Protein Misfolding Diseases Current and Emerging Principles and Therapies

by Ramirez-Alvarado, Marina; Kelly, Jeffery W.; Dobson, Christopher M.

• ISBN13: 9780471799283
• ISBN10: 0471799289
• Edition: 1st
• Format: Hardcover
• Copyright: 2010-06-28
• Publisher: Wiley

Summary

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.

Author Biography

Marina Ramirez-Alvarado is an Assistant Professor of Biochemistry and Molecular Biology at the Mayo Clinic. Despite being a young investigator, she is already a world leader in the study of molecular determinants of light chain amyloidosis, a rare misfolding disease. Jeffery W. Kelly is a Professor of Chemistry and Molecular and Experimental Medicine at the Scripps Research Institute. He is one of the leading authorities in the field of protein misfolding, with over 250 publications to his credit. Christopher M. Dobson is a Professor in the Department of Chemistry at the University of Cambridge. Dr. Dobson is a leading researcher studying the structural and biochemical bases of protein misfolding diseases and has over 500 publications.

Table of Contents

Contributors	p. xi
Foreword	p. xix
Preface	p. xxv
Acknowledgments	p. xxvii
Introduction to the Wiley Series on Protein and Peptide Science	p. xxix
Principles of Protein Misfolding
Why Proteins Misfold	p. 3
Endoplasmic Reticulum Stress and Oxidative Stress: Mechanisms and Link to Disease	p. 21
Role of Molecular Chaperones in Protein Folding	p. 47
Kinetic Models for Protein Misfolding and Association	p. 73
Toxicity in Amyloid Diseases	p. 93
Autophagy: An Alternative Degradation Mechanism for Misfolded Proteins	p. 113
Role of Posttranslational Modifications in Amyloid Formation	p. 131
Unraveling Molecular Mechanisms and Structures of Self-Perpetuating Prions	p. 145
Caenorhabditis elegans as a Model System to Study the Biology of Protein Aggregation and Toxicity	p. 175
Using Drosophila to Reveal Insight into Protein Misfolding Diseases	p. 191
Animal Models to Study the Biology of Amyloid-ß Protein Misfolding in Alzheimer Disease	p. 213
Protein Misfolding Disease: Gain-of-Function and Loss-of-Function Diseases
Alzheimer Disease: Protein Misfolding, Model Systems, and Experimental Therapeutics	p. 233
Prion Disease Therapy: Trials and Tribulations	p. 259
Misfolding and Aggregation in Huntington Disease and Other Expanded Polyglutamine Repeat Diseases	p. 305
Systemic Amyloidoses	p. 325
Hemodialysis-Related Amyloidosis	p. 347
Copper-Zinc Superoxide Dismutase, its Copper Chaperone, and Familial Amyotrophic Lateral Sclerosis	p. 381
Alpha-1-Antitrypsin Deficiency	p. 403
Folding Biology of Cystic Fibrosis: A Consortium-based Approach to Disease	p. 425
Thiopurine S-Methyltransferase Pharmacogenomics: Protein Misfolding, Aggregation, and Degradation	p. 453
Gaucher Disease	p. 469
Cataract as a Protein-Aggregation Disease	p. 487
Islet Amyloid Polypeptide	p. 517
Role of Accessory Molecules and Risk Factors
Role of Metals in Alzheimer Disease	p. 545
Why Study the Role of Heparan Sulfate in In Vivo Amyloidogenesis?	p. 559
Serum Amyloid P Component	p. 571
Role of Oxidatively Stressed Lipids in Amyloid Formation and Toxicity	p. 585
Role of Oxidative Stress in Protein Misfolding and/or Amyloid Formation	p. 615
Aging and Aggregation-Mediated Proteotoxicity	p. 631
Medical Aspects of Disease: Diagnosis and Current Therapies
Imaging of Misfolded Proteins	p. 647
Diagnosis of Systemic Amyloid Diseases	p. 673
Identification of Biomarkers for Diagnosis of Amyloid Diseases: Quantitative Free Light-Chain Assays	p. 689
Real-Time Observation of Amyloid-ß Fibril Growth by Total Internal Reflection Fluorescence Microscopy	p. 699
Current and Future Therapies for Alzheimer Disease	p. 711
Current Therapies for Light-Chain Amyloidosis	p. 775
Familial and Senile Amyloidosis Caused by Transthyretin	p. 795
Identifying Targets in ¿-Synuclein Metabolism to Treat Parkinson Disease and Related Disorders	p. 817
Emerging Molecular Targets in the Therapy of Dialysis-Related Amyloidosis	p. 843
Familial Amyloidosis Caused by Lysozyme	p. 867
Therapeutic Prospects for Polyglutamine Disease	p. 887
Approaches for New and Emerging Therapies
Chemistry and Biology of Amyloid Inhibition	p. 905
Immunotherapy in Secondary and Light-Chain Amyloidosis	p. 917
Anti-Misfolding and Anti-Fibrillization Therapies for Protein Misfolding Disorders	p. 933
Therapies Aimed at Controlling Gene Expression, Including Up-Regulating a Chaperone or Down-Regulating an Amyloidogenic Protein	p. 945
Understanding and Ameliorating the TTR Amyloidoses	p. 967
Index	p. 1005
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