Download or read book Investigation of molecular pathogenesis of amyotrophic lateral sclerosis and mouse models of neurodegeneration written by Ruth Pek Sim Chia and published by . This book was released on 2008 with total page 612 pages. Available in PDF, EPUB and Kindle. Book excerpt:

Download or read book Investigation of Molecular Pathogenesis of Amyotropic Lateral Sclerosis and Mouse Models of Neurodegeneration written by and published by . This book was released on 2008 with total page pages. Available in PDF, EPUB and Kindle. Book excerpt:

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 561 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 Cellular and molecular mechanisms of motor neuron death in amyotrophic lateral sclerosis written by Ricardo Tapia and published by Frontiers Media SA. This book was released on 2015-02-11 with total page 191 pages. Available in PDF, EPUB and Kindle. Book excerpt: Amyotrophic lateral sclerosis (ALS), which was described since 1869 by Jean Martin Charcot, is a devastating neurodegenerative disease characterized by the selective and progressive loss of upper and lower motor neurons of the cerebral cortex, brainstem and the spinal cord. The cognitive process is not affected and is not merely the result of aging because may occur at young ages. The only known cause of the disease is associated with genetic mutations, mainly in the gene encoding superoxide dismutase 1 (familial ALS), whereas there is no known cause of the sporadic form of ALS (SALS), which comprises >90% of cases. Both ALS types develop similar histopathological and clinical characteristics, and there is no treatment or prevention of the disease. Because effective treatments for ALS, as for other neurodegenerative diseases, can only result from the knowledge of their cellular and molecular pathophysiological mechanisms, research on such mechanisms is essential. Although progress in neurochemical, physiological and clinical investigations in the last decades has identified several mechanisms that seem to be involved in the cell death process, such as glutamate-mediated excitotoxicity, alterations of inhibitory circuits, inflammatory events, axonal transport deficits, oxidative stress, mitochondrial dysfunction and energy failure, the understanding of the origin and temporal progress of the disease is still incomplete and insufficient. Clearly, there is a need of further experimental models and approaches to discern the importance of such mechanisms and to discover the factors that determine the selective death of motor neurons characteristic of ALS, in contrast to other neurodegenerative diseases such as Parkinson’s and Alzheimer’s disease. Whereas studies in vitro in cell cultures, tissue slices or organotypic preparations can give useful information regarding cellular and molecular mechanisms, the experiments in living animal models obviously reflect more closely the situation in the human disease, provided that the symptoms and their development during time mimics as close as possible those of the human disease. It is necessary to correlate the experimental findings in vitro with those in vivo, as well as those obtained in genetic models with those in non-genetic models, aiming at designing and testing therapeutic strategies based on the results obtained.

Download or read book Update on Amyotrophic Lateral Sclerosis written by Humberto Foyaca Sibat and published by BoD – Books on Demand. This book was released on 2016-09-14 with total page 281 pages. Available in PDF, EPUB and Kindle. Book excerpt: This book contains selected peer-reviewed chapters which cover updated information on ALS written by international researchers. Update on Amyotrophic Lateral Sclerosis is comprised of 13 chapters from some of the world's top central nervous system researchers and neurologists to provide a timely review of the most recent developments in ALS, covering historic aspects, experimental animal models, genetics, pathogenesis, clinical aspects and imagenology among others. Contributors from Belgium, France, Japan, India, Italy, Mexico, Russia, South Africa, and Switzerland have collaborated enthusiastically and efficiently, dedicating their time to create this reader-friendly yet comprehensive work which includes many explanatory figures, tables and photos to enhance legibility and make the book clinically useful. We are looking forward with confidence and pride in the remarkable role that this book will play for a new vision and mission.

