Protein Misfolding Diseases: Current and Emerging Principles and Therapies

Copyright © 2010 John Wiley & Sons, Inc.

Protein Misfolding Diseases: Current and Emerging Principles and Therapies

Editor(s): Marina Ramirez-Alvarado, Jeffery W. Kelly, Christopher M. Dobson

Published Online: 2 JUL 2010

Print ISBN: 9780471799283

Online ISBN: 9780470572702

DOI: 10.1002/9780470572702

About this Book

Product Information

About The Product

An increasingly aging population will add to the number of individuals suffering from amyloid. Protein Misfolding Diseases provides a systematic overview of the current and emerging therapies for these types of protein misfolding diseases, including Alzheimer's, Parkinson's, and Mad Cow. The book emphasizes therapeutics in an amyloid disease context to help students, faculty, scientific researchers, and doctors working with protein misfolding diseases bridge the gap between basic science and pharmaceutical applications to protein misfolding disease.

Table of contents

    1. You have free access to this content
      Frontmatter (pages i–xxx)

    1. You have free access to this content
      Color Plates (pages c1–c24)

  3. Part I: Principles of Protein Misfolding

    1. Chapter 1

      Why Proteins Misfold (pages 1–20)

      Silvia Campioni, Elodie Monsellier and Fabrizio Chiti

    2. Chapter 2

      Endoplasmic Reticulum Stress and Oxidative Stress: Mechanisms and Link to Disease (pages 21–46)

      Jyoti D. Malhotra and Randal J. Kaufman

    3. Chapter 3

      Role of Molecular Chaperones in Protein Folding (pages 47–72)

      Kausik Chakraborty, Florian Georgescauld, Manajit Hayer-Hartl and F. Ulrich Hartl

    4. Chapter 4

      Kinetic Models for Protein Misfolding and Association (pages 73–92)

      Evan T. Powers and Frank A. Ferrone

    5. Chapter 5

      Toxicity in Amyloid Diseases (pages 93–112)

      Massimo Stefani

    6. Chapter 6

      Autophagy: An Alternative Degradation Mechanism for Misfolded Proteins (pages 113–129)

      Maria Kon and Ana Maria Cuervo

    7. Chapter 7

      Role of Posttranslational Modifications in Amyloid Formation (pages 131–144)

      Andisheh Abedini, Ruchi Gupta, Peter Marek, Fanling Meng, Daniel P. Raleigh, Humeyra Taskent and Sylvia Tracz

    8. Chapter 8

      Unraveling Molecular Mechanisms and Structures of Self-Perpetuating Prions (pages 145–174)

      Peter M. Tessier and Susan Lindquist

    9. Chapter 9

      Caenorhabditis Elegans as a Model System to Study the Biology of Protein Aggregation and Toxicity (pages 175–190)

      Elise A. Kikis, Anat Ben-Zvi and Richard I. Morimoto

    10. Chapter 10

      Using Drosophila to Reveal Insight into Protein Misfolding Diseases (pages 191–211)

      Julide Bilen and Nancy M. Bonini

    11. Chapter 11

      Animal Models to Study the Biology of Amyloid-β Protein Misfolding in Alzheimer Disease (pages 213–229)

      Karen H. Ashe

  4. Part II: Protein Misfolding Disease: Gain-of-Function and Loss-of-Function Diseases

    1. Chapter 12

      Alzheimer Disease: Protein Misfolding, Model Systems, and Experimental Therapeutics (pages 231–258)

      Donald L. Price, Alena V. Savonenko, Tong Li, Michael K. Lee and Philip C. Wong

    2. Chapter 13

      Prion Disease Therapy: Trials and Tribulations (pages 259–303)

      Valerie L. Sim and Byron Caughey

    3. Chapter 14

      Misfolding and Aggregation in Huntington Disease and Other Expanded Polyglutamine Repeat Diseases (pages 305–324)

      Ronald Wetzel

    4. Chapter 15

      Systemic Amyloidoses (pages 325–345)

      Marina Ramirez-Alvarado and Joel N. Buxbaum

    5. Chapter 16

      Hemodialysis-Related Amyloidosis (pages 347–379)

      David P. Smith, Alison E. Ashcroft and Sheena E. Radford

    6. Chapter 17

      Copper–Zinc Superoxide Dismutase, Its Copper Chaperone, and Familial Amyotrophic Lateral Sclerosis (pages 381–401)

      Duane D. Winkler, Mercedes Prudencio, Celeste Karch, David R. Borchelt and John Hart

    7. Chapter 18

      Alpha-1-Antitrypsin Deficiency (pages 403–423)

      David A. Lomas and David H. Perlmutter

    8. Chapter 19

      Folding Biology of Cystic Fibrosis: A Consortium-Based Approach to Disease (pages 425–452)

