Protein Misfolding, Aggregation and Conformational Diseases: Part B: Molecular Mechanisms of Conformational Diseases (Protein Reviews, 6)

Protein Misfolding, Aggregation and Conformational Diseases, Part B: Molecular Mechanisms of Conformational Diseases, is a comprehensive volume providing a broad and detailed discussion of the relationships of protein misfolding and aggregation with the pathogenesis of numerous conformational diseases. While the Part A was dedicated to the description of the general mechanisms underlying protein misfolding, aggregation, and development of protein deposition disorders, this volume summarizes recent achievements in the understanding of the molecular mechanisms of conformational diseases. Research indicates that these mechanisms are highly diverse and range from the altered protein structure leading to the enhanced propensity for aggregation/deposition or the impaired functions and ending with changes in supra-molecular structures or posttranslational modification. Protein Misfolding, Aggregation and Conformational Diseases, Part B: Molecular Mechanisms of Conformational Diseases, is an ideal book for pharmaceutical scientists, molecular and cellular biologists, biochemists, immunologists, protein scientists, and biophysicists. 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 Protein Misfolding, Aggregation, and Conformational Disease, is the first book to discuss significant achievements in protein structure-function relationships in the areas of biochemistry, molecular biology and molecular medicine. This volume summarizes recent achievements in the understanding of the relationships between protein misfolding, aggregation and development of protein deposition disorders. Research indicates that various human disorders, including most neurodegenerative diseases, systemic amyloidoses and many others, arise from the misfolding and aggregation of an underlying protein. Protein Misfolding, Aggregation and Conformational Diseases is an ideal book for biochemists, protein scientists, immunologists, pharmaceutical scientists, and molecular and cellular biologists This volume fills the gap in protein review and protocal literature while summarizing recent achievements in the understanding of the relationships between protein misfoldings, aggregation, and development of protein deposition disorders. It is devoted to the general questions of conformational disorders and includes discussion of involvement of such common factors as molecular chaperones, oxidative damage, proteasome, glycosoaminoglycans, serum amyloid protein P and several others in the development of different disorders. Some experimental techniques applicable for the visualization of protein deposition in vivo and in vitro are also present

The second volume continues to fill the gap in protein review and prool 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.

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. The second volume continues to fill the gap in protein review and protocal literature 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.