Protein Quality Control in Neurodegenerative Diseases

1. Front Matter

   Pages i-xii

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2. The regulation and function of the heat shock response

   • Eric Guisbert, Richard I. Morimoto

   Pages 1-18

3. The endoplasmic reticulum unfolded protein response and neurodegeneration

   • David Ron

   Pages 19-35

4. Proteostasis and the aging pathways

   • Ian Nicastro, Andrew Dillin

   Pages 37-52

5. The membrane sources of macroautophagy

   • Daniel J. Klionsky, Melinda A. Lynch-Day, Jiefei Geng, Wei-Lien Yen

   Pages 53-61

6. Selective autophagy in cellular quality control

   • Susmita Kaushik, Ana Maria Cuervo

   Pages 63-75

7. Quality control of proteins and organelles by autophagy

   • Noboru Mizushima

   Pages 77-86

8. The role of the co-chaperone BAG3 in selective macroautophagy: implications for aging and disease

   • Christian Behl

   Pages 87-96

9. Predicting fates in models of neurodegenerative disease: longitudinal measures of protein homeostasis in live neurons

   • Steven Finkbeiner

   Pages 97-110

10. Therapeutic potential of longevity modulators as neuroprotective targets in neurodegenerative disease

    • Rafael Vazquez-Manrique, Cendrine Tourette, Christian Neri

    Pages 111-120

11. The interplay between the chaperonin TRiC and N-terminal region of Huntingtin mediates Huntington’s Disease aggregation and pathogenesis

    • Koning Shen, Judith Frydman

    Pages 121-132

12. Back Matter

    Pages 133-135

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The health of the proteome depends upon protein quality control to regulate the proper synthesis, folding, translocation, and clearance of proteins. The cell is challenged constantly by environmental and physiological stress, aging, and the chronic expressions of disease associated misfolded proteins. Substantial evidence supports the hypothesis that the expression of damaged proteins initiates a cascade of molecular events that leads to Alzheimer's disease, Parkinson's disease, amyotrophic lateral sclerosis, Huntington's disease, and other diseases of protein conformation.

• autophagy
• cellular defense
• cytoplasmic degradation
• neuronal control
• protein homeostasis

From the reviews:

“This is a very good cutting edge book of scholarly import on the functioning of molecules and signal transduction in both neurodegeneration and neuroprotection. … I recommend this book highly to scientists in disease pathophysiology work.” (Joseph J. Grenier, Amazon.com, June, 2014)

• Morimoto Lab, Northwestern University, Evanston, USA

  Richard I. Morimoto

• Fondation IPSEN, Boulogne-Billancourt Cedex, France

  Yves Christen

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