Protein Misfolding Diseases: Methods and Protocols

Front Matter ....Pages i-xiii
Front Matter ....Pages 1-1
 Biophysical and Spectroscopic Methods for Monitoring Protein Misfolding and Amyloid Aggregation  (Joana S. Cristóvão, Bárbara J. Henriques, Cláudio M. Gomes)....Pages 3-18
 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
 Vesicle-Based Assays to Study Membrane Interactions of Amyloid Peptides  (Ravit Malishev, Sofiya Kolusheva, Raz Jelinek)....Pages 39-51
 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
 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
 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
 Methods for Structural Analysis of Amyloid Fibrils in Misfolding Diseases  (Devkee M. Vadukul, Youssra K. Al-Hilaly, Louise C. Serpell)....Pages 109-122
 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
Front Matter ....Pages 155-155
 Monitoring Aggregate Clearance and Formation in Cell-Based Assays  (Evelien Eenjes, Young Joo Yang-Klingler, Ai Yamamoto)....Pages 157-169
 Monitoring Proteome Stress in Live Cells Using HaloTag-Based Fluorogenic Sensor  (Yu Liu, Matthew Fares, Xin Zhang)....Pages 171-182
 Quantification of Protein Aggregates Using Bimolecular Fluorescence Complementation  (Vibha Prasad, Aaron Voigt)....Pages 183-193
 Screening Protein Aggregation in Cells Using Fluorescent Labels Coupled to Flow Cytometry  (Salvador Ventura, Susanna Navarro)....Pages 195-212
 Induction of Cu/Zn Superoxide Dismutase (SOD1) Aggregation in Living Cells  (Edward Pokrishevsky, Jeremy Nan, Neil R. Cashman)....Pages 213-224
 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
 Superresolution Fluorescence Imaging of Mutant Huntingtin Aggregation in Cells  (Steffen J. Sahl, Willianne I. M. Vonk)....Pages 241-251
Front Matter ....Pages 253-253
 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
 Methods to Screen Compounds Against Mutant p53 Misfolding and Aggregation for Cancer Therapeutics  (Giulia Diniz da Silva Ferretti, Danielly C. Ferraz da Costa, Jerson L. Silva, Luciana Pereira Rangel)....Pages 265-277
 Early Stage Discovery and Validation of Pharmacological Chaperones for the Correction of Protein Misfolding Diseases  (Oscar Aubi, Per M. Knappskog, Aurora Martinez)....Pages 279-292
 Constructing Kinetically Controlled Denaturation Isotherms of Folded Proteins Using Denaturant-Pulse Chaperonin Binding  (Pierce T. O’Neil, Alexandra J. Machen, Jackie A. Thompson, Wei Wang, Quyen Q. Hoang, Michael R. Baldwin et al.)....Pages 293-304
 In Vitro Prion Amplification Methodology for Inhibitor Screening  (Tuane Cristine R. G. Vieira, Jerson L. Silva)....Pages 305-316
 SolubiS: Optimizing Protein Solubility by Minimal Point Mutations  (Rob van der Kant, Joost van Durme, Frederic Rousseau, Joost Schymkowitz)....Pages 317-333
Back Matter ....Pages 335-338