Fundamentals of Neurodegeneration and Protein Misfolding Disorders

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Publisher :
ISBN 13 : 9783319221182
Total Pages : pages
Book Rating : 4.2/5 (211 download)

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Book Synopsis Fundamentals of Neurodegeneration and Protein Misfolding Disorders by : Martin Beckerman

Download or read book Fundamentals of Neurodegeneration and Protein Misfolding Disorders written by Martin Beckerman and published by . This book was released on 2015 with total page 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.

Protein Misfolding Disorders

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Publisher : Bentham Science Publishers
ISBN 13 : 1608050130
Total Pages : 156 pages
Book Rating : 4.6/5 (8 download)

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Book Synopsis Protein Misfolding Disorders by : Claudio Hetz

Download or read book Protein Misfolding Disorders written by Claudio Hetz and published by Bentham Science Publishers. This book was released on 2009 with total page 156 pages. Available in PDF, EPUB and Kindle. Book excerpt: 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.

Protein folding and misfolding: neurodegenerative diseases

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Publisher : Springer Science & Business Media
ISBN 13 : 1402094345
Total Pages : 284 pages
Book Rating : 4.4/5 (2 download)

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Book Synopsis Protein folding and misfolding: neurodegenerative diseases by : Judit Ovádi

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.

Protein Misfolding in Neurodegenerative Diseases

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Publisher : CRC Press
ISBN 13 : 9781420007145
Total Pages : 592 pages
Book Rating : 4.0/5 (71 download)

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Book Synopsis Protein Misfolding in Neurodegenerative Diseases by : Robert D. E. Sewell

Download or read book Protein Misfolding in Neurodegenerative Diseases written by Robert D. E. Sewell and published by CRC Press. This book was released on 2007-12-03 with total page 592 pages. Available in PDF, EPUB and Kindle. Book excerpt: 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.

The Molecular and Cellular Basis of Neurodegenerative Diseases

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Author :
Publisher : Academic Press
ISBN 13 : 0128113057
Total Pages : 560 pages
Book Rating : 4.1/5 (281 download)

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Book Synopsis The Molecular and Cellular Basis of Neurodegenerative Diseases by : Michael S. Wolfe

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

Fundamentals of Neurodegeneration and Protein Misfolding Disorders

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Author :
Publisher : Springer
ISBN 13 : 3319221175
Total Pages : 378 pages
Book Rating : 4.3/5 (192 download)

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Book Synopsis Fundamentals of Neurodegeneration and Protein Misfolding Disorders by : Martin Beckerman

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.

Neurodegenerative Diseases

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Publisher : Oxford University Press
ISBN 13 : 0190233567
Total Pages : 361 pages
Book Rating : 4.1/5 (92 download)

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Book Synopsis Neurodegenerative Diseases by : Jeffrey L. Cummings

Download or read book Neurodegenerative Diseases written by Jeffrey L. Cummings and published by Oxford University Press. This book was released on 2016-11-14 with total page 361 pages. Available in PDF, EPUB and Kindle. Book excerpt: 'Neurodegenerative Diseases' 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 this work.

Neurodegeneration

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Publisher : National Academies Press
ISBN 13 : 9780309285674
Total Pages : 0 pages
Book Rating : 4.2/5 (856 download)

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Book Synopsis Neurodegeneration by : Forum on Neuroscience and Nervous System Disorders

