Proteostasis and Disease

Proteostasis and Disease PDF Author: Rosa Barrio
Publisher: Springer Nature
ISBN: 3030382664
Category : Science
Languages : en
Pages : 350

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Book Description
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.

Proteostasis and Disease

Proteostasis and Disease PDF Author: Rosa Barrio
Publisher: Springer Nature
ISBN: 3030382664
Category : Science
Languages : en
Pages : 350

Get Book

Book Description
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 Homeostasis

Protein Homeostasis PDF Author: Richard I. Morimoto
Publisher:
ISBN: 9781936113064
Category : Biological transport
Languages : en
Pages : 0

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Book Description
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.

Protein Homeostasis, Second Edition

Protein Homeostasis, Second Edition PDF Author: Richard I. Morimoto
Publisher: Perspectives Cshl
ISBN: 9781621822967
Category : Medical
Languages : en
Pages : 562

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Book Description
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.

HSF1 and Molecular Chaperones in Biology and Cancer

HSF1 and Molecular Chaperones in Biology and Cancer PDF Author: Marc Laurence Mendillo
Publisher: Springer Nature
ISBN: 3030402045
Category : Medical
Languages : en
Pages : 185

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Book Description
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.

Proteostasis and Proteolysis

Proteostasis and Proteolysis PDF Author: Niki Chondrogianni
Publisher: CRC Press
ISBN: 1000474585
Category : Science
Languages : en
Pages : 277

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Book Description
Proteostasis integrates biological pathways controlling biogenesis, trafficking, folding, and degradation of proteins. This book focuses on two protein breakdown/degradation processes (proteolysis), which are part of a normally functioning proteostatic system: the ubiquitin-proteasome system and autophagy. Chapter 2 of this book is freely available as a downloadable Open Access PDF at http://www.taylorfrancis.com under a Creative Commons Attribution-Non Commercial-No Derivatives (CC-BY-NC-ND) 4.0 license.

Quality Control of Cellular Protein in Neurodegenerative Disorders

Quality Control of Cellular Protein in Neurodegenerative Disorders PDF Author: Uddin, Md. Sahab
Publisher: IGI Global
ISBN: 1799813185
Category : Medical
Languages : en
Pages : 515

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Book Description
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.

Leucine-Rich Repeat Kinase 2 (LRRK2)

Leucine-Rich Repeat Kinase 2 (LRRK2) PDF Author: Hardy J. Rideout
Publisher: Springer
ISBN: 3319499696
Category : Medical
Languages : en
Pages : 271

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Book Description
This is the first book to assemble the leading researchers in the field of LRRK2 biology and neurology and provide a snapshot of the current state of knowledge, encompassing all major aspects of its function and dysfunction. The contributors are experts in cell biology and physiology, neurobiology, and medicinal chemistry, bringing a multidisciplinary perspective on the gene and its role in disease. The book covers the identification of LRRK2 as a major contributor to the pathogenesis of Parkinson's Disease. It also discusses the current state of the field after a decade of research, putative normal physiological roles of LRRK2, and the various pathways that have been identified in the search for the mechanism(s) of its induction of neurodegeneration.

Metabolic Cardiomyopathy

Metabolic Cardiomyopathy PDF Author: H. Böhles
Publisher: CRC Press
ISBN: 9783887631048
Category : Cardiomyopathy
Languages : en
Pages : 188

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Book Description
During the last years the understanding for the aetiology of cardiomyopathies could be greatly improved. A great deal of information has accumulated in the field of inherited metabolic diseases, which provides a new basis for our understanding of many heart muscle problems and their corresponding clinical disease entities. This book is meant to give the reader a comprehensive overview of the cardiological manifestations of inborn errors of metabolism. Latest information, such as cardiomyopathy in Fabry disease or in patients with CDG-syndrome is included. It should be helpful, not only to cardiologists, paediatricians, internists and general practicioners, but also to all those interested in a better understanding of the metabolic basis of clinical disease entities.

Proteostasis and Chaperone Surveillance

Proteostasis and Chaperone Surveillance PDF Author: Laishram Rajendrakumar Singh
Publisher: Springer
ISBN: 8132224671
Category : Science
Languages : en
Pages : 180

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Book Description
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.

Nature's Robots

Nature's Robots PDF Author: Charles Tanford
Publisher: OUP Oxford
ISBN: 0191578517
Category : Science
Languages : en
Pages : 312

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Book Description
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.