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The Proteasome in Neurodegeneration

The Proteasome in Neurodegeneration
Author: Leonidas Stefanis
Publisher: Springer Science & Business Media
Total Pages: 314
Release: 2007-08-02
Genre: Medical
ISBN: 0387285008

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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.


Ubiquitin and the Brain: Roles of Proteolysis in the Normal and Abnormal Nervous System

Ubiquitin and the Brain: Roles of Proteolysis in the Normal and Abnormal Nervous System
Author: Ashok N. Hegde
Publisher: Frontiers Media SA
Total Pages: 243
Release: 2017-10-10
Genre:
ISBN: 2889452859

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Proteolysis by the ubiquitin-proteasome pathway (UPP) in the nervous system has been extensively studied both in the context of normal physiological function as well as abnormal pathological conditions. Although ubiquitin was used as a marker of brain pathology, the normal functions of the UPP were not studied much in the nervous system until the 1990s. The early investigations focused on synaptic plasticity which was followed by studies on the roles of protein degradation in the development of the nervous system. Research on the role of abnormal roles of the UPP follows a parallel trajectory. Since the 2000s, the field has grown to encompass many subareas of research and several model systems. Despite the progress made, many unanswered questions still remain. For example, there are many unknowns about the precise spatial and temporal control of protein degradation in the normal nervous system. With respect to the roles of proteolysis in brain pathology a major challenge is to elucidate the connection between impaired protein degradation and disease progression. In addition, in-depth studies of the roles of ubiquitin-proteasome-mediated proteolysis in neurodegenerative diseases are promising in identifying therapeutic targets. This ebook contains original research papers and insightful reviews that cover several aspects of proteolysis by the UPP and its physiological as well as pathological functions in the nervous system.


Quality Control of Cellular Protein in Neurodegenerative Disorders

Quality Control of Cellular Protein in Neurodegenerative Disorders
Author: Uddin, Md. Sahab
Publisher: IGI Global
Total Pages: 515
Release: 2020-02-14
Genre: Medical
ISBN: 1799813185

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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.


Proteostasis and Disease

Proteostasis and Disease
Author: Rosa Barrio
Publisher: Springer Nature
Total Pages: 350
Release: 2020-04-09
Genre: Science
ISBN: 3030382664

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This book, written by members of the European network PROTEOSTASIS, provides an up-to-date review of the research regarding protein homeostasis in health and disease. With new discoveries contributing to the increasing complexity of this topic, the book offers a detailed overview of the pathways regulating protein homeostasis, including autophagy and the ubiquitin protein family. Following a basic introduction, it explains how defects in protein homeostasis contribute to numerous pathologies, including cancer, neurodegeneration, inflammation and a number of rare diseases. In addition, it discusses, the role of protein homeostasis in cellular development and physiology. Highlighting the latest research in the field of protein homeostasis and its implications for various clinically relevant diseases, the book appeals to researchers and clinicians, while also offering a reference guide for scholars who are new to the field.


The Proteasome — Ubiquitin Protein Degradation Pathway

The Proteasome — Ubiquitin Protein Degradation Pathway
Author: Peter Zwickl
Publisher: Springer Science & Business Media
Total Pages: 222
Release: 2012-12-06
Genre: Science
ISBN: 364259414X

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This volume gives an overview of pro tea some-mediated protein degradation and the regulatory role of the ubiquitin system in cellular proteolysis. The first chapter describes the molecular evolution of the proteasome and its associated activators, i. e. , the 20S core, the base and the lid of the 19S cap, and the 11 S regulator. The ensuing chapter gives an overview of the structure and assembly of the 20S proteasome and the regulation of the archaeal proteasome by PAN. The third contribution summarizes our knowledge on the eukaryotic 26S proteasome and its regulation by the 19S regu lator, followed by a chapter devoted to the llS regulator, which elucidates the structural basis for the 11 S-mediated activation of the 20S proteasome. The fifth chapter reviews in detail the role of the proteasome in the immune response. The subsequent chapter of the natural substrates of the gives a comprehensive description proteasome and their recognition by the enzymes of the ubiqui tination machinery. The penultimate chapter rounds up the in formation on intracellular distribution of proteasomes in yeast and mammalian cells, while the last contribution highlights proteasome inhibitors, tools which proved to be very valuable for dissecting the cellular roles of the proteasome and which might turn out to be of pharmacological importance.


Neuropathology of Neurodegenerative Diseases

Neuropathology of Neurodegenerative Diseases
Author: Gabor G. Kovacs
Publisher: Cambridge University Press
Total Pages: 320
Release: 2017-12-13
Genre: Medical
ISBN: 1316337650

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This practical guide to the diagnosis of neurodegenerative diseases discusses modern molecular techniques, morphological classification, fundamentals of clinical symptomology, diagnostic pitfalls and immunostaining protocols. It is based on the proteinopathy concept of neurodegenerative disease, which has influenced classification and provides new strategies for therapy. Numerous high-quality images, including histopathology photomicrographs and neuroradiology scans, accompany the description of morphologic alterations and interpretation of immunoreactivities. Diagnostic methods and criteria are placed within recent developments in neuropathology, including the now widespread application of immunohistochemistry. To aid daily practice, the guide includes diagnostic algorithms and offers personal insights from experienced experts in the field. Special focus is given to the way brain tissue should be handled during diagnosis. This is a must-have reference for medical specialists and specialist medical trainees in the fields of pathology, neuropathology and neurology working with neuropathologic features of neurodegenerative diseases.


The Axon

The Axon
Author: Stephen G. Waxman
Publisher:
Total Pages: 710
Release: 1995
Genre: Axons
ISBN: 0195082931

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The axon, interposed between the cell body and the synaptic terminals in most neurons, plays a crucial role in connecting neurons and acting as a conduit for the transmission of information between them. This book provides a comprehensive and up-to-date compendium that brings together chapterson the structure, function, and pathophysiology of axons in both the PNS and CNS. Carefully written, well-illustrated with superb illustrations, and generously referenced, the 33 chapters and introduction have been authored by 49 world-renowned authorities. Recent advances in the molecularneurobiology of axons are carefully reviewed, and new areas, such as the molecular biology of ion channels and myelination, the role of calcium in pathophysiology and regeneration, cell adhesion molecules and their roles in axo-glial interactions and axonal guidance, and optical recording methods,are highlighted. This book will provide an essential reference for neuroscientists as well as clinicians such as neurologists, neurosurgeons, and clinical electrophysiologists interested in axons.