Chaperones, Protein Homeostasis and Protein Aggregation Diseases

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Publisher :
ISBN 13 : 9789036771900
Total Pages : pages
Book Rating : 4.7/5 (719 download)

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Book Synopsis Chaperones, Protein Homeostasis and Protein Aggregation Diseases by : Harm Harmannus Kampinga

Download or read book Chaperones, Protein Homeostasis and Protein Aggregation Diseases written by Harm Harmannus Kampinga and published by . This book was released on 2014 with total page pages. Available in PDF, EPUB and Kindle. Book excerpt:

Protein Homeostasis Diseases

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Publisher : Academic Press
ISBN 13 : 0128191333
Total Pages : 450 pages
Book Rating : 4.1/5 (281 download)

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Book Synopsis Protein Homeostasis Diseases by : Angel L. Pey

Download or read book Protein Homeostasis Diseases written by Angel L. Pey and published by Academic Press. This book was released on 2020-02-13 with total page 450 pages. Available in PDF, EPUB and Kindle. Book excerpt: Protein Homeostasis Diseases: Mechanisms and Novel Therapies offers an interdisciplinary examination of the fundamental aspects, biochemistry and molecular biology of protein homeostasis disease, including the use of natural and pharmacological small molecules to treat common and rare protein homeostasis disorders. Contributions from international experts discuss the biochemical and genetic components of protein homeostasis disorders, the mechanisms by which genetic variants may cause loss-of-function and gain-of-toxic-function, and how natural ligands can restore protein function and homeostasis in genetic diseases. Applied chapters provide guidance on employing high throughput sequencing and screening methodologies to develop pharmacological chaperones and repurpose approved drugs to treat protein homeostasis disorders. Provides an interdisciplinary examination of protein homeostasis disorders, with an emphasis on treatment strategies employing small natural and pharmacological ligands Offers applied approaches in employing high throughput sequencing and screening to develop pharmacological chaperones to treat protein homeostasis disease Gathers expertise from a range of international chapter authors who work across various biological methods and disease specific disciplines of relevance

Proteostasis and Chaperone Surveillance

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Publisher : Springer
ISBN 13 : 8132224671
Total Pages : 184 pages
Book Rating : 4.1/5 (322 download)

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Book Synopsis Proteostasis and Chaperone Surveillance by : Laishram Rajendrakumar Singh

Download or read book Proteostasis and Chaperone Surveillance written by Laishram Rajendrakumar Singh and published by Springer. This book was released on 2015-11-26 with total page 184 pages. Available in PDF, EPUB and Kindle. Book excerpt: Proteostasis is central to the development of various human diseases caused due to excessive protein misfolding and the disregulation of the protein quality control system. In this book, respected researchers from many leading institutions contribute their insights on proteostasis maintenance. The coverage mainly focuses on the basics of maintaining proteostasis, the consequences of proteostatic system failure, and how chaperone systems constantly maintain proteostasis. In addition, the book presents in detail different treatment strategies for diseases caused by proteostatic system failure, as well as the inhibition of proteostatic failure using small molecule compounds. It examines advances in the modulation of proteopathies, providing a comprehensive source of key mechanistic insights on these diseases. As such, the book offers a valuable resource for beginners and more experienced investigators alike who are looking for detailed and reliable information on protein homeostasis, the diseases that can develop due to related imbalances and the essential role of molecular and chemical chaperones.

