Download or read book Quality Control of Cellular Protein in Neurodegenerative Disorders written by Uddin, Md. Sahab and published by IGI Global. This book was released on 2020-02-14 with total page 515 pages. Available in PDF, EPUB and Kindle. Book excerpt: 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.
Download or read book Protein Quality Control in Neurodegenerative Diseases written by Richard I. Morimoto and published by Springer Science & Business Media. This book was released on 2012-12-13 with total page 145 pages. Available in PDF, EPUB and Kindle. Book excerpt: 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.
Download or read book Protein Quality Control in Neurodegenerative Diseases written by Richard I. Morimoto and published by Springer. This book was released on 2012-12-12 with total page 136 pages. Available in PDF, EPUB and Kindle. Book excerpt: 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.
Download or read book Molecular Chaperones and Neurodegeneration written by Cintia Roodveldt and published by Frontiers Media SA. This book was released on 2017-12-06 with total page 182 pages. Available in PDF, EPUB and Kindle. Book excerpt: 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.
Download or read book The Molecular and Cellular Basis of Neurodegenerative Diseases written by Michael S. Wolfe and published by Academic Press. This book was released on 2018-03-29 with total page 560 pages. Available in PDF, EPUB and Kindle. Book excerpt: 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
Download or read book Fundamentals of Neurodegeneration and Protein Misfolding Disorders written by Martin Beckerman and published by Springer. This book was released on 2015-11-06 with total page 378 pages. Available in PDF, EPUB and Kindle. Book excerpt: 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.
Download or read book Protein Misfolding and Disease written by Peter Bross and published by Springer Science & Business Media. This book was released on 2008-02-02 with total page 317 pages. Available in PDF, EPUB and Kindle. Book excerpt: 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.
Download or read book The Proteasome in Neurodegeneration written by Leonidas Stefanis and published by Springer Science & Business Media. This book was released on 2007-08-02 with total page 314 pages. Available in PDF, EPUB and Kindle. Book excerpt: 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.
Download or read book Intracellular Traffic and Neurodegenerative Disorders written by Peter H. St.George-Hyslop and published by Springer Science & Business Media. This book was released on 2009-02-03 with total page 188 pages. Available in PDF, EPUB and Kindle. Book excerpt: Many adult onset neurodegenerative diseases arise from the accumulation of misfolded peptides. This book examines the role sub-cellular trafficking pathways play in the pathological accumulation of these misfolded proteins and in attempts to clear them.
Download or read book Molecular Biology of Neurodegenerative Diseases written by and published by Academic Press. This book was released on 2012-05-22 with total page 498 pages. Available in PDF, EPUB and Kindle. Book excerpt: 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
Download or read book Protein folding and misfolding neurodegenerative diseases written by Judit Ovádi and published by Springer Science & Business Media. This book was released on 2008-12-21 with total page 284 pages. Available in PDF, EPUB and Kindle. Book excerpt: 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.
Download or read book Neurodegenerative Disorders Loss of Function Through Gain of Function written by K. Beyreuther and published by Springer Science & Business Media. This book was released on 2013-06-29 with total page 216 pages. Available in PDF, EPUB and Kindle. Book excerpt:
Download or read book Molecular Mechanisms of Neurodegenerative Diseases written by Marie-Francoise Chesselet and published by Springer Science & Business Media. This book was released on 2000-10-19 with total page 416 pages. Available in PDF, EPUB and Kindle. Book excerpt: With the unprecedented identification of new mutation mechanisms in neurodegenerative diseases and the emergence of common mechanisms among diseases that were once considered unrelated, neurobiologists are poised for the development of new therapies based on high throughput screenings and a better understanding of the molecular and cellular mechanisms leading to neurodegeneration. In Molecular Mechanisms of Neurodegenerative Diseases, Marie-Francoise Chesselet, MD, PhD, and a panel of leading researchers and neurologists from industry and academia critically review the most recent advances from different yet complementary points of view. Focusing on Alzheimer's, Parkinson's, and CAG triplet repeat diseases, the authors show how studies of cellular and genetically engineered animal models have enhanced our understanding of the molecular mechanisms of neurodegenerative diseases and may lead to the development of new therapeutics. Topics include the role of Ab toxicity, glial cells, and inflammation in Alzheimer's disease; the formation of abnormal protein fragments across several diseases, the impact of dopamine and mitochondrial dysfunction on neurodegeneration; and the potential of genetics to identify the molecular mechanisms of neurodegenerative diseases. Authoritative and insightful, Molecular Mechanisms of Neurodegenerative Diseases synthesizes the novel ideas and concepts now emerging to create a fresh understanding of neurodegenerative disorders, one that promises to lead to powerful new therapies that prevent, delay the onset, slow the progression, or even cure these cruel diseases.