Download or read book Neurodegeneration written by Forum on Neuroscience and Nervous System Disorders and published by National Academies Press. This book was released on 2013-12-26 with total page 0 pages. Available in PDF, EPUB and Kindle. Book excerpt: Neurodegeneration: Exploring Commonalities Across Diseases is the summary of a workshop hosted by the Institute of Medicine\'s (IOM\'s) Forum on Neuroscience and Nervous System Disorders in Spring 2012 to explore commonalities across neurodegenerative diseases such as Alzheimer's disease, Parkinson's disease, amyotrophic lateral sclerosis (ALS), and frontotemporal dementia (FTD). Participants from academia; pharmaceutical and biotechnology industries; government agencies such as the National Institutes of Health and the U.S. Department of Veterans Affairs (VA); patient advocacy groups; and private foundations presented and identified potential opportunities for collaboration across the respective research and development communities. This report identifies and discusses commonalities related to genetic and cellular mechanisms, identifies areas of fundamental science needed to facilitate therapeutics development, and explores areas of potential collaboration among the respective research communities. Neurodegenerative diseases, such as Alzheimer\'s disease, Parkinson\'s disease, ALS, and FTD, are becoming increasingly prevalent in the United States due to an aging population. Implications are grave for quality of life and health care costs. Research on neurodegenerative diseases has expanded greatly over the past four decades. Nevertheless, fundamental questions remain about the biology of these diseases, and further insights into the mechanisms of these diseases would help to inform the development of effective means to prevent and to efficiently treat them. Recent findings have revealed certain commonalities in genetic and cellular mechanisms across neurodegenerative diseases. These findings suggest that it might be valuable - at least in some cases - to change the traditional way of studying these diseases by no longer seeing each as an independent entity, but rather as clinical variants of common cellular and molecular biological defects. This approach could help enhance basic scientific understanding of neurodegenerative disease, and could help with the development of biomarkers and new therapeutics.

Molecular Mechanisms of Neurodegenerative Diseases

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Publisher : Springer Science & Business Media
ISBN 13 : 1592590063
Total Pages : 416 pages
Book Rating : 4.5/5 (925 download)

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Book Synopsis Molecular Mechanisms of Neurodegenerative Diseases by : Marie-Francoise Chesselet

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.

Etiology of Parkinson's Disease

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Publisher : CRC Press
ISBN 13 : 9780824788230
Total Pages : 600 pages
Book Rating : 4.7/5 (882 download)

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Book Synopsis Etiology of Parkinson's Disease by : Jonas H. Ellenberg

Download or read book Etiology of Parkinson's Disease written by Jonas H. Ellenberg and published by CRC Press. This book was released on 1995-03-01 with total page 600 pages. Available in PDF, EPUB and Kindle. Book excerpt: This comprehensive reference provides a detailed overview of current concepts regarding the cause of Parkinson's disease-emphasizing the issues involved in the design, implementation, and analysis of epidemiological studies of parkinsonism.

Medicinal Plants for the Management of Neurodegenerative Diseases

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Publisher : CRC Press
ISBN 13 : 1040030319
Total Pages : 586 pages
Book Rating : 4.0/5 (4 download)

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Book Synopsis Medicinal Plants for the Management of Neurodegenerative Diseases by : Jamal Akhtar

Download or read book Medicinal Plants for the Management of Neurodegenerative Diseases written by Jamal Akhtar and published by CRC Press. This book was released on 2024-06-17 with total page 586 pages. Available in PDF, EPUB and Kindle. Book excerpt: Ageing is the main reason for most neurodegenerative diseases, including Alzheimer’s disease (AD) and Parkinson’s disease (PD). About 10% of individuals aged ≥65 years develop AD, which continuously increases with growing age. Medicinal plants have been used for age-related neurodegenerative disorders for a long time. These plants are rich in terms of various phytochemical constituents such as flavonoids, sterols, alkaloids, saponins, and terpenoids. Different scientific studies have been carried out on these medicinal herbs, e.g., anti-inflammatory, antioxidant, and neuro-regenerative activities that may be used in the treatment of AD and other age-related diseases. These medicinal plants, e.g., Curcuma longa, Bacopa monniera, Glycyrrhiza glabra, Commiphora whighitti, and Acorus calamus L., have anti-inflammatory properties that may decrease swelling of the brain tissue in AD. Despite this, there is currently no book available in the market that provides full information on the phytochemical and medicinal uses of the medicinal plants exclusively used for the management of neurodegenerative diseases in the elderly. This book aims to provide some important and evidence-based information on the plants used for neurodegenerative diseases in the elderly, especially AD and PD, in a planned manner so the academicians, researchers, and students working in the fields of medicinal plants, plant science, chemistry, biotechnology, pharmacognosy, pharmaceuticals, biochemistry, and many other interdisciplinary subjects may benefit. This book will also be useful in identifying a few medicinal plants and their prospects for the synthesis or preparation of new drugs for neurodegenerative diseases. Features: · An overview of the medicinal plants that can be helpful for neurodegenerative disorders. · Recent research results and some pointers for the management of neurodegenerative diseases. This book serve as comprehensive information about the description, phytochemistry, actions, and medicinal use of plants that are used in neurodegenerative diseases. In addition, this book will also ease the understanding and interaction of medicinal plants used in neurodegenerative diseases.

