The Prion Protein

Author : Anonim
Publisher : Elsevier
Page : 512 pages
File Size : 53,7 Mb
Release : 2018-06-07
Category : Medical
ISBN : 9780444639530

Get Book

Human Prion Diseases by Anonim Pdf

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 : Jörg Tatzelt
Publisher : Springer Science & Business Media
Page : 308 pages
File Size : 45,9 Mb
Release : 2011-09-15
Category : Science
ISBN : 9783642240669

Get Book

Prion Proteins by Jörg Tatzelt Pdf

Genetics of Prion Disease, by S. Lloyd, S. Mead and J. Collinge. Atypical Prion Diseases in Humans and Animals, by M. A. Tranulis, S. L. Benestad, T. Baron and H. Kretzschmar. Chronic Wasting Disease, by S. Gilch, N. Chitoor, Y. Taguchi, M. Stuart, J. E. Jewell and H. M. Schätzl. Transgenic Mouse Models and Prion Strains, by G. C. Telling. Neuroprotective and Neurotoxic Signaling by the Prion Protein, by U. K. Resenberger, K. F. Winklhofer and J. Tatzelt. Prion Seeded Conversion and Amplification Assays, by C. D. Orrú and B. Caughey. Prion Protein and Its Conformational Conversion: A Structural Perspective, by W. K. Surewicz and M. I. Apostol. Molecular Dynamics as an Approach to Study Prion Protein Misfolding and the Effect of Pathogenic Mutations, by M.W. van der Kamp and V. Daggett. Chemical Biology of Prion Protein: Tools to Bridge the In Vitro/Vivo Interface, by R. Seidel and M. Engelhard. The PrP-Like Proteins Shadoo and Doppel, by D. Westaway, N. Daude, S. Wohlgemuth and P. Harrison. Fungal Prions: Structure, Function and Propagation, by M. F. Tuite, R. Marchante and V. Kushnirov.

Author : Anonim
Publisher : Academic Press
Page : 516 pages
File Size : 41,9 Mb
Release : 2017-08-22
Category : Science
ISBN : 9780128112274

Get Book

Prion Protein by Anonim Pdf

Prion Protein, Volume 150, the latest volume in the Progress in Molecular Biology and Translational Science series, focuses on Prion Protein, a protein that is considered to be the archetype of intrinsically disordered proteins. This updated volume includes comprehensive sections on a variety of timely topics, including Functions of Prion Protein, Copper and Prion protein, Cell Biology of Prion Protein, Structural Studies of Prion Proteins, Molecular Simulations on Wild Type and Mutant Prion Proteins, Genetics of Prion Protein, The Prion Concept and Synthetic Prions, and Transgenic Mouse Models. As neurodegenerative diseases represent a health issue that is receiving increasing attention from the scientific community due to their social and economic impact, this series is an ideal resource for the latest research in molecular biology and translational science. Presents the latest volume in the Progress in Molecular Biology and Translational Science series Accessible to students and researcher alike Written by leading authorities in the field of prion protein

Author : Jorg Tatzelt
Publisher : Unknown
Page : 80 pages
File Size : 54,5 Mb
Release : 2010
Category : Prions
ISBN : 0954333527

Get Book

The Prion Protein by Jorg Tatzelt Pdf

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 : Jiapu Zhang
Publisher : Springer
Page : 355 pages
File Size : 41,9 Mb
Release : 2015-09-14
Category : Science
ISBN : 9789401773188

Get Book

Molecular Structures and Structural Dynamics of Prion Proteins and Prions by Jiapu Zhang Pdf

