(The) molecular and cellular basis of neurodegenerative diseases : underlying mechanisms

목차 (Table of Contents)

• CONTENTS
• List of Contributors = xi
• Preface = xiii
• 1. Solving the Puzzle of Neurodegeneration / MICHAEL S. WOLFE
• Introduction : The General Problem of Neurodegeneration = 1
• Epidemiology and Clinical Presentation = 3
• Molecular Pathology = 5
• Genetics = 6
• Molecular Clues to Mechanisms of Pathogenesis = 8
• Common Themes and Controversies in Neurodegeneration = 10
• Animal Models = 13
• Prospects for Therapeutics = 16
• Conclusions and Perspective = 17
• References = 18
• 2. Prion Diseases / ROBERT C.C. MERCER ; ALEX J. MCDONALD ; ERIN BOVE-FENDERSON ; CHENG FANG ; BEI WU ; DAVID A. HARRIS
• Introduction and Historical Perspective =23
• Molecular Mechanism of Prion Propagation = 24
• The Cellular Prion Protein : Structure and Proteolytic Processing = 27
• Physiological Function of PrPc = 27
• Mechanisms of PrPSc Toxicity : The N-Terminal Domain of PrPᵓ Possess a Toxic Effector Activity = 30
• Human Prion Diseases = 34
• Animal Prion Diseases = 37
• Prion Strains and Species Barriers = 38
• Methods for Propagation and Detection of Prions = 39
• Therapeutic Approaches = 39
• PrPc and the Alzheimer's Aβ Peptide = 41
• Prion-like Propagation of Misfolded Proteins in Other Neurodegenerative Diseases = 43
• Concluding Remarks = 45
• References = 45
• 3. Alzheimer's Disease : Toward a Quantitative Biological Approach in Describing its Natural History and Underlying Mechanisms / COLIN L. MASTERS ; BEN GU ; SIMON LAWS ; YEN Y. LIM ; BLAINE ROBERTS ; VICTOR L. VILLEMAGNE ; KONRAD BEYREUTHER
• Quantitative Approach to Basic AD Demographics = 59
• Clearance Mechanisms and Impaired Phagocytosis = 70
• Calculating Polygenic Risk Scores = 71
• Disease-Modifying Strategies : Models of Aβ Accumulation in Alzheimer's Disease - Implications for Aβ Amyloid-Targeting Therapies = 73
• References = 74
• 4. Neurodegeneration and the Ordered Assembly of Tau / MICHEL GOEDERT
• Introduction = 81
• Tau Isoforms = 82
• Tau Aggregation = 84
• Genetics of MAPT = 86
• Propagation of Tau Aggregates = 88
• Strains of Aggregated Tau = 92
• Acknowledgments = 92
• References = 93
• Further Reading = 98
• 5. Amyotrophic Lateral Sclerosis and Other TDP - 43 Proteinopathies / JORGE GOMEZ-DEZA ; CHRISTOPHER E. SHAW
• TDP-43 Biology = 99
• Amyotrophic Lateral Sclerosis = 100
• ALS-TDP-43 = 102
• ALS-SOD1 = 103
• ALS-FUS = 104
• ALS-C90RF72 = 104
• Mechanisms Dysregulated = 106
• Other TDP-43 Proteinopathies = 109
• Conclusions = 111
• References = 111
• 6. Parkinson's Disease and Other Synucleinopathies / MARK R. COOKSON
• Introduction : The Pathology of Parkinson's Disease = 111
• Genes Associated with Synucleinopathies = 120
• Summary = 132
• Acknowledgment = 132
• References = 132
• 7. Huntington's Disease and Other Polyglutamine Repeat Diseases: Molecular Mechanisms and Pathogenic Pathways / AUDREY S. DICKEY ; ALBERT R. LASPADA
• Polyglutamine Expansion = 146
• Posttranslational Modifications = 159
• Other Mechanisms = 170
• Therapeutic Opportunities for polyQ Diseases = 171
• Acknowledgments = 174
• References = 174
• 8. Prion-Like Propagation in Neurodegenerative Diseases / WOUTER PEELAERTS ; VEERLE BAEKELANDT ; PATRIK BRUNDIN
• Introduction = 190
• Prion-Like Proteins in Neurodegenerative Diseases = 196
• Mechanistic, Functional, and Pathogenic Properties of Prions = 206
• Mechanisms of Transport and Cell-to-Cell Propagation = 209
• The Strain Hypothesis = 214
• How Prion Strains Might Come to Our Aid = 219
• The Issue of Communicability of "Prion-Like" Diseases = 223
• Conclusions = 226
• References = 227
• 9. Neurodegenerative Diseases as Protein Folding Disorders / JEREMY D. BAKER ; JACK M. WEBSTER ; LINDSEY B. SHELTON ; JOHN KOREN Ⅲ ; VLADIMIR N. UVERSKY ; LAURA J. BLAIR ; CHAD A. DICKEY
• Introduction = 243
• Roles for Protein Folding, Modification, and Degradation = 244
• What is Protein Misfolding and Why Does it Occur? = 246
• How do Misfolded Proteins and Aggregates Cause Neurodegeneration? = 249
