Human Prion Diseases

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Human Prion Diseases

Human Prion Diseases
  • Author : Anonim
  • Publisher : Elsevier
  • Release Date : 2018-06-07
  • Total pages : 512
  • ISBN : 9780444639530
  • File Size : 21,9 Mb
  • Total Download : 879
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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

Human Prion Diseases

Human Prion Diseases
  • Author : Jean Manson
  • Publisher : Unknown
  • Release Date : 2018-06-26
  • Total pages : 512
  • ISBN : 0444639454
  • File Size : 55,9 Mb
  • Total Download : 850
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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

Neuropathology of Neurodegenerative Diseases Book and Online

Neuropathology of Neurodegenerative Diseases Book and Online
  • Author : Gabor G. Kovacs
  • Publisher : Cambridge University Press
  • Release Date : 2014-12-04
  • Total pages : 319
  • ISBN : 9781107442429
  • File Size : 42,7 Mb
  • Total Download : 581
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This heavily illustrated, must-have practical guide aids medical specialists and trainees in the diagnosis of neurodegenerative diseases. Includes diagnostic algorithms.

Prions and Diseases

Prions and Diseases
  • Author : Wen-Quan Zou,Pierluigi Gambetti
  • Publisher : Springer Nature
  • Release Date : 2023-01-01
  • Total pages : 773
  • ISBN : 9783031205651
  • File Size : 45,6 Mb
  • Total Download : 667
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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.

Prions in Humans and Animals

Prions in Humans and Animals
  • Author : Beat Hörnlimann,Detlev Riesner,Hans A. Kretzschmar
  • Publisher : Walter de Gruyter
  • Release Date : 2006-01-01
  • Total pages : 741
  • ISBN : 9783110200171
  • File Size : 17,5 Mb
  • Total Download : 534
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This comprehensive work, aimed at both students and researchers alike, systematically covers all aspects of prion diseases (transmissible spongiform encephalopathies), from their history, microbiology and pathology to their transmissibility and prevention. The book describes diseases such as Creutzfeldt-Jakob disease, kuru, mad cow disease (BSE), chronic wasting disease and scrapie, highlighting their biochemical, molecular biological, genetic, and clinical aspects. A renowned editorial team brought together 80 internationally respected authors for this translation and new edition of the successful German publication. The book includes chapters by, among many other notable scientists, William J. Hadlow, who discovered the relationship between the human and animal forms of prion diseases and Michael P. Alpers, with 45 years of experience in Papua New Guinea investigating the first known human epidemic form, kuru, transmitted by endocannibalism. Carefully edited with numerous illustrations, this work offers a systematic approach committed to a clear presentation of the current knowledge of prion diseases. It aims to inspire and stimulate interdisciplinary cooperation, innovative research ideas and effective prevention.

Prions

Prions
  • Author : Claudio Soto
  • Publisher : CRC Press
  • Release Date : 2005-12-20
  • Total pages : 184
  • ISBN : 9781420040128
  • File Size : 22,5 Mb
  • Total Download : 264
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Prion-related diseases, known as transmissible spongiform encephalopathies (TSEs), are infectious, fatal neurodegenerative disorders for which there is no cure, treatment, nor even a means for early diagnosis. The horrific advent of Mad Cow Disease -- transmitted to humans through eating meat from steers sickened by bovine spongiform encephalopathy

The Prion Protein

The Prion Protein
  • Author : Jorg Tatzelt
  • Publisher : Unknown
  • Release Date : 2010
  • Total pages : 80
  • ISBN : 0954333527
  • File Size : 40,6 Mb
  • Total Download : 526
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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.

Prions and Diseases

Prions and Diseases
  • Author : Wen-Quan Zou,Pierluigi Gambetti
  • Publisher : Springer Science & Business Media
  • Release Date : 2012-11-08
  • Total pages : 298
  • ISBN : 9781461453383
  • File Size : 53,9 Mb
  • Total Download : 439
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Volume II features a variety of animal and human prion diseases, including the newly-identified atypical forms of bovine spongiform encephalopathy and scrapie in animals, and variably protease-sensitive prionopathy in humans, prions in the environment, Tau pathology in human prion disease, transmission of the disease by blood transfusion, mammalian and non-mammalian models, conventional and advanced diagnoses, prion-specific antibodies, as well as decontamination of prions and development of therapeutics of prion diseases, such as the application of immunomodulation. This volume provides up-to-date knowledge about the etiology, pathogenesis, classification, histopathological, and clinical aspects of the highly publicized animal and human prion diseases.

