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Protein Homeostasis Diseases

Protein Homeostasis Diseases
  • Author : Angel L. Pey
  • Publisher :Unknown
  • Release Date :2020-02-13
  • Total pages :450
  • ISBN : 9780128191330
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Summary : 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

Protein Homeostasis
  • Author : Richard I. Morimoto,Dennis J. Selkoe,Jeffery W. Kelly
  • Publisher :Unknown
  • Release Date :2012
  • Total pages :349
  • ISBN : 1936113066
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Summary : 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 Homeostasis

Protein Homeostasis
  • Author : Richard Morimoto
  • Publisher :Unknown
  • Release Date :2019
  • Total pages :350
  • ISBN : 1621822966
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Summary : 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.

Proteostasis and Disease

Proteostasis and Disease
  • Author : Rosa Barrio,James D. Sutherland,Manuel S. Rodriguez
  • Publisher :Unknown
  • Release Date :2020-06-01
  • Total pages :345
  • ISBN : 3030382656
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Summary : 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.

Protein Misfolding and Spreading Pathology in Neurodegenerative Diseases

Protein Misfolding and Spreading Pathology in Neurodegenerative Diseases
  • Author : Diana Fernandes Lázaro,Tiago F. Outeiro,Arianna Bellucci,Patrik Brundin
  • Publisher :Unknown
  • Release Date :2020-02-20
  • Total pages :229
  • ISBN : 9782889635078
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Summary :

The Networking of Chaperones by Co-chaperones

The Networking of Chaperones by Co-chaperones
  • Author : Gregory Lloyd Blatch,Adrienne Lesley Edkins
  • Publisher :Unknown
  • Release Date :2014-12-08
  • Total pages :276
  • ISBN : 9783319117317
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Summary : 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

Ageing: Lessons from C. elegans
  • Author : Anders Olsen,Matthew S. Gill
  • Publisher :Unknown
  • Release Date :2016-12-06
  • Total pages :439
  • ISBN : 9783319447032
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Summary : 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.

Prion

Prion
  • Author : Yusuf Tutar
  • Publisher :Unknown
  • Release Date :2017-03-08
  • Total pages :238
  • ISBN : 9789535130017
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Summary : 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.

The Molecular and Cellular Basis of Neurodegenerative Diseases

The Molecular and Cellular Basis of Neurodegenerative Diseases
  • Author : Michael S. Wolfe
  • Publisher :Unknown
  • Release Date :2018-03-29
  • Total pages :560
  • ISBN : 9780128113059
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Summary : 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

Early Diagnosis of Alzheimer’s Disease

Early Diagnosis of Alzheimer’s Disease
  • Author : Leonard F. M. Scinto,Kirk R. Daffner
  • Publisher :Unknown
  • Release Date :2000-02-09
  • Total pages :359
  • ISBN : 9781592590056
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Summary : Drs. Leonard Scinto and Kirk Daffner provide a comprehensive survey of new diagnostic approaches to Alzheimer's disease. The authoritative contributors critically survey the most promising current research on early diagnostic markers for Alzheimer's disease, including the elucidation of changes in the brain revealed by structural and functional neuroimaging, as well as the characteristic patterns of cognitive decline that are documented by sensitive neuropsychological tests, various genetic markers, and biological assays. Early Diagnosis of Alzheimer's Disease illuminates the complex issues surrounding the search for early markers of this increasingly widespread disease. It will establish a new standard reference guide for all those working with Alzheimer's patients.

Anatomy and Physiology

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 :Unknown
  • Release Date :2013-04-25
  • Total pages :229
  • ISBN : 1947172808
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Summary :

Cellular Endocrinology in Health and Disease

Cellular Endocrinology in Health and Disease
  • Author : Alfredo Ulloa-Aguirre,Ya-Xiong Tao
  • Publisher :Unknown
  • Release Date :2021-02-15
  • Total pages :486
  • ISBN : 9780128198025
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Summary : Cellular Endocrinology in Health and Disease, Second Edition, 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. The new edition provides an understanding of how endocrine glands function by integrating information resulting in biological effects on both local and systemic levels, also providing new information on the molecular physiopathogenesis of endocrine neoplasic cells. The new edition expands the most used chapters from the first edition and proposes a series of substitutions and additions to the table of contents. New chapters cover signaling, brown adipose tissue, hypothalamic cell models, cellular basis of insulin resistance, genetics and epigenetics of neuroendocrine tumors, and a series of chapters on endocrine-related cancer. Providing content that crosses disciplines, Cellular Endocrinology in Health and Disease, Second Edition, 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 Ya-Xiong Tao, experts in molecular and cellular aspects of endocrinology, deliver contributions carefully selected for relevance, impact, and clarity of expression from leading field experts Explores endocrine cells biology in normal and pathologic conditions Covers new aspects of endocrine cell function in distinct tissues Provides a view into the biological effect in local and systemic levels 15 new chapters covering the recent developments in the field

Cell Death

Cell Death
  • Author : Tobias Ntuli
  • Publisher :Unknown
  • Release Date :2015-12-16
  • Total pages :446
  • ISBN : 9789535122364
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Summary : This book is a collection of selected and relevant research, concerning the developments within the Cell Death field of study. Each contribution comes as a separate chapter complete in itself but directly related to the books topics and objectives. The target audience comprises scholars and specialists in the field.

