Protein Homeostasis

by Morimoto, Richard; Selkoe, Dennis J.; Kelly, Jeffrey W.

• ISBN13: 9781936113064
• ISBN10: 1936113066
• Format: Hardcover
• Copyright: 2011-09-29
• Publisher: Cold Spring Harbor Laboratory Press

Summary

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.

Table of Contents

Preface	p. vii
Chemistry and Biology of Proteostasis
Protein Solubility and Protein Homeostasis: A Generic View of Protein Misfolding Disorders	p. 1
Integrating Protein Homeostasis Strategies in Prokaryotes	p. 13
Cellular Strategies of Protein Quality Control	p. 33
Integration of Clearance Mechanisms: The Proteasome and Autophagy	p. 47
Aging as an Event of Proteostasis Collapse	p. 67
The Stress of Protein Misfolding: From Single Cells to Multicellular Organisms	p. 85
Protein Quality Control in Cellular Compartments
Protein Folding in the Cytoplasm and the Heat Shock Response	p. 103
Protein Folding and Quality Control in the ER	p. 121
ER Stress and Its Functional Link to Mitochondria: Role in Cell Survival and Death	p. 147
Quality Control of Mitochondrial Proteostasis	p. 161
Disorders of Protein Homeostasis
Hepatic Fibrosis and Carcinogenesis in ¿1-Antitrypsin Deficiency: A Prototype for Chronic Tissue Damage in Gain-of-Function Disorders	p. 181
Alzheimer's Disease	p. 195
Huntington's Disease	p. 211
Proteostasis and Movement Disorders: Parkinson's Disease and Amyotrophic Lateral Sclerosis	p. 235
Prions	p. 259
Emergent Properties of Proteostasis in Managing Cystic Fibrosis	p. 281
Protein Misfolding and Retinal Degeneration	p. 297
Chemical and Biological Approaches for Adapting Proteostasis to Ameliorate Protein Misfolding and Aggregation Diseases-Progress and Prognosis	p. 307
Index	p. 341
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