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Amyloid Proteins. 2 Volumes

  • ID: 2179589
  • Book
  • 799 Pages
  • John Wiley and Sons Ltd
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There is increasing evidence that a significant number of clinical disorders involve changes in protein folding. The hallmark of these diseases is the formation of intracellular protein aggregates, the so–called amyloid plaques or fibrils. Progress in amyloid research has now finally provided the link between biochemical cause and pathological effect, opening up new ways of diagnosis and treatment of these severe disorders.

This is the first book 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.

From the contents:

∗ Overview of Amyloidosis and Amyloid Proteins

∗ Protein Structure and the Beta Pleated Sheet Conformation

∗ Protein Folding, Unfolding and Refolding

∗ Pathway to Amyloid Fibril Formation

∗ Pathophysiology of Amyloid Fibril Formation

∗ Amyloid Proteins –– Brain, Systemic, Hormone

The chapters on specific amyloid proteins all follow a common structure, allowing quick access to the desired biochemical and medical data, making this a first–stop reference for clinicians and researchers alike.
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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 3Per Westermark

2 Anatomic and Clinical Clues to In Vivo Mechanisms of Amyloidogenesis 29Vittorio Bellotti, Laura Obici, Robert Kisilevsky and Giampaolo Merlini

Part II Protein Structure and the Beta Pleated Sheet Conformation

3 The –pleated Sheet Conformation and Protein Folding: A Brief History 49Jean D. Sipe

Part III Protein Folding, Unfolding and Refolding

4 Thermodynamics and Protein Folding 65Ilia V Baskakov

5 Role of Post–translational Chemical Modifications in Amyloid Fibril Formation 81Melanie R. Nilsson

6 Lipid Modulators of Protein Misfolding and Aggregation 111Christopher A. MacRaild and Geoffrey J. Howlett

7 Extracellular Matrix Heparan Sulfate Proteoglycans 131Peter J. Neame and John T. Gallagher

8 Serum Amyloid P Component 169David C. Kilpatrick

9 Serum amyloid P Component Structural Features and Amyloid Recognition 189S. P. Wood and A. R. Coker

10 Apolipoprotein E: Structural and Functional Interactions with Amyloid  211W. 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 247Vladimir N. Uversky and Anthony L. Fink

12 Structural Intermediates of Globular Proteins as Precursors to Amyloid Formation 275Daniel F. Moriarty and Wilfredo Colón

13 Computational Approaches and Tools for Establishing Structural Models for Short Amyloid–forming Peptides 301Nurit 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 319Bruce Kagan

15 The Future of Molecular Diagnostics and Targeted Therapeutics in the Amyloidoses 343David C. Seldin

16 Brain Dysfunction Associated with Amyloid Fibrils and Other Aggregated Proteins 355Giorgio Giaccone, Mario Salmona, Fabrizio Tagliavini and Gianluigi Forloni

Volume 2

Part VI Amyloid Proteins Brain 385

17 The Amyloid Protein 385Noel D. Lazo, Samir K. Maji, Erica A. Fradinger, Gal Bitan and David B. Teplow

18 Prion Protein 493Philippe Derreumaux

19 Familial British and Danish Dementias 515Jorge Ghiso, Agueda Rostagno, Yasushi Tomidokoro, Tammaryn Lashley, Janice L. Holton, Gordon Plant, Tamas Revesz and Blas Frangione

Systemic 527

20 Immunoglobulin 527Fred J. Stevens

21 Transthyretin 571Ana Margarida Damas and Maria João Saraiva

22 High–Density Lipoprotein Amyloid Proteins 589Barbara Kluve–Beckerman

23 Gelsolin 625Hadar Benyamini, Kannan Gunasekaran, Haim Wolfson and Ruth Nussinov

24 Lysozyme 635Mireille Dumoulin, Vittorio Bellotti and Christopher M. Dobson

25 Fibrinogen 657Gilles Grateau and Marc Delpech

26 2–Microglobulin 667Thomas R. Jahn and Sheena E. Radford

27 Cystatin C 697Mariusz Jaskolski and Anders Grubb

Hormone 723

28 Endocrine Amyloid 723Gunilla T. Westermark

Glossary of Terms 755

Subject Index 759

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Jean D. Sipe
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