Protein Quality Control In Neurodegenerative Diseases

Author : Richard I. Morimoto,Yves Christen
Publisher : Springer Science & Business Media
Page : 145 pages
File Size : 52,6 Mb
Release : 2012-12-13
Category : Medical
ISBN : 9783642279287

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Protein Quality Control in Neurodegenerative Diseases by Richard I. Morimoto,Yves Christen Pdf

The health of the proteome depends upon protein quality control to regulate the proper synthesis, folding, translocation, and clearance of proteins. The cell is challenged constantly by environmental and physiological stress, aging, and the chronic expressions of disease associated misfolded proteins. Substantial evidence supports the hypothesis that the expression of damaged proteins initiates a cascade of molecular events that leads to Alzheimer's disease, Parkinson's disease, amyotrophic lateral sclerosis, Huntington's disease, and other diseases of protein conformation.

Author : Richard I. Morimoto,Yves Christen
Publisher : Springer
Page : 136 pages
File Size : 40,8 Mb
Release : 2012-12-12
Category : Medical
ISBN : 3642279295

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Protein Quality Control in Neurodegenerative Diseases by Richard I. Morimoto,Yves Christen Pdf

The health of the proteome depends upon protein quality control to regulate the proper synthesis, folding, translocation, and clearance of proteins. The cell is challenged constantly by environmental and physiological stress, aging, and the chronic expressions of disease associated misfolded proteins. Substantial evidence supports the hypothesis that the expression of damaged proteins initiates a cascade of molecular events that leads to Alzheimer's disease, Parkinson's disease, amyotrophic lateral sclerosis, Huntington's disease, and other diseases of protein conformation.

Author : Uddin, Md. Sahab,Ashraf, Ghulam Md.
Publisher : IGI Global
Page : 515 pages
File Size : 44,5 Mb
Release : 2020-02-14
Category : Medical
ISBN : 9781799813187

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Quality Control of Cellular Protein in Neurodegenerative Disorders by Uddin, Md. Sahab,Ashraf, Ghulam Md. Pdf

Protein misfolding and aggregation are hallmarks of several neurodegenerative proteinopathies. Though multiple factors like aging, oxidative stress, mitochondrial dysfunction, proteotoxic insults, genetic inconsistency, etc. are responsible for the dysfunction of the neuronal protein quality control system, targeting protein quality control has become an auspicious approach to halt the propagation of neurodegeneration. Quality Control of Cellular Protein in Neurodegenerative Disorders provides diverse aspects exploring the role of the protein quality control in neurodegenerative disorders and potential therapeutic strategies to combat the development and propagation of neurodegeneration. Featuring coverage on a broad range of topics such as molecular chaperones, protein misfolding, and stress signaling, this book is ideally designed for neurobiologists, neuropsychologists, neurophysiologists, medical professionals, neuropathologists, researchers, academicians, students, and practitioners engaged in studies of the protein quality control system in neuronal cells.

Author : Cintia Roodveldt,Tiago F. Outeiro,Janice E. Braun
Publisher : Frontiers Media SA
Page : 182 pages
File Size : 52,7 Mb
Release : 2017-12-06
Category : Electronic book
ISBN : 9782889453429

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Molecular Chaperones and Neurodegeneration by Cintia Roodveldt,Tiago F. Outeiro,Janice E. Braun Pdf

Molecular chaperones or heat-shock proteins (HSPs) play essential roles in safeguarding structural stability and preventing misfolding and aggregation of proteins, and maintaining the proteome functionality in the cell. For over two decades until the present time, new functions have been discovered and several molecular mechanisms have been elucidated for many chaperones, while the field is being continuously challenged by new open questions. Probably as a consequence of the increasing research on the molecular bases of neurodegenerative diseases, and the realisation that many such disorders are linked to protein misfolding processes, unleashing the roles and mechanisms of chaperones in the context of neurodegeneration has become a prime scientific goal. This e-book contains a diversity of reviews, perspective and original research articles highlighting the importance and potential of this emerging subject.