Download or read book Pathogenesis of Neurodegenerative Disorders written by Mark P. Mattson and published by Springer Science & Business Media. This book was released on 2001-06-01 with total page 296 pages. Available in PDF, EPUB and Kindle. Book excerpt: As the average life expectancy of many populations throughout the world increases, so to does the incidence of such age-related neurodegenerative disorders as Alzheimer's, Parkinson's, and Huntington's diseases. Rapid advances in our understanding of the molecular genetics and environmental factors that either cause or increase risk for age-related neurodegenerative disor ders have been made in the past decade. The ability to evaluate, at the cellular and molecular level, abnormalities in postmortem brain tissue from patients, when taken together with the development of valuable animal and cell-culture models of neurodegenerative disorders has allowed the identification of sequences of events within neurons that result in their demise in specific neurodegenerative disorders. Though the genetic and environmental factors that pro mote neurodegeneration may differ among disorders, shared biochemical cascades that will ultimately lead to the death of neurons have been identified. These cascades involve oxyradical production, aberrant regulation of cellular ion homeostasis and activation of a stereotyped sequence of events involving mitochondrial dysfunction and activa tion of specific proteases. Pathogenesis of Neurodegenerative Disorders provides a timely compilation of articles that encompasses fundamental mechanisms involved in neurodegenerative disorders. In addition, mechanisms that may prevent age-related neurodegenerative disorders are presented. Each chapter is written by an expert in the particular neurodegenerative disorder or mechanism or neuronal death discussed.

Download or read book Neurodegenerative Diseases written by Philip Beart and published by Springer. This book was released on 2017-07-03 with total page 546 pages. Available in PDF, EPUB and Kindle. Book excerpt: Provides a timely overview of critical advances in molecular and cellular neurobiology, covers key methodologies driving progress, and highlights key future directions for research on neuronal injury and neurodegeneration relevant to neuronal brain pathologies. The editors bring together contributions from internationally recognized workers in the field to provide an up to date account of how and why molecular and cellular neurobiology is such an important area for clinical neuroscience. Understanding the molecular aspects of a number of neurodegenerative conditions such as Parkinson's or Alzheimer's disease for the purpose of improving patient management remains a major challenge of neurobiology be it from the basic or clinical perspective. A strategic evaluation of research contributions and the power of modern methods will help advance knowledge over the next years.

Download or read book Molecular Intervention in Mouse Models of Amyotrophic Lateral Sclerosis and Alzheimer s Disease written by Steven Prescott Bennett and published by . This book was released on 2009 with total page 534 pages. Available in PDF, EPUB and Kindle. Book excerpt: ABSTRACT: Neurodegeneration describes the progressive loss of structure and function of neurons, leading ultimately to cell and organism death. Although the initiating factors of neurodegenerative diseases such as Alzheimer's, Parkinson's, Huntington's, and Amyotrophic Lateral Sclerosis may be different, they share common pathophysiologies. Proteinopathies, as these diseases are now termed, are characterized by atypical deposits of proteins, often due to misfolding. Associated with these deposits are dysfunctional mitochondria, oxidative stress, disrupted axonal transport, inflammation, and apoptotic cell death. If this occurs in motor neurons, as in ALS, ataxia precedes death with little or no change in cognition. On the other hand, if the deposits are found in cortical neurons, as in Alzheimer's disease, the outcome is dementia and motor function remains largely intact. Each disease is selective for particular types of neurons and brain regions. Although research has elucidated much of the molecular biology involved in these diseases, their initiating causes remain largely unknown. Most of our current understanding originated with the identification of gene mutations that cause rare familial forms of these diseases. As a result, numerous strains of transgenic animals have been developed to study neurodegenerative disease phenomena and were central to the studies presented in this body of work. Novel routes of drug and gene delivery are described here as well as characterization of the mouse models studied. In particular, this work demonstrates that the blood brain barrier is disrupted in ALS followed by the formation of autorosettes in ALS mice. In various Alzheimer's disease mouse models, it was demonstrated that the acute phase reactant alpha-1-antichymotrypsin (ACT) not only interacts with amyloid plaques, but also induces tau phosphorylation in vivo; tying together these disease hallmarks. It was also shown that small fragments of amyloid beta (1-11) could disrupt the formation of mature amyloid plaques in these mice. Lastly, it was demonstrated that mature plaques could also be decreased by intracranial delivery of granulocyte-macrophage stimulating factor (GM-CSF). My dissertation research goal was to understand and develop these treatment strategies based on protein disaggregation, neuroprotection, and inflammation, meanwhile developing novel methods for targeted delivery of molecules into the CNS of mice.

