Contributors |
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xi | |
Preface |
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xiii | |
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Pathologic Lesions in Neurodegenerative Diseases |
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1 | (40) |
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2 | (21) |
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II Parkinsonian Signs and Symptoms ("Parkinsonism"), PD, and Diffuse Lewy Body Disease |
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23 | (5) |
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III Frontotemporal Lobar Degeneration(s) |
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28 | (4) |
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IV Other (Miscellaneous) Disorders |
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32 | (1) |
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V Conclusion and Future Directions |
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33 | (8) |
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33 | (1) |
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33 | (8) |
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Cerebral Amyloid Angiopathy |
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41 | (38) |
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42 | (1) |
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II Clinical Aspects of CAA |
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42 | (14) |
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III Molecular Aspects of CAA |
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56 | (9) |
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65 | (14) |
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67 | (1) |
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68 | (11) |
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The Genetics of Alzheimer's Disease |
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79 | (22) |
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80 | (4) |
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II Early-Onset Familial AD with Mendelian Transmission |
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84 | (3) |
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III Late-Onset AD Without Mendelian Transmission |
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87 | (7) |
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IV Outlook for Future Genetics Studies of AD |
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94 | (1) |
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95 | (6) |
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96 | (5) |
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Alzheimer's Disease and the Amyloid β-Protein |
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101 | (24) |
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I Clinical Features of Alzheimer's Disease |
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101 | (1) |
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II Genetics and Molecular Biology of Alzheimer's Disease |
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102 | (4) |
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III The Amyloid Cascade Hypothesis |
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106 | (1) |
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IV The Amyloid β-Protein--Lessons from In Vitro Studies |
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107 | (4) |
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V The Amyloid β-Protein--Lessons from the Human Brain |
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111 | (2) |
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VI Testing the Amyloid Hypothesis in Humans |
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113 | (12) |
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115 | (1) |
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115 | (10) |
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Molecular Insights into Parkinson's Disease |
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125 | (64) |
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126 | (1) |
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II Role of αSynuclein Aggregation in PD |
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127 | (21) |
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III Role of Mitochondrial Dysfunction in PD |
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148 | (12) |
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IV Convergent Pathways and Future Directions |
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160 | (29) |
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162 | (1) |
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162 | (27) |
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Huntington Disease and the Huntingtin Protein |
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189 | (26) |
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190 | (1) |
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II Genetic and Clinical Features |
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190 | (2) |
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III Structural Features of the Huntingtin Protein |
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192 | (1) |
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193 | (2) |
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V Animal Models of Huntington Disease |
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195 | (2) |
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197 | (4) |
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VII Therapeutic Targets for HD |
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201 | (3) |
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204 | (11) |
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205 | (10) |
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The Complex Molecular Biology of Amyotrophic Lateral Sclerosis (ALS) |
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215 | (48) |
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I ALS Is a Deadly Neurodegenerative Disorder |
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216 | (1) |
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217 | (2) |
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III SOD1-Related Pathology as a General Model for ALS |
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219 | (1) |
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IV Misfolding and Aggregation Is the Most Likely Source of SOD1 Toxicity |
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219 | (8) |
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V Motor Neuron Death in ALS: Apoptotic Versus Necrotic, Cell-Autonomous Versus Non-Cell-Autonomous |
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227 | (3) |
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VI ALS Comprises a Spectrum of Pathologies |
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230 | (11) |
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241 | (22) |
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242 | (21) |
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263 | (32) |
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264 | (2) |
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II Tau in Neurodegenerative Disease |
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266 | (2) |
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III Interactions with the Cytoskeleton |
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268 | (4) |
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IV Phosphorylation and Other Post-translational Modifications |
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272 | (3) |
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275 | (4) |
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279 | (16) |
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279 | (16) |
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Membrane Pores in the Pathogenesis of Neurodegenerative Disease |
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295 | (32) |
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296 | (1) |
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II Aggregation and the β-Sheet Conformation |
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297 | (2) |
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299 | (4) |
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303 | (2) |
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305 | (2) |
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VI AG Triplet Repeat Diseases/Huntington's |
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307 | (1) |
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VII Amyotrophic Lateral Sclerosis/Superoxide Dismutase |
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308 | (1) |
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VIII How Channels Damage Neurons |
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309 | (3) |
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312 | (2) |
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X β-Sheet Conformation and Amyloid Peptide Channels |
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314 | (2) |
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316 | (11) |
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319 | (1) |
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320 | (7) |
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Protein Quality Control in Neurodegenerative Disease |
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327 | (28) |
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I Overview of Protein Quality Control |
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328 | (9) |
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II Proteotoxic Proteins and Quality Control |
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337 | (5) |
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III Reflections and Prospectus |
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342 | (13) |
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346 | (9) |
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Biology of Mitochondria in Neurodegenerative Diseases |
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355 | (62) |
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357 | (2) |
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II Some Aspects of Mitochondrial Biology Relevant to Neurodegeneration |
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359 | (4) |
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III Mitochondria and Cell Death |
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363 | (10) |
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IV Mitochondrial Autophagy |
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373 | (1) |
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V Mitochondrial Fission and Fusion |
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374 | (1) |
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VI Mitochondrial Involvement in Adult-Onset Neurodegenerative Diseases |
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374 | (22) |
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396 | (21) |
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396 | (1) |
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396 | (21) |
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417 | (40) |
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418 | (1) |
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II Establishing the Existence of Prions in Fungi |
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419 | (4) |
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III The Prions of Yeast and Their Cellular Roles |
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423 | (5) |
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IV Propagating the [ PRION+] State |
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428 | (8) |
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V Yeast Prion Variants and Phenotypic Variability |
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436 | (3) |
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VI Fungal Prions: Friend or Foe? |
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439 | (4) |
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VII Yeast Prions for Therapeutic Discovery |
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443 | (3) |
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446 | (11) |
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447 | (1) |
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447 | (10) |
Index |
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457 | |