Author : Peter Bross,Niels Gregersen
Publisher : Springer Science & Business Media
Page : 317 pages
File Size : 49,6 Mb
Release : 2008-02-02
Category : Science
ISBN : 9781592593941

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Protein Misfolding and Disease by Peter Bross,Niels Gregersen Pdf

For decades it has been known that structured conformations are important for the proper functioning of most cellular proteins. However, appreciation that protein folding to the functional conformations as well as the structural maintenance of protein molecules are very complex processes has only emerged during the last ten years. The intimate interplay uncovered by this scientific development led us to realize that perturbations of the protein folding process and disturbances of conformational maintenance are major disease mechanisms. This development has given rise to the concept of conformational diseases and the broader signature of protein folding diseases, comprising diseases in which mutations or environmental stresses may result in a partial misfolding that leads then to alternative conformations capable of disturbing cellular processes. This may happen by self-association (aggregation), as in prion and Alzheimer’s diseases, or by incorporation of alternatively folded subunits into structural entities, as in collagen diseases. Another possibility is that folding to the native structure is impaired or abolished, resulting in decreased stea- state levels of the correctly folded protein, as is observed in cystic fibrosis and 1-antitrypsin deficiency, as well as in many enzyme deficiencies. In addition, deficiencies of proteins that are engaged in assisting and supervising protein folding (protein quality control) may impair the folding of many other proteins, resulting in pathological phenotypes. Examples of this are the spastic paraplegia attributable to mutations in mitochondrial protease/chaperone complexes.

Author : Judit Ovádi,Ferenc Orosz
Publisher : Springer Science & Business Media
Page : 284 pages
File Size : 53,8 Mb
Release : 2008-12-21
Category : Medical
ISBN : 9781402094347

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Protein folding and misfolding: neurodegenerative diseases by Judit Ovádi,Ferenc Orosz Pdf

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.

Author : Martin Beckerman
Publisher : Springer
Page : 378 pages
File Size : 43,7 Mb
Release : 2015-11-06
Category : Medical
ISBN : 9783319221175

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Fundamentals of Neurodegeneration and Protein Misfolding Disorders by Martin Beckerman Pdf

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.

Author : Robert D. E. Sewell
Publisher : CRC Press
Page : 592 pages
File Size : 54,9 Mb
Release : 2019-08-30
Category : Electronic
ISBN : 036738812X

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Protein Misfolding in Neurodegenerative Diseases by Robert D. E. Sewell Pdf

Research focused on protein folding, misfolding, and aggregation is leading to major advances across biochemistry and medicine. The elucidation of a folding code is proving to be of extreme importance in the postgenomic era, where a number of orphan genes have been identified for which no clear function has yet been established. This research is starting to shed light on the molecular and biochemical basis of a number of neurodegenerative diseases of dramatic impact. Protein Misfolding in Neurodegenerative Diseases: Mechanisms and Therapeutic Strategies addresses key issues concerning protein misfolding and aggregation in neurodegenerative diseases. Building on recent developments, including the recognition of protein misfolding as both a marker and a causal agent, the text presents the work of those who are actively pursuing more effective treatments, as well as preventative measures, and a possible cure. These include the use of molecular chaperones to control misfolding and novel pharmaceuticals, as well as the potential role of various inhibitors and NSAIDS. A Comprehensive Multifaceted Examination of the Complex Causal Agents Implicated in Protein Misfolding Divided into five sections, this groundbreaking text provides up-to-date accounts for Alzheimer's, Parkinson's, Huntington's, Amyotrophic Lateral Sclerosis and Transmissible Spongiform Encephalitis. It also explores the highly likelihood that multiple factors, including oxidative stress, play a role in these complex diseases.

Author : Anonim
Publisher : Academic Press
Page : 498 pages
File Size : 46,7 Mb
Release : 2012-05-22
Category : Science
ISBN : 9780123858849

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Molecular Biology of Neurodegenerative Diseases by Anonim Pdf

Neurodegenerative diseases result in progressive degeneration and / or death of nerve cells which leads to problems with movement and mental functioning. Examples include Parkinson’s, Alzheimer’s and Huntington’s disease. Much research is taking place to try to identify ways to prevent or lessen the impact of these diseases. This volume reviews the latest research and developments in the molecular biology of neurodegenerative diseases. Contributions from leading authorities Informs and updates on all the latest developments in the field

Author : Leonidas Stefanis,J. N. Keller
Publisher : Springer
Page : 306 pages
File Size : 52,7 Mb
Release : 2006-02-07
Category : Medical
ISBN : 0387284990

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The Proteasome in Neurodegeneration by Leonidas Stefanis,J. N. Keller Pdf

In the last 50 years a wealth of information has allowed us to understand the contribution of various regulatory factors that alter mRNA and protein s- thesis to a variety of physiological and pathological conditions. However, such regulation is only one of many factors that contribute to the levels of a given p- tein. One major factor that has been relatively obscure until recently has been the contribution of protein degradation to the regulation of the steady state level of protein expression and protein function. This rapidly evolving field has made a significant mark on the scientific community, as highlighted by the Award of the Nobel Prize in Chemistry for 2004 to Aaron Ciechanover, Avram Hershko and Irwin Rose for their pioneering work on the ubiquitin-proteasome system (UPS) of protein degradation, which is the subject of this volume. In recent years e- dence has been accumulating that suggests a role for UPS function in both ph- iological and pathological settings. In particular, studies have implicated a central role for the UPS in cell cycle regulation, cancer and neurodegeneration. Two points are however worth bearing in mind: First, ubiquitin’s function appears to extend far beyond the UPS and protein degradation; second, there are other important systems of intracellular protein degradation, most notably autophagic systems through the lysosomes, and these may also be involved in disease pat- physiology.

