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Progress in molecular biology and translational science. Volume 107, Molecular biology of neurodegenerative diseases / edited by David B. Teplow. — Amsterdam : Elsevier, c2012. – (58.178/P964/v.107) |
Contents
Contents
Contributors
Preface
Pathologic Lesions in Neurodegenerative Diseases
I. Introduction
II. Parkinsonian Signs and Symptoms ("Parkinsonism")， PD， and Diffuse Lewy Body Disease
III. Frontotemporal Lobar Degeneration(s)
IV. Other (Miscellaneous) Disorders
V. Conclusion and Future Directions
Acknowledgments
References
Cerebral Amyloid Angiopathy 41
I. Introduction
II. Clinical Aspects of CAA
III. Molecular Aspects of CAA
IV. Reflections
Acknowledgments
References
The Genetics of Alzheimer's Disease 79
I. Introduction
II. Early-Onset Familial AD with Mendelian Transmission
III. Late-Onset AD 'Without Mendelian Transmission
IV. Outlook for Future Genetics Studies of AD
V. Conclusion
References
Alzheimer's Disease and the Amyloid 13-Protein
I. Clinical Features of Alzheimer's Disease
II. Genetics and Molecular Biology of Alzheimer's Disease
III. The Amyloid Cascade Hypothesis
IV. The Amyloid 13-Protein--Lessons from In Vitro Studies
V. The Amyloid 13-Protein--Lessons from the Human Brain
VI. Testing the Amyloid Hypothesis in Humans
Acknowledgment
References
Molecular Insights into Parkinson's Disease 125
I. Introduction
II. Role of αSynuelein Aggregation in PD
III. Role of Mitochondrial Dysfunction in PD
IV. Convergent Pathways and Future Directions
Acknowledgments
References
Huntington Disease and the Huntingtin Protein 189
I. Introduction
II. Genetic and Clinical Features
III. Structural Features of the Huntingtin Protein
IV. Htt Function(s)
V. Animal Models of Huntington Disease
VI. Pathogenesis of HD
VII. Therapeutic Targets for HD
VIII. Conclusion
References
The Complex Molecular Biology of Amyotrophic Lateral Sclerosis (ALS) 215
I. ALS Is a Deadly Neurodegenerative Disorder 216
II. Etiology of ALS 217
III. SOD1-Related Pathology as a General Model for ALS 219
IV. Misfolding and Aggregation Is the Most Likely Source of SOD1 Toxicity
V. Motor Neuron Death in ALS: Apoptotic Versus Necrotic， Cell-Autonomous Versus Non-Cell-Autonomous
VI. ALS Comprises a Spectrum of Pathologies
VII. Concluding Remarks
References
Tau and Tauopathies 263
I. Tau Gene and Isoforms 264
II. Tau in Neurodegenerative Disease 266
III. Interactions with the Cytoskeleton 268
IV. Phosphorylation and Other Post-translational Modifications 272
V. Other Interactions 275
VI. Reflections 279
References 279
Membrane Pores in the Pathogenesis of Neurodegenerative Disease 295
I. Introduction
II. Aggregation and the β-Sheet Conformation
III. Aβ
IV. Prion Channels
V. α-Synuclein
VI. AG Triplet Repeat Diseases/Huntington's
VII. Amyotrophic Lateral Selerosis/Superoxide Dismutase.
VIII. How Channels Damage Neurons
IX. Molecular Pore Models
X. β-Sheet Conformation and Amyloid Peptide Channels
XI. Reflections
Acknowledgments
References
Protein Quality Control in Neurodegenerative Disease 327
I. Overview of Protein Quality Control 328
II. Proteotoxic Proteins and Quality Control
III. Reflections and Prospectus
References
Biology of Mitochondria in Neurodegenerative Diseases 355
I. Introduction
II. Some Aspects of Mitoehondrial Biology Relevant to Neurodegeneration
III. Mitochondria and Cell Death
IV. Mitochondrial Autophagy
V. Mitoehondrial Fission and Fusion
VI. Mitoehondrial Involvement in Adult-Onset Neurodegenerative Diseases
VII. Reflections
Acknowledgments
References
Fungal Prions 417
I. Introduction
II. Establishing the Existence of Prions in Fungi
III. The Prions of Yeast and Their Cellular Boles
IV. Propagating the [PBION+] State
V. Yeast Prion Variants and Phenotypie Variability
VI. Fungal Prions: Friend or Foe?
VII. Yeast Prions for Therapeutic Discovery
VIII. Reflections
Acknowledgments
References
Index
