Protein Folding Disorders Of The Central Nervous System
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Protein Folding Disorders Of The Central Nervous System
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Author : Ghiso Jorge A
language : en
Publisher: World Scientific
Release Date : 2017-09-15
Protein Folding Disorders Of The Central Nervous System written by Ghiso Jorge A and has been published by World Scientific this book supported file pdf, txt, epub, kindle and other format this book has been release on 2017-09-15 with Medical categories.
This exciting new book explores the dark side of the molecular protein assembly bringing an updated view of how failures in the homeostatic mechanisms that efficiently regulate protein folding leads to the accumulation of structurally abnormal pathogenic assemblies, encompassing an emerging group of diseases collectively known as "Protein Folding Disorders." This complex and diverse group of chronic and progressive entities are bridged together by their relationship to structural transitions in the native state of specific proteinaceous components, which for reasons poorly understood, convert into polymeric aggregates that generate poorly soluble tissue deposits and which are considered today the culprit of the disease pathogenesis in their respective diseases. Despite the diversity in the amino acid sequence of the different proteins involved in these heterogeneous disorders, all the pathologic conformers can trigger cascades of events ultimately resulting in cell dysfunction and death with devastating clinical consequences in many of the most precious aspects of human existence including personality, cognition, memory, and skilled movements. This book, which is composed of a compilation of chapters authored by outstanding and well-published scientists in the respective fields currently performing active investigations at world renowned universities and research centers, focuses on the growing number of diseases associated with protein misfolding in the central nervous system. Individual chapters are dedicated to the most common neurodegenerative diseases associated with protein aggregation/fibrillization focusing on the nature of the pathogenic species and the cellular pathways involved in the molecular pathogenesis of Alzheimer's, Parkinson's, and Huntington's diseases as well as in Amyotrophic Lateral Sclerosis, and Prion disorders. A group of contributions is centered on the current knowledge of the intracellular pathways and subcellular organelles affected by the different disease conditions, while others are focused in the emerging pathogenic role of misfolded subunits assembled into neurotoxic soluble oligomers, and in the novel notion of the transmissibility of the protein misfolded species, an innovative concept until recently only accepted for Prion diseases. Lastly, a different set of chapters is dedicated to the evaluation of novel therapeutic strategies for these devastating diseases. Contents: Misfolding, Aggregation, and Amyloid Formation: The Dark Side of Proteins (Agueda Rostagno and Jorge A Ghiso)Oligomers at the Synapse: Synaptic Dysfunction and Neurodegeneration (Emily Vogler, Matthew Mahavongtrakul, and Jorge Busciglio)Prion-Like Protein Seeding and the Pathobiology of Alzheimer's Disease (Lary C Walker)The Tau Misfolding Pathway to Dementia (Alejandra D Alonso, Leah S Cohen, and Viktoriya Morozova)The Biology and Pathobiology of α-Synuclein (Joel C Watts, Anurag Tandon, and Paul E Fraser)Impact of Loss of Proteostasis on Central Nervous System Disorders (Sentiljana Gumeni, Eleni N Tsakiri, Christina-Maria Cheimonidi, Zoi Evangelakou, Despoina Gianniou, Kostantinos Tallas, Eleni-Dimitra Papanagnou, Aimilia D Sklirou, and Ioannis P Trougakos)Protein Misfolding and Mitochondrial Dysfunction in Amyotrophic Lateral Sclerosis (Giovanni Manfredi and Hibiki Kawamata)Impact of Mitostasis and the Role of the Anti-Oxidant Responses on Central Nervous System Disorders (Sentiljana Gumeni, Eleni N Tsakiri, Christina-Maria Cheimonidi, Zoi Evangelakou, Despoina Gianniou, Kostantinos Tallas, Eleni-Dimitra Papanagnou, Aimilia D Sklirou, and Ioannis P Trougakos)Propagation of Misfolded Proteins in Neurodegeneration: Insights and Cautions from the Study of Prion Disease Prototypes (Robert C C Mercer, Nathalie Daude,
Protein Misfolding In Neurodegenerative Diseases
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Author : Robert D. E. Sewell
language : en
Publisher: CRC Press
Release Date : 2007-12-03
Protein Misfolding In Neurodegenerative Diseases written by Robert D. E. Sewell and has been published by CRC Press this book supported file pdf, txt, epub, kindle and other format this book has been release on 2007-12-03 with Medical categories.
Current research suggests that neurodegenerative diseases such as Alzheimer's, Parkinson's, Huntington's, and Creutzfeldt-Jacob may be linked to disorders in protein shape referred to as protein misfolding. Continued study in this area could lead to promising advances in future treatment of these diseases. This groundbreaking text describes the latest findings regarding protein misfolding in the context of it being a marker, and perhaps a cause, in neurodegenerative diseases. Comprehensive coverage includes the diverse biochemical targets/markers for each disease, the currently limited success of drug therapies, and the cutting-edge research that could lead to more promising treatments.
