protein homeostasis diseases

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Protein Homeostasis Diseases
Author : Angel L. Pey
Publisher : Academic Press
Release Date : 2020-02-13
ISBN 10 : 0128191333
Pages : 450 pages
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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

Protein Homeostasis Diseases
Author : Angel L. Pey
Publisher : Academic Press
Release Date : 2020-02-13
ISBN 10 : 0128191333
Pages : 450 pages
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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

Protein Homeostasis
Author : Richard Morimoto
Publisher : Perspectives Cshl
Release Date : 2019
ISBN 10 : 9781621822967
Pages : 350 pages
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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.

Protein Homeostasis
Author : Richard I. Morimoto,Dennis J. Selkoe,Jeffery W. Kelly
Publisher : N.A
Release Date : 2012
ISBN 10 : 9781936113064
Pages : 349 pages
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Proper expression, folding, transport, and clearance of proteins is critical for cell function. Chaperones and enzymes that posttranslationally assist newly synthesized proteins help ensure that they fold correctly or are degraded. Translocation machineries, proteasomes, and autophagic activities help to localize and degrade proteins as necessary. Stress and aging can cause such mechanisms to become dysfunctional or overloaded, resulting in the accumulation and 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 covers the entire spectrum of protein homeostasis in healthy cells and the diseases that result when control of protein production, protein folding, and protein degradation goes awry. The contributors examine the physical biochemistry of protein folding and the roles of the various cellular compartments in protein quality control, as well as approaches for ameliorating protein misfolding and aggregation diseases. Including discussions of specific disorders such as Alzheimer's disease, Huntington's disease, and prion diseases, this book is an essential reference for not only molecular and cellular biologists but also medical scientists wishing to understand the pathological consequences of and potential therapies for protein homeostasis deficiencies in common human diseases.

Protein Quality Control in Neurodegenerative Diseases
Author : Richard I. Morimoto,Yves Christen
Publisher : Springer Science & Business Media
Release Date : 2012-12-13
ISBN 10 : 3642279287
Pages : 136 pages
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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.

Protein Metabolism and Homeostasis in Aging
Author : Nektarios Tavernarakis
Publisher : Springer Science & Business Media
Release Date : 2011-01-11
ISBN 10 : 1441970029
Pages : 249 pages
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Aging is loosely defined as the accumulation of changes in an organism over time. At the cellular level such changes are distinct and multidimensional: DNA replication ceases, cells stop dividing, they become senescent and eventually die. DNA metabolism and chromosomal maintenance, together with protein metabolism are critical in the aging process. The focus of this book is on the role of protein metabolism and homeostasis in aging. An overview is provided of the current knowledge in the area, including protein synthesis, accuracy and repair, post-translational modifications, degradation and turnover, and how they define and influence aging. The chapters mainly focus on well-characterised factors and pathways, but new areas are also presented, where associations with aging are just being elucidated by current experimental data. Protein turnover, the balance between protein synthesis and protein degradation are carefully maintained in healthy cells. Chapters 1 and 2 illustrate that aging cells are characterised by alterations in the rate, level and accuracy of protein synthesis compared to young ones, and that mRNA translation, essential for cell growth and survival, is controlled at multiple levels. The theory that growth and somatic maintenance are believed to be antagonistic processes is described in Chapter 3: inhibition of protein synthesis results in decreased rates of growth and development, but also confers an extension of lifespan, as shown for example by the effects of dietary restriction in various models organisms.

Nature's Robots
Author : Charles Tanford,Jacqueline Reynolds
Publisher : OUP Oxford
Release Date : 2003-11-27
ISBN 10 : 0191578517
Pages : 312 pages
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Proteins are amazingly versatile molecules. They make the chemical reactions happen that form the basis for life, they transmit signals in the body, they identify and kill foreign invaders, they form the engines that make us move, and they record visual images. All of this is now common knowledge, but it was not so a hundred years ago. Nature's Robots is an authoritative history of protein science, from the origins of protein research in the nineteenth century, when the chemical constitution of 'protein' was first studied and heatedly debated and when there was as yet no glimmer of the functional potential of substances in the 'protein' category, to the determination of the first structures of individual proteins at atomic resolution - when positions of individual atoms were first specified exactly and bonding between neighbouring atoms precisely defined. Tanford and Reynolds, who themselves made major contributions to the golden age of protein science, have written a remarkably vivid account of this history. It is a fascinating story, involving heroes from the past, working mostly alone or in small groups, usually with little support from formal research groups. It is also a story that embraces a number of historically important scientific controversies. Written in clear and accessible prose, Nature's Robots will appeal to general readers with an interest in popular science, in addition to professional scientists and historians of science.

