R. John EllisVladimir N. UverskySilvia Campioni and Elodie Monsellier and Fabrizio ChitiJyoti D. Malhotra and Randal J. KaufmanKausik Chakraborty and Florian Georgescauld and Manajit Hayer-Hartl and F. Ulrich HartlEvan T. Powers and Frank A. FerroneMassimo StefaniMaria Kon and Ana Maria CuervoAndisheh Abedini and Ruchi Gupta and Peter Marek and Fanling Meng and Daniel P. Raleigh and Humeyra Taskent and Sylvia TraczPeter M. Tessier and Susan LindquistElise A. Kikis and Anat Ben-Zvi and Richard I. MorimotoJulide Bilen and Nancy M. BoniniKaren H. AsheDonald L. Price and Alena V. Savonenko and Tong Li and Michael K. Lee and Philip C. WongValerie L. Sim and Byron CaugheyRonald WetzelMarina Ramirez-Alvarado and Joel N. BuxbaumDavid P. Smith and Alison E. Ashcroft and Sheena E. RadfordDuane D. Winkler and Mercedes Prudencio and Celeste Karch and David R. Borchelt and P. John HartDavid A. Lomas and David H. PerlmutterWilliam E. Balch and Ineke Braakman and Jeff Brodsky and Raymond Frizzell and William Guggino and Gergely L. Lukacs and Christopher Penland and Harvey Pollard and William Skach and Eric Sorscher and Philip ThomasFang Li and Richard M. WeinshilboumTim EdmundsYongting Wang and Jonathan A. KingAndisheh Abedini and Daniel P. RaleighBlaine R. Roberts and Ashley I. BushRobert Kisilevsky and John AncsinSimon Kolstoe and Steve WoodPaul H. Axelsen and Hiroaki KomatsuJohanna C. Scheinost and Daniel P. Witter and Grant E. Boldt and Paul Wentworth, Jr.Ehud Cohen and Andrew DillinHarry LeVine, IIIMorie A. GertzJerry A. KatzmannTadato Ban and Yuji GotoParamita Chakrabarty and Pritam Das and Todd E. GoldeAngela Dispenzieri and Shaji KumarSteven R. Zeldenrust and Merrill D. BensonJulianna Tomlinson and Valerie Cullen and Michael G. SchlossmacherGennaro Esposito and Vittorio BellottiMireille DumoulinMaria Pennuto and Kenneth H. FischbeckMark A. FindeisJonathan WallZane Martin and Claudio SotoGregor P. Lotz and Paul J. MuchowskiSteven M. Johnson and R. Luke Wiseman and Natàlia Reixach and Johan F. Paulsson and Sungwook Choi and Evan T. Powers and Joel N. Buxbaum and Jeffery W. Kelly

Contributors	p. xi
Foreword	p. xix
Preface	p. xxv
Acknowledgments	p. xxvii
Introduction to the Wiley Series on Protein and Peptide Science	p. xxix
Part I Principles of Protein Misfolding
1 Why Proteins Misfold	p. 3
2 Endoplasmic Reticulum Stress and Oxidative Stress: Mechanisms and Link to Disease	p. 21
3 Role of Molecular Chaperones in Protein Folding	p. 47
4 Kinetic Models for Protein Misfolding and Association	p. 73
5 Toxicity in Amyloid Diseases	p. 93
6 Autophagy: An Alternative Degradation Mechanism for Misfolded Proteins	p. 113
7 Role of Posttranslational Modifications in Amyloid Formation	p. 131
8 Unraveling Molecular Mechanisms and Structures of Self-Perpetuating Prions	p. 145
9 Caenorhabditis elegans as a Model System to Study the Biology of Protein Aggregation and Toxicity	p. 175
10 Using Drosophila to Reveal Insight into Protein Misfolding Diseases	p. 191
11 Animal Models to Study the Biology of Amyloid-ß Protein Misfolding in Alzheimer Disease	p. 213
Part II Protein Misfolding Disease: Gain-of-Function and Loss-of-Function Diseases
12 Alzheimer Disease: Protein Misfolding, Model Systems, and Experimental Therapeutics	p. 233
13 Prion Disease Therapy: Trials and Tribulations	p. 259
14 Misfolding and Aggregation in Huntington Disease and Other Expanded Polyglutamine Repeat Diseases	p. 305
15 Systemic Amyloidoses	p. 325
16 Hemodialysis-Related Amyloidosis	p. 347
17 Copper-Zinc Superoxide Dismutase, its Copper Chaperone, and Familial Amyotrophic Lateral Sclerosis	p. 381
18 Alpha-1-Antitrypsin Deficiency	p. 403
19 Folding Biology of Cystic Fibrosis: A Consortium-based Approach to Disease	p. 425
20 Thiopurine S-Methyltransferase Pharmacogenomics: Protein Misfolding, Aggregation, and Degradation	p. 453
21 Gaucher Disease	p. 469
22 Cataract as a Protein-Aggregation Disease	p. 487
23 Islet Amyloid Polypeptide	p. 517
Part III Role of Accessory Molecules and Risk Factors
24 Role of Metals in Alzheimer Disease	p. 545
25 Why Study the Role of Heparan Sulfate in In Vivo Amyloidogenesis?	p. 559
26 Serum Amyloid P Component	p. 571
27 Role of Oxidatively Stressed Lipids in Amyloid Formation and Toxicity	p. 585
28 Role of Oxidative Stress in Protein Misfolding and/or Amyloid Formation	p. 615
29 Aging and Aggregation-Mediated Proteotoxicity	p. 631
Part IV Medical Aspects of Disease: Diagnosis and Current Therapies
30 Imaging of Misfolded Proteins	p. 647
31 Diagnosis of Systemic Amyloid Diseases	p. 673
32 Identification of Biomarkers for Diagnosis of Amyloid Diseases: Quantitative Free Light-Chain Assays	p. 689
33 Real-Time Observation of Amyloid-ß Fibril Growth by Total Internal Reflection Fluorescence Microscopy	p. 699
34 Current and Future Therapies for Alzheimer Disease	p. 711
35 Current Therapies for Light-Chain Amyloidosis	p. 775
36 Familial and Senile Amyloidosis Caused by Transthyretin	p. 795
37 Identifying Targets in ¿-Synuclein Metabolism to Treat Parkinson Disease and Related Disorders	p. 817
38 Emerging Molecular Targets in the Therapy of Dialysis-Related Amyloidosis	p. 843
39 Familial Amyloidosis Caused by Lysozyme	p. 867
40 Therapeutic Prospects for Polyglutamine Disease	p. 887
Part V Approaches for New and Emerging Therapies
41 Chemistry and Biology of Amyloid Inhibition	p. 905
42 Immunotherapy in Secondary and Light-Chain Amyloidosis	p. 917
43 Anti-Misfolding and Anti-Fibrillization Therapies for Protein Misfolding Disorders	p. 933
44 Therapies Aimed at Controlling Gene Expression, Including Up-Regulating a Chaperone or Down-Regulating an Amyloidogenic Protein	p. 945
45 Understanding and Ameliorating the TTR Amyloidoses	p. 967
Index	p. 1005