A first-stop reference on proteins associated with amyloidosis. This book is the first to present a systematic overview of all known fibril-forming proteins, including their biochemical characteristics and pathophysiology. It considers the clinically recognized amyloid proteins that are known to be associated with the amyloid protein folding disorders, dealing with their common structural and thermodynamic features that lead to amyloid fibril formation and disease. Emphasis is on the thermodynamics of protein folding, the structure and physiologic effects of common oligomeric and subfibrillar intermediates and the influence of the extracellular matrix and cellular trafficking and metabolism on the genesis and catabolism of beta pleated sheet proteins. The chapters on specific amyloid proteins all follow a common structure, allowing quick access to the desired biochemical and medical data, making this an invaluable tool for clinicians and researchers alike.

Table of Contents:
Volume 1 Preface XXI List of Contributors XXIII Part I Overview of Amyloidosis and Amyloid Proteins 1 Amyloidosis and Amyloid Proteins: Brief History and Definitions 3 Per Westermark 2 Anatomic and Clinical Clues to In Vivo Mechanisms of Amyloidogenesis 29 Vittorio Bellotti, Laura Obici, Robert Kisilevsky and Giampaolo Merlini Part II Protein Structure and the Beta Pleated Sheet Conformation 3 The ß-pleated Sheet Conformation and Protein Folding: A Brief History 49 Jean D. Sipe Part III Protein Folding, Unfolding and Refolding 4 Thermodynamics and Protein Folding 65 Ilia V Baskakov 5 Role of Post-translational Chemical Modifications in Amyloid Fibril Formation 81 Melanie R. Nilsson 6 Lipid Modulators of Protein Misfolding and Aggregation 111 Christopher A. MacRaild and Geoffrey J. Howlett 7 Extracellular Matrix Heparan Sulfate Proteoglycans 131 Peter J. Neame and John T. Gallagher 8 Serum Amyloid P Component 169 David C. Kilpatrick 9 Serum amyloid P Component – Structural Features and Amyloid Recognition 189 S. P. Wood and A. R. Coker 10 Apolipoprotein E: Structural and Functional Interactions with Amyloid ß 211 W. Blaine Stine Jr. and Mary Jo LaDu Part IV Pathway to Amyloid Fibril Formation 11 Pathways to Amyloid Fibril Formation: Partially Folded Intermediates in the Fibrillation of Natively Unfolded Proteins 247 Vladimir N. Uversky and Anthony L. Fink 12 Structural Intermediates of Globular Proteins as Precursors to Amyloid Formation 275 Daniel F. Moriarty and Wilfredo Colón 13 Computational Approaches and Tools for Establishing Structural Models for Short Amyloid-forming Peptides 301 Nurit Haspel, David Zanuy, Hui-Hsu (Gavin) Tsai, Buyong Ma, Haim Wolfson and Ruth Nussinov Part V Pathophysiology of Amyloid Fibril Formation 14 Oligomers and Cellular Toxicity 319 Bruce Kagan 15 The Future of Molecular Diagnostics and Targeted Therapeutics in the Amyloidoses 343 David C. Seldin 16 Brain Dysfunction Associated with Amyloid Fibrils and Other Aggregated Proteins 355 Giorgio Giaccone, Mario Salmona, Fabrizio Tagliavini and Gianluigi Forloni Volume 2 Part VI Amyloid Proteins Brain 385 17 The Amyloid ß Protein 385 Noel D. Lazo, Samir K. Maji, Erica A. Fradinger, Gal Bitan and David B. Teplow 18 Prion Protein 493 Philippe Derreumaux 19 Familial British and Danish Dementias 515 Jorge Ghiso, Agueda Rostagno, Yasushi Tomidokoro, Tammaryn Lashley, Janice L. Holton, Gordon Plant, Tamas Revesz and Blas Frangione Systemic 527 20 Immunoglobulin 527 Fred J. Stevens 21 Transthyretin 571 Ana Margarida Damas and Maria João Saraiva 22 High-Density Lipoprotein Amyloid Proteins 589 Barbara Kluve-Beckerman 23 Gelsolin 625 Hadar Benyamini, Kannan Gunasekaran, Haim Wolfson and Ruth Nussinov 24 Lysozyme 635 Mireille Dumoulin, Vittorio Bellotti and Christopher M. Dobson 25 Fibrinogen 657 Gilles Grateau and Marc Delpech 26 ß2-Microglobulin 667 Thomas R. Jahn and Sheena E. Radford 27 Cystatin C 697 Mariusz Jaskolski and Anders Grubb Hormone 723 28 Endocrine Amyloid 723 Gunilla T. Westermark Glossary of Terms 755 Subject Index 759