Protein Misfolding Diseases

1. Front Matter

   Pages i-xiii

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2. Protein Biophysics Assays

   1. Front Matter

      Pages 1-1

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

      • Joana S. Cristóvão, Bárbara J. Henriques, Cláudio M. Gomes

      Pages 3-18

   3. 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. Orrù, Andrew G. Hughson et al.

      Pages 19-37

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

      • Ravit Malishev, Sofiya Kolusheva, Raz Jelinek

      Pages 39-51

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

      • Naoto Soya, Ariel Roldan, Gergely L. Lukacs

      Pages 53-67

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

      • Donatella Bulone, Pier Luigi San Biagio, Tatiana Quiñones-Ruiz, Manuel Rosario-Alomar, Igor K. Lednev, Frank T. Robb et al.

      Pages 69-92

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

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

      Pages 93-108

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

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

      Pages 109-122

   9. 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 et al.

      Pages 123-153

3. Cellular and Proteostasis Assays

   1. Front Matter

      Pages 155-155

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

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

      Pages 157-169

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

      • Yu Liu, Matthew Fares, Xin Zhang

      Pages 171-182

   4. Quantification of Protein Aggregates Using Bimolecular Fluorescence Complementation

      • Vibha Prasad, Aaron Voigt

      Pages 183-193

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

      • Salvador Ventura, Susanna Navarro

      Pages 195-212

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

      • Edward Pokrishevsky, Jeremy Nan, Neil R. Cashman

      Pages 213-224

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

      • Cagla Cömert, Paula Fernandez-Guerra, Peter Bross

      Pages 225-239

   8. Superresolution Fluorescence Imaging of Mutant Huntingtin Aggregation in Cells

      • Steffen J. Sahl, Willianne I. M. Vonk

      Pages 241-251

4. Protein Folding Recovery and Correction Assays

   1. Front Matter

      Pages 253-253

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   2. Thermal Shift and Stability Assays of Disease-Related Misfolded Proteins Using Differential Scanning Fluorimetry

      • Tânia G. Lucas, Cláudio M. Gomes, Bárbara J. Henriques

      Pages 255-264

This detailed book gathers a broad collection of experimental approaches to assist researchers in setting up different methods to investigate protein conformational disorders. Beginning with a section on assays focusing on biophysical approaches to study protein (mis)folding, the volume continues with sections on cellular and proteostasis assays as well as assays for protein folding correction and recovery, combining methods such as thermal shift assays, in silico improvement of protein solubility, and compound screening, an important area of research as it may open avenues for therapeutic strategies. Written for the highly successful Methods in Molecular Biology series, chapters include introductions to their respective topics, lists of the necessary materials and reagents, step-by-step, readily reproducible laboratory protocols, and tips for troubleshooting and avoiding known pitfalls. 

Authoritative and practical, Protein Misfolding Diseases:Methods and Protocols serves as an ideal guide for researchers seeking to advance our knowledge of protein conformational disorders.

• Protein biophysics
• Chaperonopathies
• Proteostasis
• Protein folding diseases
• Conformational disorders
• Amyloid forming diseases
• Protein folding correction

• BioISI – Biosystems and Integrative Sciences Institute, Faculty of Sciences University of Lisbon, Lisbon, Portugal

  Cláudio M. Gomes

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