Cellular Osmolytes

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Publisher : Springer
ISBN 13 : 9811037078
Total Pages : 251 pages
Book Rating : 4.8/5 (11 download)

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Book Synopsis Cellular Osmolytes by : Laishram Rajendrakumar Singh

Download or read book Cellular Osmolytes written by Laishram Rajendrakumar Singh and published by Springer. This book was released on 2017-05-16 with total page 251 pages. Available in PDF, EPUB and Kindle. Book excerpt: This book provides essential information on improving protein folding/stability, which is a result of the balance between the intra-molecular interactions of protein functional groups and their interactions with the solvent environment. The protein folding solvent environment mainly consists of salts, small molecule compounds, metabolites, molecular chaperones and other chemical species. Therefore, subtle change in the composition of the environment will alter the protein folding process. The importance of the solvent environment in protein folding is precisely due to the fact that various disease-causing proteopathies can be reversed by manipulating the solvent environment of the malfolded proteins. Hostile environmental stresses represent one of the basic causes of such challenges in protein folding or misfolding. Since cells commonly encounter extreme environmental fluctuations, it is crucial that they equip themselves with strategies to circumvent the hostile environmental conditions. Nature has developed many strategies to ensure that the complex and challenging protein folding reaction occurs with adequate efficiency and fidelity for the success of the organism. Among the strategies employed in a wide range of species and cell types is the elaboration of small organic molecules called osmolytes. Additionally, recent advances have also revealed that certain specific osmolytes might be key biomarkers of cancer, infectious diseases and vaccine flocculation. In fact, a large pool of data has been generated regarding their potential for the therapeutic intervention of neurodegenerative diseases and other metabolic disorders caused by protein aggregation or proteostasis failure. Reflecting the multiple applications of these small molecules in the health and other industries, this book combines contributions by respected leaders in the field and will help to inspire college students, basic researchers, and clinicians to translate these biological roles of osmolytes into clinical practice. It will also shed light on some important future prospects of osmolytes like their role as drug excipients and provide a deeper understanding of their mechanism of action in the prevention of neuro-degenerative diseases.

Protein Homeostasis, Second Edition

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Publisher : Perspectives Cshl
ISBN 13 : 9781621822967
Total Pages : 562 pages
Book Rating : 4.8/5 (229 download)

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Book Synopsis Protein Homeostasis, Second Edition by : Richard I. Morimoto

Download or read book Protein Homeostasis, Second Edition written by Richard I. Morimoto and published by Perspectives Cshl. This book was released on 2019 with total page 562 pages. Available in PDF, EPUB and Kindle. Book excerpt: The entire life cycle of a protein-from synthesis and folding to transport and degradation-is carefully controlled by the proteostasis network. This network, consisting of many interconnected pathways and processes, manages protein homeostasis by dynamically responding to the needs of the cell. Stress and aging can challenge the proteostasis network, resulting in the aggregation of misfolded proteins-a feature of numerous neurodegenerative conditions. Written and edited by experts in the field, this collection from Cold Spring Harbor Perspectives in Biology provides a comprehensive update on how the proteostasis network functions in healthy cells and the diseases that result when protein quality control goes awry. The contributors examine the relevant biochemical attributes of proteins (e.g., solubility), the functions of normal protein aggregates (e.g., biofilm formation in bacteria), and the various heat shock proteins, chaperones, translocation machineries, proteasomes, signaling factors, and transcriptional programs involved in proteostasis. The roles of specific subcellular structures-the endoplasmic reticulum, mitochondria, ribosomes, lysosomes, and cytoplasm-in protein quality control are covered, as is the regulation of proteostasis at the organismal level (e.g., via neuronal activity). Discussions of the responses by cells when errors in protein quality control occur, the medical disorders that can result (e.g., Alzheimer disease), and pharmacologic approaches to ameliorate protein conformational disorders are also included. This book is therefore an essential reference for biochemists, cell biologists, and all biomedical scientists wishing to understand the pathological consequences of and potential therapies for proteostasis deficiencies in common human diseases.

Molecular Chaperones and Neurodegeneration

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Publisher : Frontiers Media SA
ISBN 13 : 2889453421
Total Pages : 182 pages
Book Rating : 4.8/5 (894 download)

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Book Synopsis Molecular Chaperones and Neurodegeneration by : Cintia Roodveldt

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.