Download or read book Neurodegenerative Diseases written by Jagan A Pillai PhD and published by Oxford University Press. This book was released on 2016-10-17 with total page 304 pages. Available in PDF, EPUB and Kindle. Book excerpt: As the global population ages the impact of neurodegenerative diseases like Alzheimer's disease and Parkinson's disease are significant forces in shaping human health and quality of life in the 21st century. Insights into understanding these diseases, and knowing how to treat them are major frontiers of scientific research. Neurodegenerative Diseases: Unifying Principles is the result of a conceptual revolution over the last decade in our understanding of neurodegenerative diseases as sharing unifying features. There is an increasing appreciation of the common biological and pathological features across seemingly varied neurodegenerative diseases that entail protein misfolding dysfunction and its consequences over time. Providing an overview of this conceptual change is the main theme for the book. Conventional approach emphasize the differences among neurodegenerative disorders, here Drs. Cummings and Pillai compile the increasingly compelling evidence that these disorders share many features and that insights in one may be rapidly translated into advances in another. The goal is to accelerate understanding by showing linkages among biological, pathological, can clinical aspects of this class of diseases. This collection of 19, inter-related chapters, articulates and broadens our view of the unifying features that initiate and drive disease progression across a variety of neurodegenerative diseases over time. This book will serve as an outstanding sourcebook of insights from experts that have played key roles in this story.
Download or read book The Molecular Basis for Neurodegenerative Diseases written by Daniel Offen and published by . This book was released on 2007-01-01 with total page 213 pages. Available in PDF, EPUB and Kindle. Book excerpt: Neurodegenerative disorders are characterized by progressive atrophy and dysfunction of anatomically or physiologically related neurologic systems. There are several common features of neurodegenerative disorders. They are mostly chronic, progressive and start due to the death of specific neuronal populations in the central nerve system (CNS). The distinct clinical phenotypes are generally linked to neuronal loss and often associated with aging. These disorders might share common pathogenetic mechanisms such as intracytoplasmic or intranuclear inclusions in neurons or glia which are linked to the intracellular deposition of abnormal misfolded protein aggregates. The upsurge in longevity is accelerating. The Baby Boomers turned 60 in 2006, and are part of the rapid growth in the aging population in the United States and worldwide. Nearly 36 million people in the United States are aged 65 or older, and life expectancy at birth has reached 77.5 years compared with 3 million in 1900 with life expectancy of 47.3. Most notable is the growth in the population of individuals age 85 and older when neurodegenerative diseases are prevalent. Indeed, these disorders affect increasing populations and it is estimated, for example, that around 4.5 million Alzheimer's patients are living in the United States. In this book, we focus on the major and most common neurodegenerative disorders namely, Alzheimer s (AD), Parkinson s diseases (PD), multiple system atrophy (MSA), Huntington s disease (HD) and amyotrophic lateral sclerosis (ALS). We also included the less common, but clinically and scientifically most interesting occurrance, the prion diseases. In addition to the description of the pathophysiology and the molecular basis of the inherited and sporadic phenomena we tried to illustrate the common basis for this range of clinical conditions. One of the shared histological features of many neurodegenerative diseases is the accumulation of misfolded proteins. The aggregated proteins, such as alpha-synuclein and synphilin-1 in PD, and beta-amyloid (Ab) and tau in AD affect neuronal connectivity and plasticity, and trigger cell death signaling pathways. The inclusions observed in PD are called Lewy bodies and are found mostly in the cytoplasm. AD brains show intracellular neurofibrillary tangles, which contain tau, and extracellular plaques, which contain Ab. Other illnesses with inclusions are Huntington s (polyQ), ALS, and prion diseases. The intra- or extracellular protein aggregates are thought to accumulate in the brain as a result of a decrease in molecular chaperone or proteasome activities which are used as cellular quality control systems. In fact, several mutations that disturb the activity of molecular chaperones or the ubiquitin-proteasome system associated enzymes, can cause neurodegeneration. It has been suggested that either genetic mutations or an increase in nitrosative/oxidative stress can facilitate protein aggregation. A growing body of evidence indicates that neurodegenerative processes in general, are associated with oxidative stress. These data lead to the convincing idea that several neurological disorders may be prevented and/or cured by antioxidant properties. Therefore, one of the chapters review some aspects related to the role of antioxidants in neurodegenerative diseases. The emerging concept of regenerative medicine holds great promise for the numerous patients suffering from various ailments of the nervous system. The last chapter focuses on the adult stem cells residing in the bone marrow being a possible remedy for some of the nervous system diseases. Whether through cell replacement, cell restoration or gene delivery, bone marrow stem cells allow physicians to perform autologous cell transplantation, thus avoiding immune rejection, a major obstacle of cellular therapy.
