, Huntington's chorea and multiple sclerosis, are examined and discussed. It is hoped that recent scientific advances will lead to the discovery of new drugs to fill the current therapeutic void. There are hopes of an early indication of this in the case of amyotrophic lateral sclerosis.

Author: Brian K. Nunnally
Publisher: CRC Press
ISBN: 9780203912973
Category : Medical
Languages : en
Pages : 456

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Book Description
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).

Author: Stanley B. Prusiner
Publisher:
ISBN: 9781621820932
Category : Medical
Languages : en
Pages : 0

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Book Description
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.

Author: Gabor G. Kovacs
Publisher: Cambridge University Press
ISBN: 1316337650
Category : Medical
Languages : en
Pages : 320

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Book Description
This practical guide to the diagnosis of neurodegenerative diseases discusses modern molecular techniques, morphological classification, fundamentals of clinical symptomology, diagnostic pitfalls and immunostaining protocols. It is based on the proteinopathy concept of neurodegenerative disease, which has influenced classification and provides new strategies for therapy. Numerous high-quality images, including histopathology photomicrographs and neuroradiology scans, accompany the description of morphologic alterations and interpretation of immunoreactivities. Diagnostic methods and criteria are placed within recent developments in neuropathology, including the now widespread application of immunohistochemistry. To aid daily practice, the guide includes diagnostic algorithms and offers personal insights from experienced experts in the field. Special focus is given to the way brain tissue should be handled during diagnosis. This is a must-have reference for medical specialists and specialist medical trainees in the fields of pathology, neuropathology and neurology working with neuropathologic features of neurodegenerative diseases.

Author: Stanley B. Prusiner
Publisher: CSHL Press
ISBN: 9780879696931
Category : Medical
Languages : en
Pages : 1130

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Book Description
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.