Protein Homeostasis

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Publisher :
ISBN 13 : 9781936113064
Total Pages : 0 pages
Book Rating : 4.1/5 (13 download)

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Book Synopsis Protein Homeostasis by : Richard I. Morimoto

Download or read book Protein Homeostasis written by Richard I. Morimoto and published by . This book was released on 2012 with total page 0 pages. Available in PDF, EPUB and Kindle. Book excerpt: 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.

The Networking of Chaperones by Co-Chaperones

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Publisher : Springer Nature
ISBN 13 : 3031147405
Total Pages : 436 pages
Book Rating : 4.0/5 (311 download)

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Book Synopsis The Networking of Chaperones by Co-Chaperones by : Adrienne L. Edkins

Download or read book The Networking of Chaperones by Co-Chaperones written by Adrienne L. Edkins and published by Springer Nature. This book was released on 2022-12-15 with total page 436 pages. Available in PDF, EPUB and Kindle. Book excerpt: Co-chaperones are important mediators of the outcome of chaperone assisted protein homeostasis, which is the dynamic integration of the processes of protein folding, degradation and translocation to ensure that cellular function is finely tuned in space and time. This third edition of the book The Networking of Chaperones by Co-chaperones describes how the function of the major molecular chaperones is regulated by co-chaperones, a diverse cohort of non-client proteins. Since the second edition was released, not only has knowledge deepened on how co-chaperones act as nodes to network and functionalise chaperones, but an understanding of their broader biological function has started to emerge. The third edition provides new and updated chapters highlighting recent developments and emerging themes on co-chaperones, such as their extracellular functions, their role in human disease and their status as putative drug targets. The book is a useful resource for both newcomers and established researchers in the field of cell stress and chaperones, as well as those interested in cross-cutting disciplines such as cellular networks and systems biology.

HSF1 and Molecular Chaperones in Biology and Cancer

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Publisher : Springer Nature
ISBN 13 : 3030402045
Total Pages : 185 pages
Book Rating : 4.0/5 (34 download)

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Book Synopsis HSF1 and Molecular Chaperones in Biology and Cancer by : Marc Laurence Mendillo

Download or read book HSF1 and Molecular Chaperones in Biology and Cancer written by Marc Laurence Mendillo and published by Springer Nature. This book was released on 2020-04-15 with total page 185 pages. Available in PDF, EPUB and Kindle. Book excerpt: Protein homeostasis, or “Proteostasis”, lies at the heart of human health and disease. From the folding of single polypeptide chains into functional proteins, to the regulation of intracellular signaling pathways, to the secreted signals that coordinate cells in tissues and throughout the body, the proteostasis network operates to support cell health and physiological fitness. However, cancer cells also hijack the proteostasis network and many of these same processes to sustain the growth and spread of tumors. The chapters in this book are written by world experts in the many facets of the proteostasis network. They describe cutting-edge insights into the structure and function of the major chaperone and degradation systems in healthy cells and how these systems are co-opted in cancer cells and the cells of the tumor microenvironment. The chapters also cover therapeutic interventions such as the FDA-approved proteasome inhibitors Velcade and Krypolis as well as other therapies currently under clinical investigation to disarm the ability of the proteostasis network to support malignancy. This compendium is the first of its kind and aims to serve as a reference manual for active investigators and a primer for newcomers to the field. This book is dedicated to the memory of Susan Lindquist, a pioneer of the proteostasis field and a champion of the power of basic scientific inquiry to unlock the mechanisms of human disease. The chapter “Reflections and Outlook on Targeting HSP90, HSP70 and HSF1 in Cancer: A Personal Perspective” is available open access under a Creative Commons Attribution 4.0 International License via link.springer.com.

Protein Quality Control in Neurodegenerative Diseases

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Publisher : Springer Science & Business Media
ISBN 13 : 3642279287
Total Pages : 145 pages
Book Rating : 4.6/5 (422 download)

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Book Synopsis Protein Quality Control in Neurodegenerative Diseases by : Richard I. Morimoto

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.

