Protein Misfolding Diseases : Current and Emerging Principles and Therapies

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

• ISBN13: 9780470572696
• ISBN10: 0470572698
• Format: eBook
• Copyright: 2010-06-01
• 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.

Table of Contents

ContributorS

Foreword

Preface

AcknowledgmentS

Introduction: To The Wiley Series On Protein And Peptide Science

Principles Of Protein Misfolding

Why Proteins Misfold

Endoplasmic Reticulum Stress and Oxidative Stress: Mechanisms and Link to Disease

Role of Molecular Chaperones in Protein Folding

Kinetic Models for Protein Misfolding and Association

Toxicity in Amyloid Diseases

Autophagy: An Alternative Degradation Mechanism for Misfolded Proteins

Role of Posttranslational Modifications in Amyloid Formation

Unraveling Molecular Mechanisms and Structures of Self-Perpetuating Prions

Caenorhabditis elegans as a Model System to Study the Biology of Protein Aggregation and Toxicity

Using Drosophila to Reveal Insight Into Protein Misfolding Diseases

Animal Models to Study the Biology of Amyloid-b Protein Misfolding in Alzheimer Disease

Protein Misfolding Disease: Gain-Of-Function And Loss-Of-Function Diseases

Alzheimer Disease: Protein Misfolding, Model Systems, and Experimental Therapeutics

Prion Disease Therapy: Trials and Tribulations

Misfolding and Aggregation in Huntington Disease and Other Expanded Polyglutamine Repeat Diseases

Systemic Amyloidoses

Hemodialysis-Related Amyloidosis

Copper-Zinc Superoxide Dismutase, Its Copper Chaperone, and Familial Amyotrophic Lateral Sclerosis

Alpha-1-Antitrypsin Deficiency

Folding Biology of Cystic Fibrosis: A Consortium-Based Approach to Disease

Thiopurine S-Methyltransferase Pharmacogenomics: Protein Misfolding, Aggregation, and Degradation

Gaucher Disease

Cataract as a Protein-Aggregation Disease

Islet Amyloid Polypeptide

Role Of Accessory Molecules

Role of Metals in Alzheimer Disease

Why Study the Role of Heparan Sulfate in In Vivo Amyloidogenesis?

Serum Amyloid P Component

Role of Oxidatively Stressed Lipids in Amyloid Formation and Toxicity

Role of Oxidative Stress in Protein Misfolding and/or Amyloid Formation

Aging and Aggregation-Mediated Proteotoxicity

Medical Aspects Of Disease: Diagnosis And Current Therapies

Imaging of Misfolded Proteins

Diagnosis of Systemic Amyloid Diseases

Identification of Biomarkers for Diagnosis of Amyloid Diseases: Quantitative Free Light-Chain Assays

Real-Time Observation of Amyloid-b Fibril Growth by Total Internal Reflection Fluorescence Microscopy

Current and Future Therapies for Alzheimer Disease

Current Therapies for Light-Chain Amyloidosis

Familial and Senile Amyloidosis Caused by Transthyretin

Identifying Targets in a-Synuclein Metabolism to Treat Parkinson Disease and Related Disorders

Emerging Molecular Targets in the Therapy of Dialysis-Related Amyloidosis

Familial Amyloidosis Caused by Lysozyme

Therapeutic Prospects for Polyglutamine Disease

Approaches For New And Emerging Therapies

Chemistry and Biology of Amyloid Inhibition

Immunotherapy in Secondary and Light-Chain Amyloidosis

Anti-Misfolding and Anti-Fibrillization Therapies for Protein Misfolding Disorders

Therapies Aimed at Controlling Gene Expression, Including Up-Regulating a Chaperone or Down-Regulating an Amyloidogenic Protein

Understanding and Ameliorating the TTR Amyloidoses

Index

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