Fundamentals Of Neurodegeneration And Protein Misfolding Disorders
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Author : Martin Beckerman
language : en
Publisher:
Release Date : 2015
Fundamentals Of Neurodegeneration And Protein Misfolding Disorders written by Martin Beckerman and has been published by this book supported file pdf, txt, epub, kindle and other format this book has been release on 2015 with categories.
This unique text introduces students and researchers to the world of misfolded proteins, toxic oligomers, and amyloid assemblages, and the diseases of the brain that result. During the past few years the connections between failures in protein quality control and neurological disorders have been reinforced and strengthened by discoveries on multiple fronts. These findings provide novel insights on how amyloidogenic oligomers and fibrils form, interconvert from one state to another, and propagate from cell to cell and region to region. Starting with protein folding and protein quality control basics, the reader will learn how misfolded proteins can cause diseases ranging from prion diseases to Alzheimer's disease and Parkinson's disease to Huntington's disease, amyotrophic lateral sclerosis and frontotemporal lobar degeneration. Authoritative but written in a clear and engaging style, Fundamentals of Neurodegeneration and Protein Misfolding Disorders addresses one of today's forefront areas of science and medicine. The text emphasizes the new groundbreaking biophysical and biochemical methods that enable molecular-level explorations and the conceptual breakthroughs that result. It contains separate chapters on each of the major disease classes. Special emphasis is placed on those factors and themes that are common to the diseases, especially failures in synaptic transmission, mitochondrial control, and axonal transport; breakdowns in RNA processing; the potential role of environmental factors; and the confounding effects of neuroinflammation. The book is ideal for use in teaching at the advanced undergraduate and graduate levels, and serves as a comprehensive reference for a broad audience of students and researchers in neuroscience, molecular biology, biological physics and biomedical engineering.
Protein Folding And Misfolding Neurodegenerative Diseases
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Author : Judit Ovádi
language : en
Publisher: Springer Science & Business Media
Release Date : 2008-12-21
Protein Folding And Misfolding Neurodegenerative Diseases written by Judit Ovádi 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 2008-12-21 with Medical categories.
Offering all the latest in the study of neurodegenerative diseases, this book reviews the molecular events initiated by unfolded or misfolded proteins leading to conformational human diseases, especially those found in Parkinson’s and Alzheimer’s diseases.
Protein Misfolding Disorders
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Author : Claudio Hetz
language : en
Publisher: Bentham Science Publishers
Release Date : 2009
Protein Misfolding Disorders written by Claudio Hetz and has been published by Bentham Science Publishers this book supported file pdf, txt, epub, kindle and other format this book has been release on 2009 with Medical categories.
Neurodegenerative disorders such as Amyotrophic lateral sclerosis (ALS), Alzheimer’s disease (AD), Parkinson’s disease (PD), Prion-related disorders (PrD) and Huntington’s disease (HD) share a common neuropathology, primarily featuring the presence of abnormal protein inclusions containing specific misfolded proteins. These groups of diseases are now classified as Protein Misfolding Disorders. This book gives a comprehensive overview of the possible mechanisms involved in Protein Misfolding Disorders and possible therapeutic strategies to treat these diseases. The Ebook provides the most recent evidence addressing the role of cellular stress responses to neurological diseases, along with therapeutic strategies to alleviate ER stress in a disease context. -- Publisher.
Non Fibrillar Amyloidogenic Protein Assemblies Common Cytotoxins Underlying Degenerative Diseases
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Author : Farid Rahimi
language : en
Publisher: Springer
Release Date : 2014-02-24
Non Fibrillar Amyloidogenic Protein Assemblies Common Cytotoxins Underlying Degenerative Diseases written by Farid Rahimi and has been published by Springer this book supported file pdf, txt, epub, kindle and other format this book has been release on 2014-02-24 with Medical categories.
Amyloid-forming proteins are implicated in over 30 human diseases. The proteins involved in each disease have unrelated sequences and dissimilar native structures, but they all undergo conformational alterations to form fibrillar polymers. The fibrillar assemblies accumulate progressively into disease-specific lesions in vivo. Substantial evidence suggests these lesions are the end state of aberrant protein folding whereas the actual disease-causing culprits likely are soluble, non-fibrillar assemblies preceding the aggregates. The non-fibrillar protein assemblies range from small, low-order oligomers to spherical, annular, and protofibrillar species. Oligomeric species are believed to mediate various pathogenic mechanisms that lead to cellular dysfunction, cytotoxicity, and cell loss, eventuating in disease-specific degeneration and systemic morbidity. The particular pathologies thus are determined by the afflicted cell types, organs, systems, and the proteins involved. Evidence suggests that the oligomeric species may share structural features and possibly common mechanisms of action. In many cases, the structure–function interrelationships amongst the various protein assemblies described in vitro are still elusive. Deciphering these intricate structure–function correlations will help understanding a complex array of pathogenic mechanisms, some of which may be common across different diseases albeit affecting different cell types and systems.
