Protein Misfolding and Cellular Stress in Disease and Aging: Concepts and Protocols

Part I: Concepts 1. Protein Misfolding and Cellular Stress: An Overview Niels Gregersen and Peter Bross 2. Protein Aggregation Diseases: Toxicity of Soluble Prefibrillar Aggregates and Their Clinical Significance Massimo Stefani 3. Consequences of Stress in the Secretory Pathway: The ER Stress Response and Its Role in the Metabolic Syndrome Martin Schr?der and Louise Sutcliffe 4. What Role Does Mitochondrial Stress Play in Neurodegenerative Diseases? Alicia Mae Pickrell and Carlos Torres Moraes 5. Autophagy in Disease Dalibor Mijaljica, Mark Prescott, and Rodney J. Devenish 6. Mitophagy and Mitoptosis in Disease Processes Dalibor Mijaljica, Mark Prescott, and Rodney J. Devenish 7. Cellular Stress and Protein Misfolding During Aging Rajiv Vaid Basaiawmoit and Suresh I.S. Rattan 8. Measuring Consequences of Protein Misfolding and Cellular Stress Using OMICS Techniques Peter Bross, Johan Palmfeldt, Jakob Hansen, S ren Vang, and Niels Gregersen Part II: Protocols 9. Production of Cells with Targeted Integration of Gene Variants of Human ABC Transporter for Stable and Regulated Expression Using the Flp Recombinase System Kanako Wakabayashi-Nakao, Ai Tamura, Shoko Koshiba, Yu Toyoda, Hiroshi Nakagawa, and Toshihisa Ishikawa 10. Stress Response Profiles in Human Fibroblasts Exposed to Heat Shock or Oxidative Stress Christina Bak Pedersen and Niels Gregersen 11. Examining Ubiquitinated Protein Aggregates in Tissue Sections Natalia A. Kaniuk and John H. Brumell 12. Determination of Proteasomal Activities Cristovao F. Lima and Suresh I.S. Rattan 13. Measurement of Autophagy in Cells and Tissues Isei Tanida and Satoshi Waguri 14. Transcription Factor Sequestration by Polyglutamine Proteins Tomoyuki Yamanaka and Nobuyuki Nukina 15. Biological Membranes as Protein Aggregation Matrices and Targets of Amyloid Toxicity Monica Bucciantini and Cristina Cecchi 16. Measurement of Mitochondrial ROS Production Anatoly A. Starkov 17. In vivo Detectionof Oxidized Proteins: A Practical Approach to Tissue-Derived Mitochondria Francesca Maltecca and Giorgio Casari 18. Measurement of Oxidized/Reduced Glutathione Ratio Joshua B. Owen and D. Allan Butterfield 19. Determination of Altered Mitochondria Ultrastructure by Electron Microscopy Shoichi Sasaki 20. Assessing Bad Sub-Cellular Localization under Conditions Associated with Prevention or Promotion of Mitochondrial Permeability Transition-Dependent Toxicity Liana Cerioni and Orazio Cantoni 21. A Cellular Viability Assay to Monitor Drug Toxicity Jakob Hansen and Peter Bross 22. Rescue of Misfolded Proteins and Stabilization by Small Molecules Raymond C. Stevens, Javier Sancho, and Aurora Martine