Protein misfolding diseases : current and emerging principles and therapies / edited by Marina Ramirez-Alvarado, Jeffery W. Kelly, Christopher M. Dobson.

Hoboken, N.J. : Wiley, c2010.
xxx, 1045 p., [24] p. of plates : ill. (some col,) ; 25 cm.


Related name
  • Wiley series in protein and peptide science. [More in this series]
  • Wiley series on protein and peptide science
Summary note
"Protein misfolding/aggregation diseases are a medical puzzle that has baffled researchers for many years. These diseases are characterized by the presence of protein deposits, or amyloid fibrils, which gather in the intracellular and/or extracellular spaces in a variety of tissues. Unchecked, the process of amyloidogenesis leads to proteotoxicity, cell loss, and eventually organ dysfunction, and causes such well-known and devastating diseases as Alzheimer's, Parkinson's, and Mad Cow." "Employing a diversity of research approaches, including chemical and biophysical, cellular, and animal models, the authors investigate various amyloid diseases and outline progress made in understanding them in this important book. Included are novel strategies to combat a wide variety of degenerative diseases." "Protein Misfolding Diseases uses the latest medical data from expert scientists and protein misfolding clinicians to launch a multi-targeted assault on the process of amyloid fibril formation. Fresh insight into the etiology of these maladies helps researchers gain a thorough understanding of the bases of these diseases and offers guidance in designing therapeutic strategies to build on promising new findings---and bring hope one step closer to reality."--BOOK JACKET.
Bibliographic references
Includes bibliographical references and index.
Foreword / R. John Ellis -- Introduction to the Wiley Series on Protein and Peptide Science / Vladimir N. Uversky -- PART I. PRINCIPLES OF PROTEIN MISFOLDING -- 1. Why Proteins Misfold / Fabrizio Chiti -- 2. Endoplasmic Reticulum Stress and Oxidative Stress: Mechanisms and Link to Disease / Randal J. Kaufman -- 3. Role of Molecular Chaperones in Protein Folding / F. Ulrich Hartl -- 4. Kinetic Models for Protein Misfolding and Association / Frank A. Ferrone -- 5. Toxicity in Amyloid Diseases / Massimo Stefani -- 6. Autophagy: An Alternative Degradation Mechanism for Misfolded Proteins / Ana Maria Cuervo -- 7. Role of Posttranslational Modifications in Amyloid Formation / Sylvia Tracz -- 8. Unraveling Molecular Mechanisms and Structures of Self-Perpetuating Prions / Susan Lindquist -- 9. Caenorhabditis elegans as a Model System to Study the Biology of Protein Aggregation and Toxicity / Richard I. Morimoto -- 10. Using Drosophila to Reveal Insight Into Protein Misfolding Diseases / Nancy M. Bonini -- 11. Animal Models to Study the Biology of Amyloid-β Protein Misfolding in Alzheimer Disease / Karen H. Ashe -- PART II. PROTEIN MISFOLDING DISEASE: GAIN-OF-FUNCTION AND LOSS-OF-FUNCTION DISEASES -- 12. Alzheimer Disease: Protein Misfolding, Model Systems, and Experimental Therapeutics / Philip C. Wong -- 13. Prion Disease Therapy: Trials and Tribulations / Byron Caughey -- 14. Misfolding and Aggregation in Huntington Disease and Other Expanded Polyglutamine Repeat Diseases / Ronald Wetzel -- 15. Systemic Amyloidoses / Joel N. Buxbaum -- 16. Hemodialysis-Related Amyloidosis / Sheena E. Radford -- 17. Copper-Zinc Superoxide Dismutase, Its Copper Chaperone, and Familial Amyotrophic Lateral Sclerosis / P. John Hart -- 18. Alpha-1-Antitrypsin Deficiency / David H. Perlmutter -- 19. Folding Biology of Cystic Fibrosis: A Consortium-Based Approach to Disease / Philip Thomas -- 20. Thiopurine S-Methyltransferase Pharmacogenomics: Protein Misfolding, Aggregation, and Degradation / Richard M. Weinshilboum -- 21. Gaucher Disease / Tim Edmunds -- 22. Cataract as a Protein-Aggregation Disease / Jonathan A. King -- 23. Islet Amyloid Polypeptide / Daniel P. Raleigh -- PART III. ROLE OF ACCESSORY MOLECULES AND RISK FACTORS -- 24. Role of Metals in Alzheimer Disease / Ashley I. Bush -- 25. Why Study the Role of Heparan Sulfate in In Vivo Amyloidogenesis? / John Ancsin -- 26. Serum Amyloid P Component / Steve Wood -- 27. Role of Oxidatively Stressed Lipids in Amyloid Formation and Toxicity / Hiroaki Komatsu -- 28. Role of Oxidative Stress in Protein Misfolding and/or Amyloid Formation / Paul Wentworth, Jr. -- 29. Aging and Aggregation-Mediated Proteotoxicity / Andrew Dillin -- PART IV. MEDICAL ASPECTS OF DISEASE: DIAGNOSIS AND CURRENT THERAPIES -- 30. Imaging of Misfolded Proteins / Harry LeVine, III -- 31. Diagnosis of Systemic Amyloid Diseases / Morie A. Gertz -- 32. Identification of Biomarkers for Diagnosis of Amyloid Diseases: Quantitative Free Light-Chain Assays / Jerry A. Katzmann -- 33. Real-Time Observation of Amyloid-β Fibril Growth by Total Internal Reflection Fluorescence Microscopy / Yuji Goto -- 34. Current and Future Therapies for Alzheimer Disease / Todd E. Golde -- 35. Current Therapies for Light-Chain Amyloidosis / Shaji Kumar -- 36. Familial and Senile Amyloidosis Caused by Transthyretin / Merrill D. Benson -- 37. Identifying Targets in β-Synuclein Metabolism to Treat Parkinson Disease and Related Disorders / Michael G. Schlossmacher -- 38. Emerging Molecular Targets in the Therapy of Dialysis-Related Amyloidosis / Vittorio Bellotti -- 39. Familial Amyloidosis Caused by Lysozyme / Mireille Dumoulin -- 40. Therapeutic Prospects for Polyglutamine Disease / Kenneth H. Fischbeck -- PART V. APPROACHES FOR NEW AND EMERGING THERAPIES -- 41. Chemistry and Biology of Amyloid Inhibition / Mark A. Findeis -- 42. Immunotherapy in Secondary and Light-Chain Amyloidosis / Jonathan Wall -- 43. Anti-Misfolding and Anti-Fibrillization Therapies for Protein Misfolding Disorders / Claudio Soto -- 44. Therapies Aimed at Controlling Gene Expression, Including Up-Regulating a Chaperone or Down-Regulating an Amyloidogenic Protein / Paul J. Muchowski -- 45. Understanding and Ameliorating the TTR Amyloidoses / Jeffery W. Kelly.
  • 9780471799283 (cloth)
  • 0471799289 (cloth)
C - S

Supplementary Information