The Networking of Chaperones by Co-chaperones
Author : Gregory Lloyd Blatch,Adrienne Lesley Edkins
Publisher : Springer
Release Date : 2014-12-08
ISBN 10 : 3319117319
Pages : 276 pages
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Co-chaperones are important mediators of the outcome of chaperone assisted protein homeostasis, which is a dynamic balance between the integrated processes of protein folding, degradation and translocation. The Networking of Chaperones by Co-chaperones describes how the function of the major molecular chaperones is regulated by a cohort of diverse non-client proteins, known as co-chaperones. The second edition includes the current status of the field and descriptions of a number of novel co-chaperones that have been recently identified. This new edition has a strong focus on the role of co-chaperones in human disease and as putative drug targets. The book will be a 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.

Ageing: Lessons from C. elegans
Author : Anders Olsen,Matthew S. Gill
Publisher : Springer
Release Date : 2016-12-06
ISBN 10 : 3319447033
Pages : 439 pages
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This book brings together in one volume the current state of ageing research in the nematode Caenorhabditis elegans. The authors are leading researchers in the field, placing this topic in the context of human ageing, describing how and why basic discoveries in this simple organism have impacted our prospects for intervention in the ageing process. The authors cover a broad range of topics with regards to organismal and reproductive ageing including anatomical, physiological and biochemical changes, as well as genetic and environmental interventions that promote longevity and ameliorate age-related disease. Ageing is the single most important factor determining the onset of human disease in developed countries. With current worldwide demographic trends indicating that the number of individuals over the age of 65 will continue to rise, it is clear that an understanding of the processes that underpin ageing and age-related disease represents a key challenge in the biomedical sciences. In recent years there have been huge advances in our understanding of the ageing process and many of these have stemmed from genetic analysis of C. elegans. With no analogous book in this subject area this work will be of interest to a wide audience, ranging from academic researchers to the general public.

Neuronal Inputs and Outputs of Aging and Longevity
Author : Joy Alcedo,Thomas Flatt,Elena G. Pasyukova
Publisher : Frontiers
Release Date : 2013-08-23
ISBN 10 : 2889191605
Pages : 329 pages
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An animal’s survival strongly depends on its ability to maintain homeostasis in response to the changing quality of its external and internal environments. This is achieved through intercellular communication not only within a single tissue but also among different tissues and organ systems. Thus, alterations in tissue-to-tissue or organ-to-organ communications, which are under genetic regulation, can affect organismal homeostasis, and consequently impact the aging process. One of the organ systems that play a major role in maintaining homeostasis is the nervous system. Considering that the nervous system includes the sensory system, which perceives the complexity of an animal’s environment, it should be no surprise that there would be a sensory influence on homeostasis and aging. To promote homeostasis, any given sensory information is transmitted through short-range signals via neural circuits and/or through long-range endocrine signals to target tissues, which may in turn be neuronal or non-neuronal in nature. At the same time, since homeostasis involves a number of feedback mechanisms, non-neuronal tissues can also modulate sensory and other neuronal functions. Several genes that regulate signaling pathways known to affect homeostasis and aging have been shown to act in neurons, in tissues that are likely downstream targets of the nervous system, or through feedback regulation of neuronal activities. These genes can have different temporal requirements: some might function early, e.g., by affecting neural development, while others may only be required later in adulthood. Some well-known examples of genes involved in the neuronal regulation of homeostasis and longevity encode components of the evolutionarily conserved nutrient-sensing insulin/insulin-like signaling pathway, the stress-sensing internal repair system, and the mitochondrial electron transport chain. Indeed, the genetic perturbation of these pathways has been found to lead to numerous diseases, many of which are age-related and involve the nervous system, such as neurodegeneration and the metabolic syndrome. Despite much progress, however, many aspects of the neuronal inputs and outputs that affect aging and longevity are poorly understood to date. For example, the precise neuronal and non-neuronal circuitries and the details of the molecular mechanisms through which genes/signaling pathways maintain homeostasis and affect aging in response to the environment remain to be elucidated. Similarly, it is presently unclear whether genes that regulate the early development of the nervous system and its consequent circuitry influence homeostasis and longevity during adulthood. At the same time, although many genes affecting aging are conserved, both the nervous system and the aging process are highly variable within populations and among taxa. Accordingly, the role of natural genetic variation in shaping the neurobiology of aging is also presently unknown. The aim of this Research Topic is therefore to highlight the genetic, developmental, and physiological aspects of the signaling networks that mediate the neuronal inputs and outputs that are required to maintain organismal homeostasis. The elucidation of the effects of these neuronal activities on homeostasis may thus provide much-needed insight into mechanisms that affect aging and longevity.