This monograph is the first easy-to-read-and-understand book on prion proteins' molecular dynamics (MD) simulations and on prions' molecular modelling (MM) constructions. It enables researchers to see what is crucial to the conformational change from normal cellular prion protein (PrPC) to diseased infectious prions (PrPSc), using MD and MM techniques. As we all know, prion diseases, caused by the body's own proteins, are invariably fatal and highly infectious neurodegenerative diseases effecting humans and almost all animals for a major public health concern. Prion contains no nucleic acids and it is a misshapen or conformation-changed protein that acts like an infectious agent; thus prion diseases are called “protein structural conformational” diseases. PrPC is predominant in α-helices but PrPSc are rich in β-sheets in the form as amyloid fibrils; so very amenable to be studied by MD techniques. Through MD, studies on the protein structures and the structural conversion are very important for revealing secrets of prion diseases and for structure-based drug design or discovery. Rabbits, dogs, horses and buffaloes are reported to be the few low susceptibility species to prion diseases; this book's MD studies on these species are clearly helpful to understand the mechanism underlying the resistance to prion diseases. PrP(1-120) usually has no clear molecular structures; this book also studies this unstructured region through MD and especially MM techniques from the global optimization point of view. This book is ideal for practitioners in computing of biophysics, biochemistry, biomedicine, bioinformatics, cheminformatics, materials science and engineering, applied mathematics and theoretical physics, information technology, operations research, biostatistics, etc. As an accessible introduction to these fields, this book is also ideal as a teaching material for students.

Author : Jorg Tatzelt
Publisher : Unknown
Page : 86 pages
File Size : 53,7 Mb
Release : 2010
Category : Science
ISBN : 1912530074

Get Book

The Prion Protein by Jorg Tatzelt Pdf

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 : Claudio Soto
Publisher : CRC Press
Page : 184 pages
File Size : 51,9 Mb
Release : 2005-12-20
Category : Medical
ISBN : 9781420040128

Get Book

Prions by Claudio Soto Pdf

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

Author : Anonim
Publisher : Elsevier
Page : 480 pages
File Size : 42,7 Mb
Release : 2018-01-09
Category : Medical
ISBN : 9780444640772

Get Book

Neurogenetics by Anonim Pdf

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

Author : Ivo Nikolaev Sirakov
Publisher : BoD – Books on Demand
Page : 84 pages
File Size : 50,9 Mb
Release : 2019-01-30
Category : Medical
ISBN : 9781789850178

Get Book

Prions by Ivo Nikolaev Sirakov Pdf

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.

Author : Stanley B. Prusiner
Publisher : Springer
Page : 0 pages
File Size : 50,7 Mb
Release : 2011-09-21
Category : Medical
ISBN : 3642646409

Get Book

Prions Prions Prions by Stanley B. Prusiner Pdf

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.

Author : Stanley B. Prusiner
Publisher : Yale University Press
Page : 344 pages
File Size : 43,6 Mb
Release : 2014-04-29
Category : Science
ISBN : 9780300191141

Get Book

Madness and Memory by Stanley B. Prusiner Pdf

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 : David R. Brown
Publisher : Springer Science & Business Media
Page : 498 pages
File Size : 45,6 Mb
Release : 2005-05-06
Category : Medical
ISBN : 0387239227

Get Book

Neurodegeneration and Prion Disease by David R. Brown Pdf

This is the first and only book on the subject of prions to cover the cause of cell death in the disease. It covers the full range of competing theories on the subject, from broad description and basic points up to the final details of the basic science.

Author : Stanley B. Prusiner,Michael P. McKinley
Publisher : Unknown
Page : 568 pages
File Size : 46,9 Mb
Release : 1987
Category : Creutzfeldt-Jakob disease
ISBN : UOM:39015035236846

Get Book

Prions by Stanley B. Prusiner,Michael P. McKinley Pdf

Author : Vincent Béringue
Publisher : CRC Press
Page : 283 pages
File Size : 40,7 Mb
Release : 2013-04-08
Category : Science
ISBN : 9781926895376

Get Book

Prion Biology by Vincent Béringue Pdf

This book contains a selection of chapters aimed to provide a better understanding prion structure and biology. Together these chapters provide an overview of prion biology and underscore some of the challenges we face if we want to understand how this lively pathogen propagates and evolves in mammals. There is also mounting evidence that studying prion biology has a wider relevance due to similarities in the processes of protein misfolding and aggregation between prion disorders and other neurodegenerative disorders such as Alzheimer’s, Parkinson’s, and Huntington’s diseases.

Author : Institute of Medicine,Medical Follow-up Agency,Committee on Transmissible Spongiform Encephalopathies: Assessment of Relevant Science
Publisher : National Academies Press
Page : 125 pages
File Size : 52,5 Mb
Release : 2003-03-20
Category : Medical
ISBN : 9780309168595

Get Book

Advancing Prion Science by Institute of Medicine,Medical Follow-up Agency,Committee on Transmissible Spongiform Encephalopathies: Assessment of Relevant Science Pdf

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.