• How can Protein Misfolding be Targeted? = 253
• Conclusions = 257
• Dedication = 258
• References = 258
• 10. Heat Shock Proteins and Protein Quality Control in Alzheimer's Disease / FRED W. VAN LEEUWEN ; HARM H. KAMPINGA
• General Introduction = 269
• De Novo Folding, Refolding and Degradation : Triaging = 273
• HSP and Proteasomal Degradation = 273
• HSP and Autophagosomal Degradation = 275
• HSP, UPS, and AD = 277
• HSP : Preventing Neurodegenerative Effects of Aβ and Tau = 280
• Perspectives = 289
• Acknowledgments = 289
• References = 289
• 11. Neurodegenerative Diseases and Autophagy / ANGELEEN FLEMING ; MARIELLA VICINANZA ; MAURIZIO RENNA ; CLAUDIA PURI ; THOMAS RICKETTS ; JENS FULLGRABE ; ANA LOPEZ ; SARAH M. DE JAGER ; AVRAHAM ASHKENAZI ; MARIANA PAVEL ; FLORIANA LICITRA ; ANDREA CARICASOLE ; STEPHEN P. ANDREWS ; JOHN SKIDMORE ; DAVID C. RUBINSZTEIN
• Autophagy Cell Biology = 300
• Autophagy in Neurodegenerative Diseases = 309
• Autophagy Upregulation = 321
• Acknowledgments = 325
• References = 325
• Further Reading = 343
• 12. Neurodegenerative Diseases and Axonal transport / LAWRENCE S. GOLDSTEIN ; UTPAL DAS
• Introduction to Axonal Transport = 345
• Axonal Transport and Neurodegenerative Disease = 348
• Alzheimer's Disease = 348
• Huntington's and Other Polyglutamine Diseases = 351
• Spinal and Bulbar Muscular Atrophy = 352
• Hereditary Spastic Paraplegia = 352
• Amyotrophic Lateral Sclerosis = 353
• Charcot - Marie - Tooth Disease = 355
• Parkinson's Disease and Related Synucleinopathies = 356
• Frontotemporal Dementia (FTD) and Related Tauopathies = 357
• Conclusion = 357
• References = 358
• 13. Mitochondrial Function and Neurodegenerative Diseases / HEATHER M. WILKINS ; IAN WEIDLING ; SCOTT KOPPEL ; XIAOWAN WANG ; ALEX VON SCHULZE ; RUSSELL H. SWERDLOW
• Introduction = 369
• Mitochondria and Bioenergetics = 370
• Mitochondria in Neurodegenerative Diseases = 382
• Therapeutic Targeting of Mitochondria = 392
• Conclusions = 394
• Acknowledgment = 395
• References = 395
• 14. Non-cell Autonomous Degeneration : Role of Astrocytes in Neurodegenerative Diseases / SARAH E. SMITH ; AZAD BONNI
• Introduction = 415
• Astrocytes in Amyotrophic Lateral Sclerosis = 419
• Astrocytes in Alzheimer's Disease = 424
• Astrocytes in Parkinson's Disease = 426
• Astrocytes in Huntington's Disease = 428
• Astrocytes in Spinocerebellar Ataxia Type 7 = 430
• Non-cell Autonomous Roles of Astrocytes in Other Diseases = 430
• Perspectives = 431
• Acknowledgments = 433
• References = 433
• 15. Neurodegenerative Diseases and RNA-Mediated Toxicity / TIFFANY W. TODD ; LEONARD PETRUCELLI
• The Identification of RNA-Mediated Toxicity : The Myotonic Dystrophies and CTG Repeats = 441
• RNA Foci and the Sequestration Hypothesis in Other Repeat-Associated Diseases = 446
• Bidirectional Transcription = 454
• Repeat-Associated Non-ATG (RAN) Translation : When RNA Toxicity Results in Proteotoxicity = 457
• Conclusions and Therapeutic Directions = 464
• References = 465
• 16. Neuroinflammation in Age-Related Neurodegenerative Diseases / KATHRYN P. MACPHERSON ; MARIA E. DE SOUSA RODRIGUES ; AMARALLYS F. CINTRON ; MALU G. TANSEY
• Peripheral and Brain-Immune Mediators in Brain Health and Disease = 477
• The Role of Inflammation in Age-Related Neurodegenerative Disease : A Paradigm Shift = 487
• The Role of Peripheral Inflammation in Neurodegenerative Disease = 490
• References = 498
• 17. Neurodegenerative Diseases and the Aging Brain / STEPHEN K. GODIN ; JINSOO SEO ; LI-HUEI TSAI
• General Mechanisms Underlying Neuronal Cell Dysfunction and Cognitive Decline = 509
• Protein Degradation and Synapse Loss = 510
• Unfolded Protein Response = 510
• Ubiquitin - Proteasome = 512
• Autophagy = 513
• Oxidative Damage in the Aging and Neurodegenerating Brain = 515
• DNA Break Repair in the Neurodegenerating Brain = 518
• DNA Damage Reinforces the Metabolic and Gene Expression Changes in the Aging and Neurodegenerating Brain = 519
• Conclusions = 522
• Acknowledgment = 522
• References = 522
• Index = 527