Heat Shock Protein 90 in Human Diseases and Disorders
Author : Alexzander A. A. Asea,Punit Kaur
Publisher : Springer Nature
Release Date : 2019-11-04
ISBN 10 : 3030231585
Pages : 607 pages
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The book Heat Shock Protein 90 in Human Diseases and Disorders provides the most comprehensive review on contemporary knowledge on the role of HSP90. Using an integrative approach, the contributors provide a synopsis of novel mechanisms, previously unknown signal transduction pathways. To enhance the ease of reading and comprehension, this book has been subdivided into various section including; Section I, reviews current progress on our understanding Oncogenic Aspects of HSP90; Section II, focuses on Bimolecular Aspects of HSP90; Section III, emphasizes and HSP90 in Natural Products Development and Section IV; give the most up to date reviews on Clinical Aspects of HSP90. Key basic and clinical research laboratories from major universities, academic medical hospitals, biotechnology and pharmaceutical laboratories around the world have contributed chapters that review present research activity and importantly project the field into the future. The book is a must read for starters and professionals in the fields of Translational Medicine, Clinical Research, Human Physiology, Biotechnology, Natural Products, Cell & Molecular Medicine, Pharmaceutical Scientists and Researchers involved in Drug Discovery.

Prion
Author : Yusuf Tutar
Publisher : BoD – Books on Demand
Release Date : 2017-03-08
ISBN 10 : 9535130013
Pages : 238 pages
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Protein aggregation causes malfunction in several biochemical processes. Genetic and spontaneous formations of these transmissible spongiform encephalopathies are fatal to humans and animals. Conformational change of normal form of the protein to misfolded form causes its accumulation. The misfolded infectious protein agent forms the pathogenesis of the disease. This book presents pathology of the disease along with current knowledge of the structure-activity mechanism in the first two sections. Dyshomeostasis of metals is implicated in the pathogenesis of prions, and this influence is discussed further to understand the prion mechanism. Genetic resistance and immunobiology of the disease are elaborated in the following section. Finally, a computational study on the dynamics of the prion propagation provides a structural basis of the mechanism.

Textbook of Autoinflammation
Author : Philip J. Hashkes,Ronald M. Laxer,Anna Simon
Publisher : Springer
Release Date : 2019-02-13
ISBN 10 : 3319986058
Pages : 820 pages
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This book, the first complete textbook on this novel field in Medicine, comprehensively covers the clinical presentation, pathogenesis, genetics, and latest management strategies for autoinflammatory disorders as well as the basic science of autoinflammation. Relevant concepts such as how translational science of genetics and immunology relates to the innate immune system and autoinflammation are covered. Descriptions of the monogenic and polygenic/complex diseases that fall under the umbrella of autoinflammatory diseases are provided. Further topics covered include the latest clinical and genetic diagnostic approaches, concepts on the relationship between autoinflammation and autoimmunity/immunodeficiency, the role of autoinflammation in cancer, treatments and management strategies for these diseases, and potential areas of future development. The Textbook of Autoinflammation systematically describes and reviews diagnostic and treatment options for autoinflammatory disorders as well as all aspects of the concept of autoinflammation, and represents a valuable resource for professionals in a variety of disciplines who encounter these patients or who study autoinflammation.