Tau Oligomers
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Author : Jesus Avila
language : en
Publisher: Frontiers E-books
Release Date : 2014-08-18
Tau Oligomers written by Jesus Avila and has been published by Frontiers E-books this book supported file pdf, txt, epub, kindle and other format this book has been release on 2014-08-18 with Medicine (General) categories.
Neurofibrillary tangles (NFTs) composed of intracellular aggregates of tau protein are a key neuropathological feature of Alzheimer’s Disease (AD) and other neurodegenerative diseases, collectively termed tauopathies. The abundance of NFTs has been reported to correlate positively with the severity of cognitive impairment in AD. However, accumulating evidences derived from studies of experimental models have identified that NFTs themselves may not be neurotoxic. Now, many of tau researchers are seeking a “toxic” form of tau protein. Moreover, it was suggested that a “toxic” tau was capable to seed aggregation of native tau protein and to propagate in a prion-like manner. However, the exact neurotoxic tau species remain unclear. Because mature tangles seem to be non-toxic component, “tau oligomers” as the candidate of “toxic” tau have been investigated for more than one decade. In this topic, we will discuss our consensus of “tau oligomers” because the term of “tau oligomers” [e.g. dimer (disulfide bond-dependent or independent), multimer (more than dimer), granular (definition by EM or AFM) and maybe small filamentous aggregates] has been used by each researchers definition. From a biochemical point of view, tau protein has several unique characteristics such as natively unfolded conformation, thermo-stability, acid-stability, and capability of post-translational modifications. Although tau protein research has been continued for a long time, we are still missing the mechanisms of NFT formation. It is unclear how the conversion is occurred from natively unfolded protein to abnormally mis-folded protein. It remains unknown how tau protein can be formed filaments [e.g. paired helical filament (PHF), straight filament and twisted filament] in cells albeit in vitro studies confirmed tau self-assembly by several inducing factors. Researchers are still debating whether tau oligomerization is primary event rather than tau phosphorylation in the tau pathogenesis. Inhibition of either tau phosphorylation or aggregation has been investigated for the prevention of tauopathies, however, it will make an irrelevant result if we don’t know an exact target of neurotoxicity. It is a time to have a consensus of definition, terminology and methodology for the identification of “tau oligomers”.
Tauopathies Advances In Research And Treatment 2013 Edition
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Author :
language : en
Publisher: ScholarlyEditions
Release Date : 2013-06-21
Tauopathies Advances In Research And Treatment 2013 Edition written by and has been published by ScholarlyEditions this book supported file pdf, txt, epub, kindle and other format this book has been release on 2013-06-21 with Medical categories.
Tauopathies—Advances in Research and Treatment: 2013 Edition is a ScholarlyBrief™ that delivers timely, authoritative, comprehensive, and specialized information about ZZZAdditional Research in a concise format. The editors have built Tauopathies—Advances in Research and Treatment: 2013 Edition on the vast information databases of ScholarlyNews.™ You can expect the information about ZZZAdditional Research in this book to be deeper than what you can access anywhere else, as well as consistently reliable, authoritative, informed, and relevant. The content of Tauopathies—Advances in Research and Treatment: 2013 Edition has been produced by the world’s leading scientists, engineers, analysts, research institutions, and companies. All of the content is from peer-reviewed sources, and all of it is written, assembled, and edited by the editors at ScholarlyEditions™ and available exclusively from us. You now have a source you can cite with authority, confidence, and credibility. More information is available at http://www.ScholarlyEditions.com/.
Physical Biology
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Author : Ahmed H. Zewail
language : en
Publisher: World Scientific
Release Date : 2008
Physical Biology written by Ahmed H. Zewail and has been published by World Scientific this book supported file pdf, txt, epub, kindle and other format this book has been release on 2008 with Science categories.
Addresses significant problems in physical biology and adjacent disciplines. This volume provides a perspective on the methods and concepts at the heart of chemical and biological behavior, covering the topics of visualization; theory and computation for complexity; and macromolecular function, protein folding, and protein misfolding
Neurodegenerative Diseases
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Author : Uday Kishore
language : en
Publisher: BoD – Books on Demand
Release Date : 2013-05-15
Neurodegenerative Diseases written by Uday Kishore 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 2013-05-15 with Medical categories.
This book highlights the pathophysiological complexities of the mechanisms and factors that are likely to be involved in a range of neuroinflammatory and neurodegenerative diseases including Alzheimer's disease, other Dementia, Parkinson Diseases and Multiple Sclerosis. The spectrum of diverse factors involved in neurodegeneration, such as protein aggregation, oxidative stress, caspases and secretase, regulators, cholesterol, zinc, microglia, astrocytes, oligodendrocytes, etc, have been discussed in the context of disease progression. In addition, novel approaches to therapeutic interventions have also been presented. It is hoped that students, scientists and clinicians shall find this very informative book immensely useful and thought-provoking.