      William E. Balch, Ineke Braakman, Jeff Brodsky, Raymond Frizzell, William Guggino, Gergely L. Lukacs, Christopher Penland, Harvey Pollard, William Skach, Eric Sorscher and Philip Thomas

    9. Chapter 20

      Thiopurine S-Methyltransferase Pharmacogenomics: Protein Misfolding, Aggregation, and Degradation (pages 453–468)

      Fang Li and Richard M. Weinshilboum

    10. Chapter 21

      Gaucher Disease (pages 469–485)

      Tim Edmunds

    11. Chapter 22

      Cataract as a Protein-Aggregation Disease (pages 487–515)

      Yongting Wang and Jonathan A. King

    12. Chapter 23

      Islet Amyloid Polypeptide (pages 517–541)

      Andisheh Abedini and Daniel P. Raleigh

  5. Part III: Role of Accessory Molecules and Risk Factors

    1. Chapter 24

      Role of Metals in Alzheimer Disease (pages 543–558)

      Blaine R. Roberts and Ashley I. Bush

    2. Chapter 25

      Why Study the Role of Heparan Sulfate in In Vivo Amyloidogenesis? (pages 559–569)

      Robert Kisilevsky and John Ancsin

    3. Chapter 26

      Serum Amyloid P Component (pages 571–584)

      Simon Kolstoe and Steve Wood

    4. Chapter 27

      Role of Oxidatively Stressed Lipids in Amyloid Formation and Toxicity (pages 585–614)

      Paul H. Axelsen and Hiroaki Komatsu

    5. Chapter 28

      Role of Oxidative Stress in Protein Misfolding and/or Amyloid Formation (pages 615–630)

      Johanna C. Scheinost, Daniel P. Witter, Grant E. Boldt and Paul Wentworth

    6. Chapter 29

      Aging and Aggregation-Mediated Proteotoxicity (pages 631–644)

      Ehud Cohen and Andrew Dillin

  6. Part IV: Medical Aspects of Disease: Diagnosis and Current Therapies

    1. Chapter 30

      Imaging of Misfolded Proteins (pages 645–671)

      Harry LeVine

    2. Chapter 31

      Diagnosis of Systemic Amyloid Diseases (pages 673–688)

      Morie A. Gertz

    3. Chapter 32

      Identification of Biomarkers for Diagnosis of Amyloid Diseases: Quantitative Free Light-Chain Assays (pages 689–697)

      Jerry A. Katzmann

    4. Chapter 33

      Real-Time Observation of Amyloid-β Fibril Growth by Total Internal Reflection Fluorescence Microscopy (pages 699–709)

      Tadato Ban and Yuji Goto

    5. Chapter 34

      Current and Future Therapies for Alzheimer Disease (pages 711–774)

      Paramita Chakrabarty, Pritam Das and Todd E. Golde

    6. Chapter 35

      Current Therapies for Light-Chain Amyloidosis (pages 775–793)

      Angela Dispenzieri and Shaji Kumar

    7. Chapter 36

      Familial and Senile Amyloidosis Caused by Transthyretin (pages 795–815)

      Steven R. Zeldenrust and Merrill D. Benson

    8. Chapter 37

      Identifying Targets in α-Synuclein Metabolism to Treat Parkinson Disease and Related Disorders (pages 817–841)

      Julianna Tomlinson, Valerie Cullen and Michael G. Schlossmacher

    9. Chapter 38

      Emerging Molecular Targets in the Therapy of Dialysis-Related Amyloidosis (pages 843–865)

      Gennaro Esposito and Vittorio Bellotti

    10. Chapter 39

      Familial Amyloidosis Caused by Lysozyme (pages 867–885)

      Mireille Dumoulin

    11. Chapter 40

      Therapeutic Prospects for Polyglutamine Disease (pages 887–902)

      Maria Pennuto and Kenneth H. Fischbeck

  7. Part V: Approaches for New and Emerging Therapies

    1. Chapter 41

      Chemistry and Biology of Amyloid Inhibition (pages 903–915)

      Mark A. Findeis

    2. Chapter 42

      Immunotherapy in Secondary and Light-Chain Amyloidosis (pages 917–932)

      Jonathan Wall

    3. Chapter 43

      Anti-Misfolding and Anti-Fibrillization Therapies for Protein Misfolding Disorders (pages 933–944)

      Zane Martin and Claudio Soto

    4. Chapter 44

      Therapies Aimed at Controlling Gene Expression, Including up-Regulating a Chaperone or down-Regulating an Amyloidogenic Protein (pages 945–966)

      Gregor P. Lotz and Paul J. Muchowski

    5. Chapter 45

      Understanding and Ameliorating the TTR Amyloidoses (pages 967–1003)

      Steven M. Johnson, Luke Wiseman, Johan F. Paulsson, Sungwook Choi, Evan T. Powers, Joel N. Buxbaum and Jeffery W. Kelly

    1. You have free access to this content
      Index (pages 1005–1045)

SEARCH