Author : Michael S. Wolfe
Publisher : Academic Press
Page : 560 pages
File Size : 50,7 Mb
Release : 2018-03-29
Category : Medical
ISBN : 9780128113059

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The Molecular and Cellular Basis of Neurodegenerative Diseases by Michael S. Wolfe Pdf

The Molecular and Cellular Basis of Neurodegenerative Diseases: Underlying Mechanisms presents the pathology, genetics, biochemistry and cell biology of the major human neurodegenerative diseases, including Alzheimer’s, Parkinson’s, frontotemporal dementia, ALS, Huntington’s, and prion diseases. Edited and authored by internationally recognized leaders in the field, the book's chapters explore their pathogenic commonalities and differences, also including discussions of animal models and prospects for therapeutics. Diseases are presented first, with common mechanisms later. Individual chapters discuss each major neurodegenerative disease, integrating this information to offer multiple molecular and cellular mechanisms that diseases may have in common. This book provides readers with a timely update on this rapidly advancing area of investigation, presenting an invaluable resource for researchers in the field. Covers the spectrum of neurodegenerative diseases and their complex genetic, pathological, biochemical and cellular features Focuses on leading hypotheses regarding the biochemical and cellular dysfunctions that cause neurodegeneration Details features, advantages and limitations of animal models, as well as prospects for therapeutic development Authored by internationally recognized leaders in the field Includes illustrations that help clarify and consolidate complex concepts

Author : Uday Kishore
Publisher : BoD – Books on Demand
Page : 642 pages
File Size : 51,5 Mb
Release : 2013-05-15
Category : Medical
ISBN : 9789535110880

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Neurodegenerative Diseases by Uday Kishore Pdf

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.

Author : Richard I. Morimoto,Dennis J. Selkoe,Jeffery W. Kelly
Publisher : Unknown
Page : 0 pages
File Size : 43,9 Mb
Release : 2012
Category : Biological transport
ISBN : 1936113066

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Protein Homeostasis by Richard I. Morimoto,Dennis J. Selkoe,Jeffery W. Kelly Pdf

Proper folding of proteins is crucial for cell function. Chaperones and enzymes that post-translationally modify newly synthesized proteins help ensure that proteins fold correctly, and the unfolded protein response functions as a homeostatic mechanism that removes misfolded proteins when cells are stressed. This book covers the entire spectrum of proteostasis in healthy cells and the diseases that result when control of protein production, protein folding, and protein degradation goes awry.

Author : Rahul Sunder Rajan
Publisher : Unknown
Page : 310 pages
File Size : 45,6 Mb
Release : 2003
Category : Electronic
ISBN : STANFORD:36105023748408

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The Role of ER Quality Control and Protein Misfolding in Retinal Degeneration Caused by a Mutation in Rhodopsin by Rahul Sunder Rajan Pdf

Author : Pierfausto Seneci
Publisher : Academic Press
Page : 260 pages
File Size : 48,6 Mb
Release : 2015-01-14
Category : Medical
ISBN : 9780128019597

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Chemical Modulators of Protein Misfolding and Neurodegenerative Disease by Pierfausto Seneci Pdf

This book is a neurochemistry-based companion for Protein Misfolding and Neurodegenerative Diseases: Molecular Targets, an Elsevier title by the same author publishing in December 2014. While the first book focuses on biology and molecular targets, this companion book describes how these targets are regulated by small molecules and disease-modifying compounds. The book begins with a brief introduction to how key proteins become dysfunctional, and each subsequent chapter describes major disease mechanisms in Alzheimer’s and other tauopathies. Properties and development status of these molecular targets and disease mechanisms are thoroughly described, as are small molecule effectors of autophagy and dis-aggregating agents. Written to provide comprehensive coverage of neurodegenerative disease-modifying compounds Provides discipline-specific chapters that cover medicinal chemistry and clinical applications Provides an overview of more than 200 chemical classes and lead compounds, acting on selected molecular targets that are of relevance to any neurodegenerative disorder Coverage of misfolding diseases, chaperone proteins, ubiquitination and autophagy/oncology makes this book suitable for structural neurochemists, chemists, biologists, non-CNS scientists, and scientists interested in drug discovery