The Prion Protein

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Publisher :
ISBN 13 : 9780954333522
Total Pages : 80 pages
Book Rating : 4.3/5 (335 download)

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Book Synopsis The Prion Protein by : Jorg Tatzelt

Download or read book The Prion Protein written by Jorg Tatzelt and published by . This book was released on 2010 with total page 80 pages. Available in PDF, EPUB and Kindle. Book excerpt: 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.

Protein Misfolding Diseases

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Publisher : John Wiley & Sons
ISBN 13 : 1118031814
Total Pages : 1311 pages
Book Rating : 4.1/5 (18 download)

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Book Synopsis Protein Misfolding Diseases by : Marina Ramirez-Alvarado

Download or read book Protein Misfolding Diseases written by Marina Ramirez-Alvarado and published by John Wiley & Sons. This book was released on 2010-12-01 with total page 1311 pages. Available in PDF, EPUB and Kindle. Book excerpt: An increasingly aging population will add to the number of individuals suffering from amyloid. Protein Misfolding Diseases provides a systematic overview of the current and emerging therapies for these types of protein misfolding diseases, including Alzheimer's, Parkinson's, and Mad Cow. The book emphasizes therapeutics in an amyloid disease context to help students, faculty, scientific researchers, and doctors working with protein misfolding diseases bridge the gap between basic science and pharmaceutical applications to protein misfolding disease.

Biological Soft Matter

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Publisher : John Wiley & Sons
ISBN 13 : 3527810994
Total Pages : 288 pages
Book Rating : 4.5/5 (278 download)

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Book Synopsis Biological Soft Matter by : Corinne Nardin

Download or read book Biological Soft Matter written by Corinne Nardin and published by John Wiley & Sons. This book was released on 2021-04-06 with total page 288 pages. Available in PDF, EPUB and Kindle. Book excerpt: Biological Soft Matter Explore a comprehensive, one-stop reference on biological soft matter written and edited by leading voices in the field Biological Soft Matter: Fundamentals, Properties and Applications delivers a unique and indispensable compilation of up-to-date knowledge and material on biological soft matter. The book presents a thorough overview about biological soft matter, beginning with different substance classes, including proteins, nucleic acids, lipids, and polysaccharides. It goes on to describe a variety of superstructures and aggregated and how they are formed by self-assembly processes like protein folding or crystallization. The distinguished editors have included materials with a special emphasis on macromolecular assembly, including how it applies to lipid membranes, and proteins fibrillization. Biological Soft Matter is a crucial resource for anyone working in the field, compiling information about all important substance classes and their respective roles in forming superstructures. The book is ideal for beginners and experts alike and makes the perfect guide for chemists, physicists, and life scientists with an interest in the area. Readers will also benefit from the inclusion of: An introduction to DNA nano-engineering and DNA-driven nanoparticle assembly Explorations of polysaccharides and glycoproteins, engineered biopolymers, and engineered hydrogels Discussions of macromolecular assemblies, including liquid membranes and small molecule inhibitors for amyloid aggregation A treatment of inorganic nanomaterials as promoters and inhibitors of amyloid fibril formation An examination of a wide variety of natural and artificial polymers Perfect for materials scientists, biochemists, polymer chemists, and protein chemists, Biological Soft Matter: Fundamentals, Properties and Applications will also earn a place in the libraries of biophysicists and physical chemists seeking a one-stop reference summarizing the rapidly evolving topic of biological soft matter.

