Main description:

The second volume continues to fill the gap in protein review and protocol literature. It does this while summarizing recent achievements in the understanding of the relationships between protein misfoldings, aggregation, and development of protein deposition disorders. The focus of Part B is the molecular basis of differential disorders.

Contents:

Altered Protein Structure and Enhanced Aggregation/Deposition.- The Pathogenesis of Alzheimer's Disease: General Overview.- Free Radicals, Metal Ions, and A? Aggregation and Neurotoxicity.- Progress in Understanding the Mechanisms of Neuronal Dysfunction and Degeneration in Parkinson's Disease.- ?-Synuclein Aggregation and Parkinson's Disease.- Cell Biology of ?-Synuclein: Implications in Parkinson's Disease and Other Lewy Body Diseases.- Pathogenesis of Prion Diseases.- Mammalian Prion Protein.- The Yeast Prion Proteins Sup35p and Ure2p.- Immunoglobulin Light Chain and Systemic Light-Chain Amyloidosis.- Pancreatic Islet Amyloid and Diabetes.- ?2-Microglobulin and Dialysis-Related Amyloidosis.- Serum Amyloid A and AA Amyloidosis.- Point Mutations and Enhanced Protein Deposition.- Transthyretin and the Transthyretin Amyloidoses.- Human Lysozyme.- Serpins and the Diversity of Conformational Diseases.- Altered Protein Structure and Impaired Function.- Human Copper-Zinc Superoxide Dismutase and Familial Amyotrophic Lateral Sclerosis.- Understanding the Effects of Cancer-Associated Mutations in the Tumor Suppressor Protein p53: Structural Consequences of Mutations and Possible Ways of Rescuing Oncogenic Mutants.- Changes in Supramolecular Structure.- Protein Aggregation in Muscle Fibers and Respective Neuromuscular Disorders.- Muscular Dystrophies and Protein Mutations.- The Functional Consequences of Dystrophin Deficiency in Skeletal Muscles.- Eye Lens Proteins and Cataracts.- Altered Protein Structure and Changes in Cellular/Nuclear Function.- Glutamine/Asparagine-Rich Regions in Proteins and Polyglutamine Diseases.- Mechanistic Insights into the Polyglutamine Ataxias.- Molecular Pathogenesis of the Polyglutamine Disease: Spinal and Bulbar Muscular Atrophy.- Post-Translational Modification and Protein Conformational Diseases.- Protein Glycation and Cataract: A Conformational Disease.- Defective Glycosylation and Muscular Dystrophies.