Download or read book RNA Metabolism in Neurodegenerative Diseases written by Rita Sattler and published by Springer. This book was released on 2018-06-18 with total page 310 pages. Available in PDF, EPUB and Kindle. Book excerpt: It has become evident over the last years that abnormalities in RNA processing play a fundamental part in the pathogenesis of neurodegenerative diseases. Cellular viability depends on proper regulation of RNA metabolism and subsequent protein synthesis, which requires the interplay of many processes including transcription, pre--‐mRNA splicing, mRNA editing as well as mRNA stability, transport and translation. Dysfunction in any of these processes, often caused by mutations in the coding and non--‐ coding RNAs, can be very destructive to the cellular environment and consequently impair neural viability. The result of this RNA toxicity can lead to a toxic gain of function or a loss of function, depending on the nature of the mutation. For example, in repeat expansion disorders, such as the newly discovered hexanucleotide repeat expansion in theC9orf72 gene found in amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD), a toxic gain of function leads to the formation of RNA foci and the sequestration of RNA binding proteins (RBPs). This in return leads to a loss of function of those RBPs, which is hypothesized to play a significant part in the disease progression of ALS and FTD. Other toxicities arising from repeat expansions are the formation of RNA foci, bi--‐directional transcription and production of repeat associated non--‐ATG (RAN) translation products. This book will touch upon most of these disease mechanisms triggered by aberrant RNA metabolism and will therefore provide a broad perspective of the role of RNA processing and its dysfunction in a variety of neurodegenerative disorders, including ALS, FTD, Alzheimer’s disease, Huntington’s disease, spinal muscular atrophy, myotonic dystrophy and ataxias. The proposed authors are leading scientists in the field and are expected to not only discuss their own work, but to be inclusive of historic as well as late breaking discoveries. The compiled chapters will therefore provide a unique collection of novel studies and hypotheses aimed to describe the consequences of altered RNA processing events and its newest molecular players and pathways.
Download or read book Innovative Medicine written by Kazuwa Nakao and published by Springer. This book was released on 2015-10-13 with total page 330 pages. Available in PDF, EPUB and Kindle. Book excerpt: This book is devoted to innovative medicine, comprising the proceedings of the Uehara Memorial Foundation Symposium 2014. It remains extremely rare for the findings of basic research to be developed into clinical applications, and it takes a long time for the process to be achieved. The task of advancing the development of basic research into clinical reality lies with translational science, yet the field seems to struggle to find a way to move forward. To create innovative medical technology, many steps need to be taken: development and analysis of optimal animal models of human diseases, elucidation of genomic and epidemiological data, and establishment of “proof of concept”. There is also considerable demand for progress in drug research, new surgical procedures, and new clinical devices and equipment. While the original research target may be rare diseases, it is also important to apply those findings more broadly to common diseases. The book covers a wide range of topics and is organized into three complementary parts. The first part is basic research for innovative medicine, the second is translational research for innovative medicine, and the third is new technology for innovative medicine. This book helps to understand innovative medicine and to make progress in its realization.