Cyclin Dependent Kinase 5 (Cdk5)

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Publisher : Springer Science & Business Media
ISBN 13 : 0387788875
Total Pages : 326 pages
Book Rating : 4.3/5 (877 download)

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Book Synopsis Cyclin Dependent Kinase 5 (Cdk5) by : Nancy Y. Ip

Download or read book Cyclin Dependent Kinase 5 (Cdk5) written by Nancy Y. Ip and published by Springer Science & Business Media. This book was released on 2009-02-28 with total page 326 pages. Available in PDF, EPUB and Kindle. Book excerpt: Cyclin Dependent Kinase 5 provides a comprehensive and up-to-date collection of reviews on the discovery, signaling mechanisms and functions of Cdk5, as well as the potential implication of Cdk5 in the treatment of neurodegenerative diseases. Since the identification of this unique member of the Cdk family, Cdk5 has emerged as one of the most important signal transduction mediators in the development, maintenance and fine-tuning of neuronal functions and networking. Further studies have revealed that Cdk5 is also associated with the regulation of neuronal survival during both developmental stages and in neurodegenerative diseases. These observations indicate that precise control of Cdk5 is essential for the regulation of neuronal survival. The pivotal role Cdk5 appears to play in both the regulation of neuronal survival and synaptic functions thus raises the interesting possibility that Cdk5 inhibitors may serve as therapeutic treatment for a number of neurodegenerative diseases.

Basic Neurochemistry

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Publisher : Academic Press
ISBN 13 : 0080959016
Total Pages : 1120 pages
Book Rating : 4.0/5 (89 download)

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Book Synopsis Basic Neurochemistry by : Scott Brady

Download or read book Basic Neurochemistry written by Scott Brady and published by Academic Press. This book was released on 2011-11-02 with total page 1120 pages. Available in PDF, EPUB and Kindle. Book excerpt: Basic Neurochemistry: Principles of Molecular, Cellular, and Medical Neurobiology, the outstanding and comprehensive classic text on neurochemistry, is now newly updated and revised in its Eighth Edition. For more than forty years, this text has been the worldwide standard for information on the biochemistry of the nervous system, serving as a resource for postgraduate trainees and teachers in neurology, psychiatry, and basic neuroscience, as well as for medical, graduate, and postgraduate students and instructors in the neurosciences. The text has evolved, as intended, with the science. It is also an excellent source of current information on basic biochemical and cellular processes in brain function and neurological diseases for continuing medical education and qualifying examinations. This text continues to be the standard reference and textbook for exploring the translational nature of neuroscience, bringing basic and clinical neuroscience together in one authoritative volume. Our book title reflects the expanded attention to these links between neurochemistry and neurologic disease. This new edition continues to cover the basics of neurochemistry as in the earlier editions, along with expanded and additional coverage of new research from: Intracellular trafficking; Stem cells, adult neurogenesis, regeneration; Lipid messengers; Expanded coverage of all major neurodegenerative and psychiatric disorders; Neurochemistry of addiction; Neurochemistry of pain; Neurochemistry of hearing and balance; Neurobiology of learning and memory; Sleep; Myelin structure, development, and disease; Autism; and Neuroimmunology. Completely updated text with new authors and material, and many entirely new chapters Over 400 fully revised figures in splendid color 61 chapters covering the range of cellular, molecular and medical neuroscience Translational science boxes emphasizing the connections between basic and clinical neuroscience Companion website at http://elsevierdirect.com/companions/9780123749475

RNA Metabolism in Neurodegenerative Diseases

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Author :
Publisher : Springer
ISBN 13 : 331989689X
Total Pages : 310 pages
Book Rating : 4.3/5 (198 download)

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Book Synopsis RNA Metabolism in Neurodegenerative Diseases by : Rita Sattler

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.