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.
Author(s): Jean D. Sipe
Publisher: Wiley-VCH
Year: 2005
Language: English
Commentary: 28503
Pages: 779
Halftitle......Page 2
Further Titles of Interest......Page 3
Inside Cover......Page 4
Copyright......Page 5
Contents......Page 6
Preface......Page 21
List of Contributors......Page 23
Part I Overview of Amyloidosis and Amyloid Proteins......Page 26
1: Amyloidosis and Amyloid Proteins: Brief History and Definitions......Page 27
2: Anatomic and Clinical Clues to In Vivo Mechanisms of Amyloidogenesis......Page 52
Part II: Protein Structure and the Beta Pleated Sheet Conformation......Page 70
3: The beta-pleated Sheet Conformation and Protein Folding: A Brief History......Page 71
Part III: Protein Folding, Unfolding and Refolding......Page 84
4: Thermodynamics and Protein Folding......Page 85
5: Role of Post-translational Chemical Modifications in Amyloid Fibril Formation......Page 101
6: Lipid Modulators of Protein Misfolding and Aggregation......Page 130
7: Extracellular Matrix Heparan Sulfate Proteoglycans......Page 149
8: Serum Amyloid P Component......Page 186
9: Serum amyloid P Component – Structural Features and Amyloid Recognition......Page 205
10: Apolipoprotein E: Structural and Functional Interactions with Amyloid beta......Page 226
Part IV: Pathway to Amyloid Fibril Formation......Page 259
11: Pathways to Amyloid Fibril Formation: Partially Folded Intermediates in the Fibrillation of Natively Unfolded Proteins......Page 260
12: Structural Intermediates of Globular Proteins as Precursors to Amyloid Formation......Page 287
13: Computational Approaches and Tools for Establishing Structural Models for Short Amyloid-forming Peptides......Page 313
Part V: Pathophysiology of Amyloid Fibril Formation......Page 328
14: Oligomers and Cellular Toxicity......Page 329
15: The Future of Molecular Diagnostics and Targeted Therapeutics in the Amyloidoses......Page 353
16: Brain Dysfunction Associated with Amyloid Fibrils and Other Aggregated Proteins......Page 365
Volume 2......Page 393
Part VI Amyloid Proteins......Page 395
17: The Amyloid beta-Protein......Page 396
18: Prion Protein......Page 503
19: Familial British and Danish Dementias......Page 524
20: Immunoglobulin......Page 536
21: Transthyretin......Page 579
22: High-Density Lipoprotein Amyloid Proteins......Page 597
23: Gelsolin......Page 632
24: Lysozyme......Page 642
25: Fibrinogen......Page 664
26: beta2-Microglobulin......Page 674
27: Cystatin C......Page 703
28: Endocrine Amyloid......Page 729
Glossary of Terms......Page 761
Subject Index......Page 764