Protein Chaperones and Protection from Neurodegenerative Diseases
Author : Stephan N. Witt
Publisher : John Wiley & Sons
Release Date : 2011-09-09
ISBN 10 : 1118063899
Pages : 444 pages
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How protein chaperones protect cells from neurodegenerative diseases Including contributions from leading experts, Protein Chaperones and Protection from Neurodegenerative Diseases provides an in-depth exploration of how protein chaperones are involved in shielding cells from toxic aggregated or misfolded protein states that cause ALS, Parkinson's, and related diseases. Examining how different protein chaperones ameliorate the toxicity of proteins that are known to cause neurodegenerative damage, the book addresses both research and clinical perspectives on chaperone and anti-chaperone properties. The intersection of molecular chaperones and neurodegeneration is an intensely studied area, partly because of the potential for manipulating the expression of molecular chaperones to thwart the progression of debilitating diseases, and partly because of the ever-aging global population. Discussing the potential to harness the power of protein chaperones, and future directions for research, discovery, and therapeutics, this book is essential reading for scientists working in the fields of biochemistry, molecular medicine, pharmacology and drug discovery, biotechnology and pharmaceutical companies, advanced students, and anyone interested in this cutting-edge topic.

The Protein Alpha-Synuclein: Its Normal Role (in Neurons) and its Role in Disease
Author : Fredric P. Manfredsson,Ruth G. Perez,Ivette Martinez Sandoval
Publisher : Frontiers Media SA
Release Date : 2020-03-25
ISBN 10 : 2889636100
Pages : 329 pages
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Anatomy and Physiology
Author : J. Gordon Betts,Peter DeSaix,Jody E. Johnson,Oksana Korol,Dean H. Kruse,Brandon Poe,James A. Wise,Mark Womble,Kelly A. Young
Publisher : N.A
Release Date : 2013-04-25
ISBN 10 : 9781947172807
Pages : 329 pages
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Proteostasis and Disease
Author : Rosa Barrio,James D. Sutherland,Manuel S. Rodriguez
Publisher : Springer Nature
Release Date : 2020-04-09
ISBN 10 : 3030382664
Pages : 348 pages
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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.

Cellular Endocrinology in Health and Disease
Author : Alfredo Ulloa-Aguirre,P. Michael Conn
Publisher : Elsevier
Release Date : 2014-02-12
ISBN 10 : 0124167128
Pages : 414 pages
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Cellular Endocrinology in Health and Disease describes the underlying basis of endocrine function, providing an important tool to understand the fundamentals of endocrine diseases. Delivering a comprehensive review of the basic science of endocrinology, from cell biology to human disease, this work explores and dissects the function of a number of cellular systems. Among these are those whose function was not obvious until recently, including the endocrine functions of bone and the adipose tissue. Providing content that crosses disciplines, Cellular Endocrinology in Health and Disease details how cellular endocrine function contributes to system physiology and mediates endocrine disorders. A methods section proves novel and useful approaches across research focus that will be attractive to medical students, residents, and specialists in the field of endocrinology, as well as to those interested in cellular regulation. Editors Alfredo Ulloa-Aguirre and P. Michael Conn, experts in molecular and cellular aspects of endocrinology, deliver contributions carefully selected for relevance, impact, and clarity of expression from leading field experts. Covers systemic endocrine action at the cellular level in both health and disease Delivers information on the integration of cell identity and endocrinology Incorporates recent developments in endocrinology to provide an up-to-date reference to researchers

Muscle
Author : Joseph Hill
Publisher : Academic Press
Release Date : 2012
ISBN 10 : 012381510X
Pages : 1398 pages
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The Neuronal Environment
Author : Wolfgang Walz
Publisher : Springer Science & Business Media
Release Date : 2001-12-26
ISBN 10 : 1592591086
Pages : 432 pages
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Leading neuroscience researchers offer a fresh perspective on neuronal function by examining all its many components-including their pertubation during major disease states-and relate each element to neuronal demands. Topics range from the dependency of neurons on metabolic supply, as well as on both ion and transmitter homeostasis, to their close interaction with the myelin sheath. Also addressed are the astrocytic signaling system that controls synaptic transmission, the extracellular matrix and space as communication systems, the role of blood flow regulation in neuronal demand and in blood-brain barrier function, and inflammation and the neuroimmune system. Insightful and integrative, The Neuronal Environment: Brain Homeostasis in Health and Disease demonstrates a clear new understanding that neurons do not work in isolation, that they need constant interactions with other brain components to process information, and that they are not the only information processing system in the brain.