Protein Misfolding, Aggregation and Conformational Diseases (Protein Reviews)

Front Matter....Pages i-xxv
Front Matter....Pages 1-1
The Pathogenesis of Alzheimer’s Disease: General Overview....Pages 3-29
Free Radicals, Metal Ions, and Aβ Aggregation and Neurotoxicity....Pages 31-47
Progress in Understanding the Mechanisms of Neuronal Dysfunction and Degeneration in Parkinson’s Disease....Pages 49-59
α-Synuclein Aggregation and Parkinson’s Disease....Pages 61-110
Cell Biology of α -Synuclein: Implications in Parkinson’s Disease and Other Lewy Body Diseases....Pages 111-124
Pathogenesis of Prion Diseases....Pages 125-146
Mammalian Prion Protein....Pages 147-163
The Yeast Prion Proteins Sup35p and Ure2p....Pages 165-182
Immunoglobulin Light Chain and Systemic Light-Chain Amyloidosis....Pages 183-197
Pancreatic Islet Amyloid and Diabetes....Pages 199-216
β 2 -Microglobulin and Dialysis-Related Amyloidosis....Pages 217-239
Serum Amyloid A and AA Amyloidosis....Pages 241-256
Front Matter....Pages 257-257
Transthyretin and the Transthyretin Amyloidoses....Pages 259-283
Human Lysozyme....Pages 285-308
Serpins and the Diversity of Conformational Diseases....Pages 309-323
Front Matter....Pages 325-325
Human Copper-Zinc Superoxide Dismutase and Familial Amyotrophic Lateral Sclerosis....Pages 327-344
Understanding the Effects of Cancer-Associated Mutations in the Tumor Suppressor Protein p53: Structural Consequences of Mutations and Possible Ways of Rescuing Oncogenic Mutants....Pages 345-361
Front Matter....Pages 363-363
Protein Aggregation in Muscle Fibers and Respective Neuromuscular Disorders....Pages 365-389
Muscular Dystrophies and Protein Mutations....Pages 391-407
The Functional Consequences of Dystrophin Deficiency in Skeletal Muscles....Pages 409-433
Front Matter....Pages 363-363
Eye Lens Proteins and Cataracts....Pages 435-447
Front Matter....Pages 449-449
Glutamine/Asparagine-Rich Regions in Proteins and Polyglutamine Diseases....Pages 451-463
Mechanistic Insights into the Polyglutamine Ataxias....Pages 465-479
Molecular Pathogenesis of the Polyglutamine Disease: Spinal and Bulbar Muscular Atrophy....Pages 481-495
Front Matter....Pages 497-497
Protein Glycation and Cataract: A Conformational Disease....Pages 499-514
Defective Glycosylation and Muscular Dystrophies....Pages 515-526
Back Matter....Pages 527-537