Protein Misfolding Diseases: Methods and Protocols

Part I: Protein Biophysics Assays

1. Biophysical and Spectroscopic Methods for Monitoring Protein Misfolding and Amyloid Aggregation

Joana S. Crist?v?o, B?rbara J. Henriques, and Cl?udio M. Gomes

2. Ultrasensitive RT-QuIC Seed Amplification Assays for Disease-Associated Tau, α-Synuclein, and Prion Aggregates

Eri Saijo, Bradley R. Groveman, Allison Kraus, Michael Metrick, Christina D. Orr?, Andrew G. Hughson, and Byron Caughey

3. Vesicle-Based Assays to Study Membrane Interactions of Amyloid Peptides

Ravit Malishev, Sofiya Kolusheva, and Raz Jelinek

4. Differential Scanning Fluorimetry and Hydrogen Deuterium Exchange Mass Spectrometry to Monitor the Conformational Dynamics of NBD1 in Cystic Fibrosis

Naoto Soya, Ariel Roldan, and Gergely L. Lukacs

5. A Multipronged Method for Unveiling Subtle Structural-Functional Defects of Mutant Chaperone Molecules Causing Human Chaperonopathies

Donatella Bulone, Pier Luigi San Biagio, Tatiana Qui?ones-Ruiz, Manuel Rosario-Alomar, Igor K. Lednev, Frank T. Robb, Everly Conway de Macario, and Alberto J.L. Macario

6. High-Throughput Microplate-Based Fluorescence Assays for Studying Stochastic Aggregation of Superoxide Dismutase-1

Alireza Abdolvahabi, Sanaz Rasouli, Corbin M. Croom, and Devon L. Plewman

7. Methods for Structural Analysis of Amyloid Fibrils in Misfolding Diseases

Devkee M. Vadukul, Youssra K. Al-Hilaly, and Louise C. Serpell

8. Assays for Light Chain Amyloidosis Formation and Cytotoxicity

Luis M. Blancas-Mejia, Pinaki Misra, Christopher J. Dick, Marta Marin Argany, Keely R. Redhage, Shawna A. Cooper, and Marina Ramirez-Alvarado

Part II: Cellular And Proteostasis Assays

9. Monitoring Aggregate Clearance and Formation in Cell-Based Assays

Evelien Eenjes, Young Joo Yang-Klingler, and Ai Yamamoto

10. Monitoring Proteome Stress in Live Cells Using HaloTag-Based Fluorogenic Sensor

11. Quantification of Protein Aggregates Using Bimolecular Fluorescence Complementation

Vibha Prasad and Aaron Voigt

12. Screening Protein Aggregation in Cells Using Fluorescent Labels Coupled to Flow Cytometry

Salvador Ventura and Susanna Navarro

13. Induction of Cu/Zn Superoxide Dismutase (SOD1) Aggregation in Living Cells

Edward Pokrishevsky, Jeremy Nan, and Neil R. Cashman

14. A Cell Model for HSP60 Deficiencies: Modeling Different Levels of Chaperonopathies Leading to Oxidative Stress and Mitochondrial Dysfunction

Cagla C?mert, Paula Fernandez Guerra, and