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Protein Homeostasis

Subject Area(s):  Cell BiologyProteins and ProteomicsBiochemistry

Edited by Richard I. Morimoto, Northwestern University; Dennis J. Selkoe, Brigham and Women's Hospital; Jeffrey W. Kelly, Scripps Institute

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© 2011 • 349 pp., illus. (17 4C and 7 B&W images), index
Hardcover • $135
ISBN  978-1-936113-06-4

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  •     Contents    


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.


I. Chemistry and Biology of Proteostasis
Physical Biochemistry of Protein Folding and Misfolding
Michele Vendruscolo, Tuomas P. J. Knowles, and Christopher M. Dobson
Integrating Protein Homeostasis Strategies in Prokaryotes
Axel Mogk, Damon Huber, and Bernd Bukau
Cellular Strategies of Protein Quality Control
Bryan Chen, Marco Retzlaff, Thomas Roos, and Judith Frydman
Integration of Clearance Mechanisms: The Proteasome and Autophagy
Esther Wong and Ana Maria Cuervo
Aging as an Event of Proteostasis Decline
Rebecca C. Taylor and Andrew Dillin
The Stress of Protein Misfolding: From Single Cells to Multicellular Organisms
Tali Gidalevitz, Veena Prahlad, and Richard I. Morimoto
II. Protein Quality Control in Cellular Compartments
Protein Folding in the Cytoplasm and the Heat Shock Response
Martin Vabulas, Swasti Raychaudhuri, Manajit Hayer-Hartl, and F. Ulrich Hartl
Protein folding and quality control in the ER
Kazutaka Araki and Kazuhiro Nagata
Stress and its Functional Link to Mitochondria: Role in Cell Survival and Death
Jyoti D. Malhotra and Randy Kaufman
Quality Control of Mitochondrial Proteostasis
Michael J. Baker, Takashi Tatsuta, and Thomas Langer
III. Disorders of Protein Homeostasis
Hepatic Fibrosis and Carcinogenesis in Alpha-1-Antitrypsin Deficiency: A Prototype For Chronic Tissue Damage in Gain-of-Function Disorders
David H. Perlmutter and Gary A. Silverman
Alzheimer Disease
Dennis J. Selkoe
Huntingtons’ Disease
Steven Finkbeiner
Proteostasis and Movement Disorders: Parkinson’s Disease and Amyotrophic Lateral Sclerosis
Daryl A. Bosco, Matthew J. LaVoie, Gregory A. Petsko, and Dagmar Ringe
David W. Colby and Stanley B. Prusiner
Emergent Properties of Proteostasis in Managing Cystic Fibrosis
William E. Balch, Daniela Roth, and Darren M. Hutt
Protein Misfolding and Retinal Degeneration
Radouil Tzekov, Linda Stein, and Shalesh Kaushal
Chemical and Biological Approaches for Adapting Proteostasis to Ameliorate Protein Misfolding and Aggregation Diseases–Progress and Prognosis
Susan L. Lindquist and Jeffery W. Kelly