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 499 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 Neurodegeneration written by Dennis Dickson and published by John Wiley & Sons. This book was released on 2011-09-09 with total page 497 pages. Available in PDF, EPUB and Kindle. Book excerpt: Most textbooks on neurodegenerative disorders have used a classification scheme based upon either clinical syndromes or anatomical distribution of the pathology. In contrast, this book looks to the future and uses a classification based upon molecular mechanisms, rather than clinical or anatomical boundaries. Major advances in molecular genetics and the application of biochemical and immunocytochemical techniques to neurodegenerative disorders have generated this new approach. Throughout most of the current volume, diseases are clustered according to the proteins that accumulate within cells (e.g. tau, α-synuclein and TDP-43) and in the extracellular compartments (e.g. β-amyloid and prion proteins) or according to a shared pathogenetic mechanism, such as trinucleotide repeats, that are a feature of specific genetic disorders. Chapters throughout the book conform to a standard lay-out for ease of access by the reader and are written by a panel of International Experts Since the first edition of this book, major advances have been made in the discovery of common molecular mechanisms between many neurodegenerative diseases most notably in the frontotemporal lobar degenerations (FTLD) and motor neuron disease or amyotrophic lateral sclerosis. This book will be essential reading for clinicians, neuropathologists and basic neuroscientists who require the firm up-to-date knowledge of mechanisms, diagnostic pathology and genetics of Neurodegenerative diseases that is required for progress in therapy and management.

Download or read book TDP 43 and Neurodegeneration written by Vijay Kumar and published by Academic Press. This book was released on 2021-10-23 with total page 272 pages. Available in PDF, EPUB and Kindle. Book excerpt: Aggregates of the TAR DNA binding protein 43 (TDP-43), are hallmark features of the neurodegenerative diseases Amyotrophic Lateral Sclerosis (ALS) and frontotemporal dementia (FTD), with overlapping clinical, genetic and pathological features. TDP-43 and Neurodegeneration: From Bench to Bedside summarizes new findings in TDP-43 pathobiology and proteinopathies. The book summarizes TDP-43’s structure, function, biology, misfolding, aggregation, pathogenesis and therapeutics. It includes autophagy-mediated therapy, role of stress granule, novel genetic, cell culture-based models, systems biology for precision medicine, development of stem cells and mechanism-based therapies that can target ALS and other related neurodegenerative diseases. This book is written for neuroscientists, neurologists, clinicians, advanced graduate students, drug discovery researchers, as well as cellular and molecular biologists involved in ALS, motor neuron disease (MND) and other neurodegenerative disorders. Reviews TDP-43 structure, folding, function, and pathology Identifies TDP-43 role in ALS, FTP, and other neurodegenerative diseases Presents a systems and precision biology perspective of TDP-43 Discusses therapeutics of TDP-43 proteinopathies Translates bench research to application bedside

Download or read book Spectrums of Amyotrophic Lateral Sclerosis written by Christopher A. Shaw and published by John Wiley & Sons. This book was released on 2021-04-20 with total page 240 pages. Available in PDF, EPUB and Kindle. Book excerpt: SPECTRUMS OF AMYOTROPHIC LATERAL SCLEROSIS Discover state-of-the-art research findings on ALS from leading authors and editors in the field In Spectrums of Amyotrophic Lateral Sclerosis: Heterogeneity, Pathogenesis & Therapeutic Directions, distinguished researchers and editors Dr. Christopher A. Shaw and Jessica R. Morrice deliver a practical and powerful perspective on Amyotrophic Lateral Sclerosis (ALS) as a heterogeneous spectrum of disorders. This increasingly accepted point-of-view allows researchers and medical professionals to develop better targeted interventions and more precise therapies. In the book, readers will find chapters on a wide variety of critical issues facing ALS researchers and healthcare practitioners treating ALS sufferers, including animal models of ALS, neuronal support cells known to have a pivotal role in ALS, and current challenges in ALS clinical trials, among others. The authors describe pathologic features common to all cases of ALS and why animal models, though crucial, should be interpreted with caution. Finally, multiple genetic and environmental etiologies of the disease are discussed. Readers will also benefit from the inclusion of: A thorough introduction to ALS as a spectrum disease and the implications for models, therapeutic development and clinical trial design Explorations of the genetic basis of ALS, prospective sALS etiologies, and the involvement of microbiome in ALS Discussions of ALS-PDC and environmental risk factors, protein aggregation in ALS, defects in RNA metabolism in ALS, and the non-cell autonomous nature of ALS and the involvement of glial cells Examinations of animal models of ALS and perspectives on previously failed ALS therapeutics and current therapeutic strategies Perfect for clinical neurologists, healthcare providers and caretakers, clinicians, and researchers studying motor neuron disease, Spectrums of Amyotrophic Lateral Sclerosis: Heterogeneity, Pathogenesis & Therapeutic Directions is also an indispensable resource for the neurodegenerative research community, neurology residents, and graduate-level neuroscience students.