The Chaperonopathies

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Publisher : Springer Science & Business Media
ISBN 13 : 9400746679
Total Pages : 126 pages
Book Rating : 4.4/5 (7 download)

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Book Synopsis The Chaperonopathies by : Alberto J.L. Macario

Download or read book The Chaperonopathies written by Alberto J.L. Macario and published by Springer Science & Business Media. This book was released on 2013-04-10 with total page 126 pages. Available in PDF, EPUB and Kindle. Book excerpt: This Brief provides a concise review of chaperonopathies, i.e., diseases in which molecular chaperones play an etiologic-pathogenic role. Introductory chapters deal with the chaperoning system and chaperoning teams and networks, HSP-chaperone subpopulations, the locations and functions of chaperones, and chaperone genes in humans. Other chapters present the chaperonopathies in general, including their molecular features and mechanistic classification into by defect, excess, or mistake. Subsequent chapters discuss the chaperonopathies in more detail, focusing on their distinctive characteristics: primary or secondary; quantitative and/or qualitative; structural and hereditary or acquired; genetic polymorphisms; gene dysregulation; age-related; associated with cancer, chronic inflammatory conditions, and autoimmune diseases. The interconnections between the chaperoning and the immune systems in cancer development, chronic inflammation, autoimmunity, and ageing are outlined, which leads to a discussion on the future prospects of chaperonotherapy. The latter may consist of chaperone gene and protein replacement/supplementation in cases of deficiency and of gene or protein blocking when the chaperone actively promotes disease. The last chapter presents the extracellular chaperones and details on how the chaperone Hsp60 is secreted into the extracellular space and, thus, appears in the blood of cancer patients with potential to participate in carcinogenesis and chronic inflammation and autoimmunity. Chaperones as clinically useful biomarkers are mentioned when pertinent. Likewise, guidelines for clinical evaluation of chaperonopathies and for their histopathological and molecular identification are provided throughout. The book also provides extensive bibliography organized by chapter and topic with comments.

Characterization of the Interactions Between Molecular Chaperones and the Amyloid Protein Huntingtin Throughout Its Aggregation Pathway

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Publisher :
ISBN 13 :
Total Pages : 0 pages
Book Rating : 4.:/5 (145 download)

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Book Synopsis Characterization of the Interactions Between Molecular Chaperones and the Amyloid Protein Huntingtin Throughout Its Aggregation Pathway by : Merve Özel

Download or read book Characterization of the Interactions Between Molecular Chaperones and the Amyloid Protein Huntingtin Throughout Its Aggregation Pathway written by Merve Özel and published by . This book was released on 2024 with total page 0 pages. Available in PDF, EPUB and Kindle. Book excerpt: Protein misfolding and subsequent aggregation characterize most neurodegenerative diseases and are associated with impaired protein homeostasis (proteostasis). As prominent components of the proteostasis network, molecular chaperones have been identified as modulators of protein aggregation. Understanding their mechanisms of action paves the way for their use as a therapeutic strategy against neurodegenerative diseases. Huntington's disease (HD) is an inherited neurodegenerative disease caused by the abnormal expansion of CAG repeats in the first exon of the Huntingtin protein. Aggregation of the pathogenic HTTExon1 is suggested as the primary cause of neuronal deterioration. Previously, it was shown that a trimeric chaperone complex composed of Hsc70, DNAJB1, and Apg2 can inhibit pathogenic HTTExon1 aggregation and disaggregate preformed HTTExon1 fibrils. As a member of the J-domain protein (JDP) family, and a co-chaperone of Hsc70, DNAJB1 is known to recognize protein substrates, to transfer them to Hsc70, and stimulate the ATPase activity of Hsc70. Recently, a binding interface between DNAJB1 and HTTExon1 has been identified and this work has contributed to the analysis of the effect of this binding site in HTTExon1 suppression and disaggregation activities by the trimeric chaperone complex. In vitro analyses showed that this binding site and unique features of DNAJB1 conferred specificity and functionality, distinguishing it from other JDPs. Furthermore, association between the trimeric chaperones and HTTExon1 along its aggregation pathway was investigated. The data revealed that DNAJB1 and Hsc70 associate with aggregating HTTExon1 only in the presence of each other and an intact ATPase cycle, suggesting a cooperative association of these chaperones with HTTExon1 during aggregation. This association was found to be stable to detergent, suggesting that irreversible sequestration of chaperones in HTTExon1 aggregates is associated with the eventual failure of the trimeric chaperone complex in suppressing HTTExon1 aggregation.

