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Protein Homeostasis, Second Edition

Book Series:  A Cold Spring Harbor Perspectives in Biology Collection
Subject Area(s):  Proteins and ProteomicsHuman Biology and Disease

Edited by Jeffery W. Kelly, The Scripps Research Institute, Franz-Ulrich Hartl, Max Planck Institute of Biochemistry; Richard I. Morimoto, Northwestern University

Due June 2019 • 300 pages (approx.), illustrated, index
Hardcover • $135 94.50
ISBN  978-1-621822-96-7
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  •     Description    
  •     Contents    


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.


Predictions of Aggregation Propensities of Proteins
Michele Vendruscolo and Pietro Sormanni
Structural Insights in Amyloids and Prion Diseases
David Eisenberg
Functional Amyloids
Daniel Otzen and Roland Riek
Amyloid Phenomenon and Significance for Human Disease
Chris Dobson
The Unfolded Protein Response: Detecting and Responding to Fluctuations in the Protein Folding Capacity of the Endoplasmic Reticulum
G. Elif Karagöz, Diego Acosta-Alvear, and Peter Walter
Early Events in the Endoplasmic Reticulum Unfolded Protein Response (UPS)
Steffen Preissler and David Ron
Spatial Quality Control
Ramanujan Hegde
Redox-Mediated Regulatory Mechanisms of Endoplasmic Reticulum (ER) Homeostasis
Ryo Ushioda and Kazuhiro Nagata
Chaperoning Endoplasmic Reticulum-Associated Degradation (ERAD) and Protein Conformational Diseases
Patrick G. Needham, Christopher J. Guerriero, and Jeffrey L. Brodsky
Mitochondrial Proteolysis and Metabolic Control
Sofia Ahola, Thomas Langer, and Thomas MacVicar
Signaling and Regulation of the Mitochondrial Unfolded Protein Response
Nandhitha Uma Naresh and Cole M. Haynes
Protein Folding and Misfolding in the Cytoplasm
F. Ulrich Hartl and Mark Hipp
Modulation of Amyloid States by Molecular Chaperones
Anne Wentink, Carmen Nussbaum-Krammer, and Bernd Bukau
Chaperone Interactions at the Ribosome
Elke Deuerling, Martin Gamerdinger, and Stefan Kreft
Ubiquitin Proteasome and the Remodeling of Life
Daniel Finley
Autophagy lysosomal pathways & neurodegeneration
Steve Finkbeiner
The Nuclear and DNA-Associated Molecular Chaperone Network
Zlata Gvozdenov, Janhavi Kolhe, and Brian C. Freeman
Structure, Function, and Regulation of the Hsp90 Machinery
Maximilian M. Biebl and Johannes Buchner
Mechanisms of Small Heat Shock Proteins
Maria K. Janowska, Hannah E.R. Baughman, Christopher N. Woods, and Rachel E. Klevit
Spiraling in Control: Structures and Mechanisms of the Hsp104 Disaggregase
James Shorter and Daniel R. Southworth
Role of Polyphosphate in Amyloidogenic Processes
Justine Lempart and Ursula Jakob
Protein Phase Separation as a Stress Survival Strategy
Titus M. Franzmann and Simon Alberti
Tailoring of Proteostasis Networks with Heat Shock Factors
Jenny Joutsen and Lea Sistonen
Cell Non-Autonomous Regulation of Proteostasis in Aging and Disease
Richard I. Morimoto
Viral Infections as a Proteostasis Disease
Judith Frydman
Restoring Cellular Function by Proteostasis Regulators
Jeffery W. Kelly
Hsp90 Inhibitors and Cancer
Gabriela Chiosis
Proteome-scale Mapping of Perturbed Proteostasis in Living Cells
Vikram Khurana and Joan H. Swan