Prion Diseases of Humans and Animals

Prion Diseases of Humans and Animals
  • Author : Stanley B. Prusiner
  • Publisher : Ellis Horwood Limited
  • Release Date : 1992
  • Total pages : 583
  • ISBN : UOM:39015009127419
  • File Size : 41,6 Mb
  • Total Download : 183
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PDF book entitled Prion Diseases of Humans and Animals written by Stanley B. Prusiner and published by Ellis Horwood Limited which was released on 1992 with total hardcover pages 583, the book become popular and critical acclaim.

Prion

Prion
  • Author : Yusuf Tutar
  • Publisher : BoD – Books on Demand
  • Release Date : 2017-03-08
  • Total pages : 240
  • ISBN : 9789535130017
  • File Size : 47,8 Mb
  • Total Download : 793
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Protein aggregation causes malfunction in several biochemical processes. Genetic and spontaneous formations of these transmissible spongiform encephalopathies are fatal to humans and animals. Conformational change of normal form of the protein to misfolded form causes its accumulation. The misfolded infectious protein agent forms the pathogenesis of the disease. This book presents pathology of the disease along with current knowledge of the structure-activity mechanism in the first two sections. Dyshomeostasis of metals is implicated in the pathogenesis of prions, and this influence is discussed further to understand the prion mechanism. Genetic resistance and immunobiology of the disease are elaborated in the following section. Finally, a computational study on the dynamics of the prion propagation provides a structural basis of the mechanism.

Prions Prions Prions

Prions Prions Prions
  • Author : Stanley B. Prusiner
  • Publisher : Springer Science & Business Media
  • Release Date : 2012-12-06
  • Total pages : 163
  • ISBN : 9783642609831
  • File Size : 44,9 Mb
  • Total Download : 634
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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.

PrPSc Prions: State of the Art

PrPSc Prions: State of the Art
  • Author : Joaquín Castilla,Jesús R. Requena
  • Publisher : MDPI
  • Release Date : 2018-11-07
  • Total pages : 211
  • ISBN : 9783038973089
  • File Size : 55,5 Mb
  • Total Download : 768
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This book is a printed edition of the Special Issue "PrPSc prions: state of the art" that was published in Pathogens

Prion Diseases

Prion Diseases
  • Author : Martin H. Groschup,Hans Kretzschmar
  • Publisher : Springer Science & Business Media
  • Release Date : 2012-12-06
  • Total pages : 294
  • ISBN : 9783709163085
  • File Size : 46,6 Mb
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The overwhelming interest and the participation of more than 500 scientists from 26 countries made this symposium the largest meeting ever held in the field. In this book twenty-six invited speakers comprehensively present their data on the pathogenesis of prion diseases in humans and animals, on molecular mechanisms involved in the transmissibility across species barriers, on animal and in-vitro models currently available for the detection and quantification of infectivity and on the characterization of prion strains.

Neurodegenerative Diseases

Neurodegenerative Diseases
  • Author : Shamim I. Ahmad
  • Publisher : Springer Science & Business Media
  • Release Date : 2012-03-12
  • Total pages : 390
  • ISBN : 9781461406532
  • File Size : 36,5 Mb
  • Total Download : 858
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The editor of this volume, having research interests in the field of ROS production and the damage to cellular systems, has identified a number of enzymes showing ·OH scavenging activities details of which are anticipated to be published in the near future as confirmatory experiments are awaited. It is hoped that the information presented in this book on NDs will stimulate both expert and novice researchers in the field with excellent overviews of the current status of research and pointers to future research goals. Clinicians, nurses as well as families and caregivers should also benefit from the material presented in handling and treating their specialised cases. Also the insights gained should be valuable for further understanding of the diseases at molecular levels and should lead to development of new biomarkers, novel diagnostic tools and more effective therapeutic drugs to treat the clinical problems raised by these devastating diseases.

Prions

Prions
  • Author : Holger F. Rabenau,Jindrich Cinatl,Hans Wilhelm Doerr
  • Publisher : Karger Medical and Scientific Publishers
  • Release Date : 2004
  • Total pages : 233
  • ISBN : 9783805576567
  • File Size : 38,7 Mb
  • Total Download : 488
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This second, completely revised and extended edition of Prions: A Challenge for Science, Medicine and the Public Health System is a comprehensive, up-to-date review of prions and prion-associated diseases. Leading scientists discuss the structure, molecular biology and origin of prions as well as strain variations and species barriers. Human prion diseases, prion inactivation and risks to public health are considered in detail. The new edition provides an update on basic findings of the last three years since publication of the previous edition and emphasizes practical aspects of fighting human and animal prion diseases. In addition, chapters on regulatory aspects of BSE and CJD as well as on veterinary measures have been included. A valuable single source of information on what is currently known about prions, this book will be of interest to any scientist or clinician who wants to keep up with the latest developments in this fast-moving field.

