Advancing Prion Science

Advancing Prion Science PDF 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.

Advancing Prion Science

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

Get Book Here

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.

The Prion Protein

The Prion Protein PDF 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.

Neurogenetics, Part II

Neurogenetics, Part II PDF Author:
Publisher: Elsevier
ISBN: 0444640770
Category : Medical
Languages : en
Pages : 480

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Book Description
Neurogenetics, Part II, Volume 148, the latest release in the Handbook of Clinical Neurology, provides the latest information on the genetic methodologies that are having a significant impact on the study of neurological and psychiatric disorders. Using genetic science, researchers have identified over 200 genes that cause or contribute to neurological disorders. Still an evolving field of study, defining the relationship between genes and neurological and psychiatric disorders is expected to dramatically grow in scope. Part II builds on the foundation of Part I, expanding the coverage to dementias, paroxysmal disorders, neuromuscular disorders, white matter and demyelination diseases, cerebrovascular diseases, adult psychiatric disorders and cancer and phacomatoses. - Contains comprehensive coverage of neurogenetics - Details the latest science and its impact on our understanding of neurological, psychiatric disorders - Presents a focused reference for clinical practitioners and the neuroscience/neurogenetics research community

Neuropathology of Neurodegenerative Diseases

Neuropathology of Neurodegenerative Diseases PDF 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.

Prions and Brain Diseases in Animals and Humans

Prions and Brain Diseases in Animals and Humans PDF Author: Douglas R.O. Morrison
Publisher: Springer Science & Business Media
ISBN: 1489918965
Category : Science
Languages : en
Pages : 349

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Book Description
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.

Adams and Victor's Principles of Neurology

Adams and Victor's Principles of Neurology PDF Author: Maurice Victor
Publisher: McGraw-Hill Medical Publishing
ISBN: 9780071163330
Category : Mental Disorders
Languages : en
Pages : 0

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Book Description
A modernizing revision will make it one of the most comprehensive books that incorporate new findings in growing areas of neurology, memory, genetics, imaging and biochemistry - while retaining the book's traditional size, scope, focus, and successful uniform organization. New research findings, combined with several new and updated tables and figures, the book provides reliable guidelines on diagnosis and treatment of all neurological conditions and disorders.

Madness and Memory

Madness and Memory PDF 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.

Human Prion Diseases

Human Prion Diseases PDF 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

Fatal Flaws

Fatal Flaws PDF Author: Jay Ingram
Publisher: Yale University Press
ISBN: 0300189893
Category : Medical
Languages : en
Pages : 294

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Book Description
DIVThe story of the revolutionary science that is unraveling the mysteries of mad cow and other fatal brain diseases/div

Infectious Diseases from Nature: Mechanisms of Viral Emergence and Persistence

Infectious Diseases from Nature: Mechanisms of Viral Emergence and Persistence PDF Author: C.J. Peters
Publisher: Springer Science & Business
ISBN: 9783211243343
Category : Medical
Languages : en
Pages : 234

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Book Description
Significant zoonotic diseases have appeared with increasing frequency in recent years. At a symposium held in Galveston, Texas, in March 2004, many outstanding virologists and others presented papers under the broad theme of "emergence". The intent was to elucidate the diseases themselves, the mechanisms by which they have emerged, the publication perception and response to the diseases, and the possibility of prevention or prediction. The papers in this book summarize the talks of this meeting. Among the many timely papers are those by Nobel Prize winner Peter Doherty, influenza epidemiologists Robert Webster and Jeffery Taubenberger, and important contributions by Neal Nathanson, Esteban Domingo, Barry Beaty, David Walker, James Hughes, and others of world expertise.