Functions and Mechanisms of Bacterial Protein Homeostasis and Stress Responses

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Publisher : Frontiers Media SA
ISBN 13 : 2889741931
Total Pages : 334 pages
Book Rating : 4.8/5 (897 download)

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Book Synopsis Functions and Mechanisms of Bacterial Protein Homeostasis and Stress Responses by : Axel Mogk

Download or read book Functions and Mechanisms of Bacterial Protein Homeostasis and Stress Responses written by Axel Mogk and published by Frontiers Media SA. This book was released on 2022-02-01 with total page 334 pages. Available in PDF, EPUB and Kindle. Book excerpt: The Cover Image for This Research Topic is Used With Permission of the Authors and Publishers of the Following Article: Winkler J, Seybert A, König L, Pruggnaller S, Haselmann U, Sourjik V, Weiss M, Frangakis AS, Mogk A, Bukau B.EMBO J. 2010 Mar 3;29(5):910-23. doi: 10.1038/emboj.2009.412. Epub 2010 Jan 21

The Big Book on Small Heat Shock Proteins

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Publisher : Springer
ISBN 13 : 331916077X
Total Pages : 603 pages
Book Rating : 4.3/5 (191 download)

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Book Synopsis The Big Book on Small Heat Shock Proteins by : Robert M. Tanguay

Download or read book The Big Book on Small Heat Shock Proteins written by Robert M. Tanguay and published by Springer. This book was released on 2015-06-15 with total page 603 pages. Available in PDF, EPUB and Kindle. Book excerpt: Based upon a workshop entitled “The Small HSP World” held in Québec 2-5 October 2014. Twenty-five scientists provided chapters for the book. The chapters are from the best scientists currently working in this field. These colleagues include Arrigo, Benesch, Benjamin, Buchner-Haslbeck-Weinkauf, Benndorf, Boelens, Carra, Chang, Currie, Ecroyd, Emanuelsson, Fu, Garrido, Golenhofen, Gusev, Hightower, Kampinga, Lavoie, MacRae, Quinlan, Tanguay, Vierling, Vigh, Weeks and Wu. Briefly, the book starts with the structure of small heat shock proteins, moving to their functions and finishing with their involvement in diseases. Although this is quite broad, the structural aspect will be the unifying theme of the book.

Protein Homeostasis in Growth, Development and Disease

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Publisher : Frontiers Media SA
ISBN 13 : 2832517064
Total Pages : 136 pages
Book Rating : 4.8/5 (325 download)

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Book Synopsis Protein Homeostasis in Growth, Development and Disease by : Francesco Fazi

Download or read book Protein Homeostasis in Growth, Development and Disease written by Francesco Fazi and published by Frontiers Media SA. This book was released on 2023-03-08 with total page 136 pages. Available in PDF, EPUB and Kindle. Book excerpt:

Quality Control of Cellular Protein in Neurodegenerative Disorders

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Publisher : IGI Global
ISBN 13 : 1799813185
Total Pages : 515 pages
Book Rating : 4.7/5 (998 download)

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Book Synopsis Quality Control of Cellular Protein in Neurodegenerative Disorders by : Uddin, Md. Sahab

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.

Spectrums of Amyotrophic Lateral Sclerosis

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Publisher : John Wiley & Sons
ISBN 13 : 1119745500
Total Pages : 240 pages
Book Rating : 4.1/5 (197 download)

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Book Synopsis Spectrums of Amyotrophic Lateral Sclerosis by : Christopher A. Shaw

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.