Prions

Prions
  • Author : Ivo Nikolaev Sirakov
  • Publisher : BoD – Books on Demand
  • Release Date : 2019-01-30
  • Total pages : 84
  • ISBN : 9781789850178
  • File Size : 28,9 Mb
  • Total Download : 746
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The cellular prion protein (PrPC) is expressed as a cell surface protein mainly in the central and peripheral nervous system. The discovery that prions are infectious agents changed the concept of protein synthesis in modern biology and built a bridge between the genesis of infectious and genetic diseases. This book discusses the normal function of the PrPC and its modulatory role in synaptic mechanisms. It describes the pathophysiological processes that accompany TSE - neurotoxicity, loss of anti-inflammatory protective function, neuronal death including prion-induced autophagy and apoptosis, and accumulation of PrPSc in the cytoplasm of neurons. Another aspect outlined here is that some prion diseases show strain variations that determine their development, demonstrating their key role in the development and progression of TSE.

Advancing Prion Science

Advancing Prion Science
  • Author : Institute of Medicine,Medical Follow-up Agency,Committee on Transmissible Spongiform Encephalopathies: Assessment of Relevant Science
  • Publisher : National Academies Press
  • Release Date : 2004-04-04
  • Total pages : 289
  • ISBN : 9780309090605
  • File Size : 40,6 Mb
  • Total Download : 749
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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 TSEsâ€"from basic science to the U.S. research infrastructure, from diagnostics to surveillance, and from prevention to treatment.

Guide to Foodborne Pathogens

Guide to Foodborne Pathogens
  • Author : Ronald G. Labbé,Santos García
  • Publisher : John Wiley & Sons
  • Release Date : 2013-07-12
  • Total pages : 488
  • ISBN : 9781118684832
  • File Size : 37,9 Mb
  • Total Download : 521
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Guide to Foodborne Pathogens coverspathogens—bacteria, viruses, and parasites—that aremost commonly responsible for foodborne illness. An essential guidefor anyone in the food industry, research, or regulation who needsto ensure or enforce food safety, the guide delves into the natureof illnesses, the epidemiology of pathogens, and current detection,prevention, and control methods. The guide further includeschapters on new technologies for microbial detection and theglobalization of the food supply, seafood toxins, and othermiscellaneous agents.

Prions and Brain Diseases in Animals and Humans

Prions and Brain Diseases in Animals and Humans
  • Author : Douglas R.O. Morrison
  • Publisher : Springer Science & Business Media
  • Release Date : 1998-03-31
  • Total pages : 380
  • ISBN : 030645825X
  • File Size : 19,8 Mb
  • Total Download : 141
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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.

Prion Biology

Prion Biology
  • Author : Stanley B. Prusiner
  • Publisher : Unknown
  • Release Date : 2017-04-30
  • Total pages : 350
  • ISBN : 1621820939
  • File Size : 29,7 Mb
  • Total Download : 893
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Diseases such as CreutzfeldtJakob disease and kuru develop when certain proteins form prions by misfolding, clumping together, and spreading from cell to cell. Over the past decade, a number of proteins have been reported to possess prion-like characteristics, and many of these are associated with important human diseases such as Alzheimer's and type 2 diabetes. Written and edited by experts in the field, this collection from Cold Spring Harbor Perspectives in Medicine covers the progress that has recently been made in our understanding of the pathophysiology of prion diseases, as well as ongoing efforts to develop therapeutics. The contributors discuss how proteins such as tau, islet amyloid polypeptide, and a-synuclein aggregate, spread in a prion-like manner, and contribute to various neurodegenerative and extracerebral diseases. Human and animal diseases caused by the prion protein PrP are also covered, as are efforts to develop immunotherapeutics, protein disaggregases, and other drugs to delay or halt their progression. The authors discuss the development and use of cell culture and animal models of prion diseases, as well as the technologies and small molecules that are being used to study them. This volume is therefore useful for all biomedical scientists and physicians wishing to understand and treat this expanding group of devastating diseases.

Prion Biology and Diseases

Prion Biology and Diseases
  • Author : Stanley B. Prusiner
  • Publisher : CSHL Press
  • Release Date : 2004
  • Total pages : 1130
  • ISBN : 0879696931
  • File Size : 53,6 Mb
  • Total Download : 497
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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.