Amyloid proteins : The Beta sheet confor mation & disease, 2 Volume-set

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.

Volume 1. Part I: Overview of Amyloidosis and Amyloid Proteins.1 Amyloidosis and Amyloid Proteins: Brief History and Definitions. 2 Anatomic and Clinical Clues to In Vivo Mechanisms of Amyloidogenesis. Part II: Protein Structure and the Beta Pleated Sheet Conformation. The &beta,-pleated Sheet Conformation and Protein Folding: A Brief History. Part III: Protein Folding, Unfolding and Refolding. 4 Thermodynamics and Protein Folding. 5 Role of Post-translational Chemical Modifications in Amyloid Fibril Formation. 6 Lipid Modulators of Protein Misfolding and Aggregation. 7 Extracellular Matrix Heparan Sulfate Proteoglycans. 8 Serum Amyloid P Component. 9 Serum amyloid P Component – Structural Features and Amyloid Recognition. 10 Apolipoprotein E: Structural and Functional Interactions with Amyloid &beta. Part IV: Pathway to Amyloid Fibril Formation. 11 Pathways to Amyloid Fibril Formation: Partially Folded Intermediates in the Fibrillation of Natively Unfolded Proteins. 12 Structural Intermediates of Globular Proteins as Precursors to Amyloid Formation. 13 Computational Approaches and Tools for Establishing Structural Models for Short Amyloid-forming Peptides. Part V: Pathophysiology of Amyloid Fibril Formation.14 Oligomers and Cellular Toxicity. 15 The Future of Molecular Diagnostics and Targeted Therapeutics in the Amyloidoses. 16 Brain Dysfunction Associated with Amyloid Fibrils and Other Aggregated Proteins. Volume 2. Part VI: Amyloid Proteins Brain.17 The Amyloid &beta, Protein. 18 Prion Protein. 19 Familial British and Danish Dementias. Systemic. 20 Immunoglobulin. 21 Transthyretin. 22 High-Density Lipoprotein Amyloid Proteins. 23 Gelsolin. 24 Lysozyme. 25 Fibrinogen. 26 &beta,₂-Microglobulin. 27 Cystatin C. Hormone. 28 Endocrine Amyloid. Glossary of Terms. Subject Index.