Endoplasmic Reticulum Stress in Health and Disease

Endoplasmic Reticulum Stress in Health and Disease
  • Author : Patrizia Agostinis,Samali Afshin
  • Publisher :Unknown
  • Release Date :2012-08-13
  • Total pages :452
  • ISBN : 9789400743519
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Summary : The Endoplasmic Reticulum (ER) is an organelle with extraordinary signaling and homeostatic functions. It is the organelle responsible for protein folding, maturation, quality control and trafficking of proteins destined for the plasma membrane or for secretion into the extracellular environment. Failure, overloading or malfunctioning of any of the signaling or quality control mechanisms occurring in the ER may provoke a stress condition known as ‘ER stress’. Accumulating evidence indicates that ER stress may dramatically perturb interactions between the cell and its environment, and contribute to the development of human diseases, ranging from metabolic diseases and cancer to neurodegenerative diseases, or impact therapeutic outcome. This book primarily focuses on the pathophysiology of ER stress. It introduces the molecular bases of ER stress, the emerging relevance of the ER-mitochondria cross-talk, the signaling pathways engaged and cellular responses to ER stress, including the adaptive Unfolded Protein Response (UPR), autophagy as well as cell death. Next the book addresses the role of ER stress in physiology and in the etiology of relevant pathological conditions, like carcinogenesis and inflammation, neurodegeneration and metabolic disease. The last chapter describes how ER stress pathways can be targeted for therapeutic benefit. Altogether, this book will provide the reader with an exhaustive view of ER stress biology and the latest insights in the role of ER stress in relevant human diseases.

Textbook of Autoinflammation

Textbook of Autoinflammation
  • Author : Philip J. Hashkes,Ronald M. Laxer,Anna Simon
  • Publisher :Unknown
  • Release Date :2019-02-13
  • Total pages :820
  • ISBN : 9783319986050
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Summary : 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.

The Chaperonopathies

The Chaperonopathies
  • Author : Alberto J.L. Macario,Everly Conway de Macario,Francesco Cappello
  • Publisher :Unknown
  • Release Date :2013-04-10
  • Total pages :116
  • ISBN : 9789400746671
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Summary : 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.

Muscle

Muscle
  • Author : Joseph Hill
  • Publisher :Unknown
  • Release Date :2012
  • Total pages :1398
  • ISBN : 9780123815101
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Summary :

Protein Metabolism and Homeostasis in Aging

Protein Metabolism and Homeostasis in Aging
  • Author : Nektarios Tavernarakis
  • Publisher :Unknown
  • Release Date :2011-01-11
  • Total pages :249
  • ISBN : 9781441970022
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Summary : 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.

Stem Cell Microenvironments and Beyond

Stem Cell Microenvironments and Beyond
  • Author : Alexander Birbrair
  • Publisher :Unknown
  • Release Date :2017-12-03
  • Total pages :270
  • ISBN : 9783319691947
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Summary : This book discusses the main stem cell niches under distinct pathophysiological conditions. The role of tissue microenvironments in stem cell regulation, as well as modern methodologies and new techniques for the identification and characterization of stem cell niches, are discussed by leading experts in the field. Chapters describe the major components of various stem cell microenvironments, such as cellular components, soluble factors, cell-cell interactions, extra-cellular matrix proteins, and physical forces. Stem Cell Microenvironments and Beyond is part of the highly successful Advances in Experimental Medicine and Biology series. It is essential reading for graduate students and researchers in the field of stem cells or cell biology as well as clinicians.

Heat Shock Protein 90 in Human Diseases and Disorders

Heat Shock Protein 90 in Human Diseases and Disorders
  • Author : Alexzander A. A. Asea,Punit Kaur
  • Publisher :Unknown
  • Release Date :2019-11-04
  • Total pages :607
  • ISBN : 9783030231583
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Summary : 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.

Cell Aging: Molecular Mechanisms and Implications for Disease

Cell Aging: Molecular Mechanisms and Implications for Disease
  • Author : Christian Behl,Christine Ziegler
  • Publisher :Unknown
  • Release Date :2013-12-18
  • Total pages :108
  • ISBN : 9783642451799
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Summary : Aging represents a physiological and per se non-pathological and multifactorial process involving a set of key genes and mechanisms being triggered by different endogenous and exogenous factors. Since aging is a major risk factor in connection with a variety of human disorders, it is increasingly becoming a central topic in biochemical and medical research. The plethora of theories on aging – some of which have been discussed for decades – are neither isolated nor contradictory but instead can be connected in a network of pathways and processes at the cellular and molecular levels. This book summarizes the most prominent and important approaches, focusing on telomeres, DNA damage and oxidative stress as well as on the possible role of nutrition, the interplay between genes and environment (epigenetics) and intracellular protein homeostasis and introduces some genes that have actually extended life spans in animal models. Linking these different determinants of aging with disease, this volume aims to reveal their multiple interdependencies. We see that there is no single “perfect” theory of aging and that instead it is possible to define what the authors call the molecular aging matrix of the cell. A better knowledge of its key mechanisms and the mutual connections between its components will lead to a better understanding of age-associated disorders such as Alzheimer’s disease.