Proteostasis

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.

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.

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.

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.

"Proteins are the key decision-making workforce for practically all cellular signals. The precise equilibrium between protein translation, folding, function and timely degradation, also known as "proteostasis", determine cellular health as well as organismal maintenance and survival. Loss of cellular proteostasis is linked with physiological processes of deterioration such as aging, with conditions characterized by supraphysiological oxidative stress as well as with illness, including age-related diseases, neurodegeneration, inflammation, dystrophies and cancer. This book includes 18 chapters logically organized to allow comprehensive understanding of how maintenance of proteostasis protects cellular and organismal health, and how environmental and metabolic pressure can impair proteostasis and lead to disequilibrium and disease. Each chapter contains up-to-date information on its respective topic while some of them review the interplay of certain proteostatic mechanisms, a newly arising topic. Importantly, most chapters include tangible examples of how failure of proteostasis can underlie aging and disease. We hope that the compilation of the above topics will assist both novice and experienced researchers and students to become more familiar with the subject of proteostasis. In the long run, we hope that this book will inspire its readers and eventually promote new ideas and new research studies"--

Protein Misfolding, Volume 118, covers the wide spectrum of diseases and disorders that are attributed to protein misfolding, including degenerative and neurodegenerative, cardiovascular, renal, glaucoma, cancer, cystic fibrosis, Gaucher's disease, and many others. Specific chapters cover Mass spectrometric approaches for profiling protein folding and stability, Biomembranes, a key player in protein misfolding, how Genetic and environmental factors interact to disrupt proteostasis and trigger protein misfolding diseases, Formation of oligomers and large amorphous aggregates by intrinsically disordered proteins, Protein misfolding in ER stress with applications to cardiovascular and renal disease, and much more. Integrates methods for studying protein misfolding, factors that trigger this process and its role in a wide spectrum of diseases and disorders Contains timely chapters written by well-renowned authorities in their field Provides data that is well supported by a number of high quality illustrations, figures and tables, and targets a very wide audience of specialists, researchers and students