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Molecular Biology of Neurodegenerative Diseases, Volume 107 [Hardback]

Volume editor (Department of Neurology, David Geffen School of Medicine, UCLA, Los Angeles, CA, USA)
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Neurodegenerative diseases result in progressive degeneration and / or death of nerve cells which leads to problems with movement and mental functioning. Examples include Parkinson’s, Alzheimer’s and Huntington’s disease. Much research is taking place to try to identify ways to prevent or lessen the impact of these diseases.

This volume reviews the latest research and developments in the molecular biology of neurodegenerative diseases.

* Discusses new discoveries, approaches, and ideas * Contributions from leading scholars and industry experts * Reference guide for researchers involved in molecular biology and related fields



Neurodegenerative diseases result in progressive degeneration and / or death of nerve cells which leads to problems with movement and mental functioning. Examples include Parkinson’s, Alzheimer’s and Huntington’s disease. Much research is taking place to try to identify ways to prevent or lessen the impact of these diseases.

This volume reviews the latest research and developments in the molecular biology of neurodegenerative diseases.



Key features:

* Contributions from leading authorities * Informs and updates on all the latest developments in the field

Recenzijas

Praise for the series: "Full of interest not only for the molecular biologist--for whom the numerous references will be invaluable--but will also appeal to a much wider circle of biologists, and in fact to all those who are concerned with the living cell." --British Medical Journal

Papildus informācija

Written by research experts, this volume of Progress in Molecular Biology and Translational Science focuses on current science surrounding molecular biology of neurodegenerative diseases
Contributors xi
Preface xiii
Pathologic Lesions in Neurodegenerative Diseases
1(40)
Paul M. Thompson
Harry V. Vinters
I Introduction
2(21)
II Parkinsonian Signs and Symptoms ("Parkinsonism"), PD, and Diffuse Lewy Body Disease
23(5)
III Frontotemporal Lobar Degeneration(s)
28(4)
IV Other (Miscellaneous) Disorders
32(1)
V Conclusion and Future Directions
33(8)
Acknowledgments
33(1)
References
33(8)
Cerebral Amyloid Angiopathy
41(38)
Masahito Yamada
Hironobu Naiki
I Introduction
42(1)
II Clinical Aspects of CAA
42(14)
III Molecular Aspects of CAA
56(9)
IV Reflections
65(14)
Acknowledgments
67(1)
References
68(11)
The Genetics of Alzheimer's Disease
79(22)
Lars Bertram
Rudolph E. Tanzi
I Introduction
80(4)
II Early-Onset Familial AD with Mendelian Transmission
84(3)
III Late-Onset AD Without Mendelian Transmission
87(7)
IV Outlook for Future Genetics Studies of AD
94(1)
V Conclusion
95(6)
References
96(5)
Alzheimer's Disease and the Amyloid β-Protein
101(24)
Dominic M. Walsh
David B. Teplow
I Clinical Features of Alzheimer's Disease
101(1)
II Genetics and Molecular Biology of Alzheimer's Disease
102(4)
III The Amyloid Cascade Hypothesis
106(1)
IV The Amyloid β-Protein--Lessons from In Vitro Studies
107(4)
V The Amyloid β-Protein--Lessons from the Human Brain
111(2)
VI Testing the Amyloid Hypothesis in Humans
113(12)
Acknowledgment
115(1)
References
115(10)
Molecular Insights into Parkinson's Disease
125(64)
Jean-Christophe Rochet
Bruce A. Hay
Ming Guo
I Introduction
126(1)
II Role of αSynuclein Aggregation in PD
127(21)
III Role of Mitochondrial Dysfunction in PD
148(12)
IV Convergent Pathways and Future Directions
160(29)
Acknowledgments
162(1)
References
162(27)
Huntington Disease and the Huntingtin Protein
189(26)
Zhiqiang Zheng
Marc I. Diamond
I Introduction
190(1)
II Genetic and Clinical Features
190(2)
III Structural Features of the Huntingtin Protein
192(1)
IV Htt Function(s)
193(2)
V Animal Models of Huntington Disease
195(2)
VI Pathogenesis of HD
197(4)
VII Therapeutic Targets for HD
201(3)
VIII Conclusion
204(11)
References
205(10)
The Complex Molecular Biology of Amyotrophic Lateral Sclerosis (ALS)
215(48)
Rachel L. Redler
Nikolay V. Dokholyan
I ALS Is a Deadly Neurodegenerative Disorder
216(1)
II Etiology of ALS
217(2)
III SOD1-Related Pathology as a General Model for ALS
219(1)
IV Misfolding and Aggregation Is the Most Likely Source of SOD1 Toxicity
219(8)
V Motor Neuron Death in ALS: Apoptotic Versus Necrotic, Cell-Autonomous Versus Non-Cell-Autonomous
227(3)
VI ALS Comprises a Spectrum of Pathologies
230(11)
VII Concluding Remarks
241(22)
References
242(21)
Tau and Tauopathies
263(32)
Gloria Lee
Chad J. Leugers
I Tau Gene and Isoforms
264(2)
II Tau in Neurodegenerative Disease
266(2)
III Interactions with the Cytoskeleton
268(4)
IV Phosphorylation and Other Post-translational Modifications
272(3)
V Other Interactions
275(4)
VI Reflections
279(16)
References
279(16)
Membrane Pores in the Pathogenesis of Neurodegenerative Disease
295(32)
Bruce L. Kagan
I Introduction
296(1)
II Aggregation and the β-Sheet Conformation
297(2)
III Aβ
299(4)
IV Prion Channels
303(2)
V α-Synuclein
305(2)
VI AG Triplet Repeat Diseases/Huntington's
307(1)
VII Amyotrophic Lateral Sclerosis/Superoxide Dismutase
308(1)
VIII How Channels Damage Neurons
309(3)
IX Molecular Pore Models
312(2)
X β-Sheet Conformation and Amyloid Peptide Channels
314(2)
XI Reflections
316(11)
Acknowledgments
319(1)
References
320(7)
Protein Quality Control in Neurodegenerative Disease
327(28)
Jason E. Gestwicki
Dan Garza
I Overview of Protein Quality Control
328(9)
II Proteotoxic Proteins and Quality Control
337(5)
III Reflections and Prospectus
342(13)
References
346(9)
Biology of Mitochondria in Neurodegenerative Diseases
355(62)
Lee J. Martin
I Introduction
357(2)
II Some Aspects of Mitochondrial Biology Relevant to Neurodegeneration
359(4)
III Mitochondria and Cell Death
363(10)
IV Mitochondrial Autophagy
373(1)
V Mitochondrial Fission and Fusion
374(1)
VI Mitochondrial Involvement in Adult-Onset Neurodegenerative Diseases
374(22)
VII Reflections
396(21)
Acknowledgments
396(1)
References
396(21)
Fungal Prions
417(40)
Gemma L. Staniforth
Mick F. Tuite
I Introduction
418(1)
II Establishing the Existence of Prions in Fungi
419(4)
III The Prions of Yeast and Their Cellular Roles
423(5)
IV Propagating the [ PRION+] State
428(8)
V Yeast Prion Variants and Phenotypic Variability
436(3)
VI Fungal Prions: Friend or Foe?
439(4)
VII Yeast Prions for Therapeutic Discovery
443(3)
VIII Reflections
446(11)
Acknowledgments
447(1)
References
447(10)
Index 457
David B. Teplow, