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The Prion Protein


The Prion Protein
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Prion Protein


Prion Protein
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Author :
language : en
Publisher: Academic Press
Release Date : 2017-08-22

Prion Protein written by and has been published by Academic Press this book supported file pdf, txt, epub, kindle and other format this book has been release on 2017-08-22 with Science categories.


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



Prion Proteins


Prion Proteins
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Author : Jörg Tatzelt
language : en
Publisher: Springer
Release Date : 2011-09-15

Prion Proteins written by Jörg Tatzelt and has been published by Springer this book supported file pdf, txt, epub, kindle and other format this book has been release on 2011-09-15 with Science categories.


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.



The Prion Protein


The Prion Protein
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Author : Jorg Tatzelt
language : en
Publisher:
Release Date : 2010

The Prion Protein written by Jorg Tatzelt and has been published by this book supported file pdf, txt, epub, kindle and other format this book has been release on 2010 with Science categories.


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


Prions
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Author : Claudio Soto
language : en
Publisher: CRC Press
Release Date : 2005-12-20

Prions written by Claudio Soto and has been published by CRC Press this book supported file pdf, txt, epub, kindle and other format this book has been release on 2005-12-20 with Medical categories.


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



Prion Biology


Prion Biology
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Author : Vincent Béringue
language : en
Publisher: CRC Press
Release Date : 2013-04-08

Prion Biology written by Vincent Béringue and has been published by CRC Press this book supported file pdf, txt, epub, kindle and other format this book has been release on 2013-04-08 with Science categories.


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.



Molecular Pathology Of The Prions


Molecular Pathology Of The Prions
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Author : Harry F. Baker
language : en
Publisher: Springer Science & Business Media
Release Date : 2008-02-02

Molecular Pathology Of The Prions written by Harry F. Baker and has been published by Springer Science & Business Media this book supported file pdf, txt, epub, kindle and other format this book has been release on 2008-02-02 with Medical categories.


Internationally recognized investigators review the latest developments in, and novel approaches to, understanding the prion protein and prion diseases at the molecular level. Utilizing a variety of cutting-edge techniques, these distinguished scientists seek to define the normal function of a prion protein, to detect and measure the early immune response to prion disease, and to discover possible therapeutic targets. They also use transgenic mice and new electrophysiological investigations to elucidate the pathogenetic mechanisms involved in prion diseases. State-of-the-art and richly insightful, Molecular Pathology of the Prions captures for basic and clinical neuropathologists the latest developments and approaches to understanding the pathogenesis of prion diseases, and by analogy suggests possible research techniques for the more common proteinopthies, such as Alzheimer's and Parkinson's diseases.



Promiscuous Functions Of The Prion Protein Gene Family


Promiscuous Functions Of The Prion Protein Gene Family
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Author : Sophie Mouillet-Richard
language : en
Publisher: Frontiers Media SA
Release Date : 2015-07-22

Promiscuous Functions Of The Prion Protein Gene Family written by Sophie Mouillet-Richard and has been published by Frontiers Media SA this book supported file pdf, txt, epub, kindle and other format this book has been release on 2015-07-22 with Biology (General) categories.


The cellular prion protein PrPC is a ubiquitous GPI-anchored protein. While PrPC has been the focus of intense research for its involvement in a group of neurodegenerative disorders known as transmissible spongiform encephalopathies (TSE), much less attention has been devoted to its physiological function. This notably relates to the lack of obvious abnormalities of mice, goat or cattle lacking PrPC. This apparently normal phenotype in these PrPC-deficient animals however contrasts with the very high degree of conservation of the prion protein gene (Prnp) in mammalian species (over 80%), and the presence of genes with similarities to Prnp in birds, reptiles, amphibians and fish. This high conservation together with its ubiquitous expression, - albeit at highest levels in the brain-, suggest that PrPC has major physiological functions. Dissecting PrPC function is further complicated by the occurrence, in mammals, of two potentially partially redundant homologues, Doppel, and Shadoo. The biological overlaps between members of the prion protein family are still under investigation and much debated. Similarly, although in vitro analyses have suggested various functions for PrPC, notably in cell death and survival processes, some have yielded conflicting results and/or discrepancies with in vivo studies. This Research Topic brings together the accumulated knowledge regarding the biological roles of the prion protein family, from the animal to the molecular scale.



Prion


Prion
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Author : Yusuf Tutar
language : en
Publisher: BoD – Books on Demand
Release Date : 2017-03-08

Prion written by Yusuf Tutar and has been published by BoD – Books on Demand this book supported file pdf, txt, epub, kindle and other format this book has been release on 2017-03-08 with Medical categories.


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 And Diseases


Prions And Diseases
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Author : Wen-Quan Zou
language : en
Publisher: Springer Science & Business Media
Release Date : 2012-11-27

Prions And Diseases written by Wen-Quan Zou and has been published by Springer Science & Business Media this book supported file pdf, txt, epub, kindle and other format this book has been release on 2012-11-27 with Medical categories.


Volume I highlights the association of the cellular prion protein (PrPC) with copper and zinc, the potential roles of PrPC in Alzheimer’s disease and cancers, insoluble PrPC, PMCA, molecular and cellular mechanisms of PrPSc formation and clearance, possible co-factors involved in the conversion of PrPC into PrPSc, infectious and pathogenic forms of PrP, cell biology of prions, prion strains and their interference, as well as yeast prions and their inheritable and structural traits. This unique volume will take you through the fascinating chronicle of prions in mammals, yeast, and fungi.



Molecular Structures And Structural Dynamics Of Prion Proteins And Prions


Molecular Structures And Structural Dynamics Of Prion Proteins And Prions
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Author : Jiapu Zhang
language : en
Publisher: Springer
Release Date : 2015-09-14

Molecular Structures And Structural Dynamics Of Prion Proteins And Prions written by Jiapu Zhang and has been published by Springer this book supported file pdf, txt, epub, kindle and other format this book has been release on 2015-09-14 with Science categories.


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.