Download or read book Clinical and Molecular Aspects of Motor Neuron Disease written by Johnathan Cooper-Knock and published by Biota Publishing. This book was released on 2013-09-01 with total page 62 pages. Available in PDF, EPUB and Kindle. Book excerpt: In this e-book, motor neuron disease (MND) shall refer to amyotrophic lateral sclerosis (ALS), the most common neurodegenerative disorder affecting both the upper and lower motor neurons. With the discovery of C9ORF72 expansions in approximately 10% of all MND cases, in certain populations, we stand at the brink of a new era of MND research and hopefully treatment facilitated by the ability to associate a relatively large group of patients with a similar disease mechanism. This review will summarise both current clinical management of MND and our present understanding of the molecular pathogenesis of MND. Study of C9ORF72-MND has the potential to rapidly advance both of these aspects in the coming years.

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 Animal Models for Neurodegenerative Disease written by Jesús Avila and published by Royal Society of Chemistry. This book was released on 2011 with total page 307 pages. Available in PDF, EPUB and Kindle. Book excerpt: In recent years, medical developments have resulted in an increase in human life expectancy. The editors have extensive knowledge and experience in this field and the book is aimed at undergraduates, postgraduates, and academics. The chapters cover Alzheimer's disease, Parkinson's disease, Huntington's, and other neurodegenerative disorders.

Download or read book Advances in Research on Neurodegeneration written by Werner Poewe and published by Springer Science & Business Media. This book was released on 1999-04-15 with total page 170 pages. Available in PDF, EPUB and Kindle. Book excerpt: The book contains original articles and reviews presented at the 6th International Winter Conference on Neurodegeneration, held from November 20a "23, 1997 in KitzbA1/4hel/Austria. The aim of this conference was to present and discuss recent data on the interface between neuroimmunology and neurodegeneration, in particular susceptibility to autoimmune and neurodegenerative processes, neuroprotection and restorative treatment strategies. These issues were discussed in the light of recent developments in multiple sclerosis, Parkinsona (TM)s disease, amyotrophic lateral sclerosis, Huntingtona (TM)s disease and multisystem atrophy. The articles highlight topics, which are particularly interesting for the clinician and the neuroscientist. Some of the neuroprotective strategies have already been developed or are at present clinically investigated. The neuroprotective role of deep brain stimulation, antiglutamatergic therapy and apomorphine will be verified in clinical studies in the near future. This is also the case for future therapeutic approaches to restoration of the damaged nervous system, such as somatic gene therapy, implantation of genetically modified cell-lines and medically controlled and directed development of embryonic cells. The conference highlighted the role of the glia, which seems to play a key role, both in traditionally as neuroimmunological and neurodegenerative disorders classified diseases, as indicated above. The topics discussed in the book are significant for recent developments in clinical neurology and neuroscience, as shown in the current literature and at international congresses.

Download or read book Omic Studies of Neurodegenerative Disease Part A written by and published by Academic Press. This book was released on 2015-08-25 with total page 214 pages. Available in PDF, EPUB and Kindle. Book excerpt: Omic Studies of Neurodegenerative Disease: Part A is part of a well-established international series on neuroscience that examines major areas of basic and clinical research, along with emerging and promising subfields. The book informs the reader on the current state of the studies used to evaluate the mechanisms, causes, and treatment of neurodegeneration through a combination of literature reviews and examples of current research. Provides the expertise of leading contributors in the field Demonstrates how findings in the fields of genomics, proteomics, and metabolomic studies can combined for further insights Informs the reader on the current state of the studies used to study the mechanisms, causes, and treatment of neurodegeneration through a combination of literature reviews and examples of current research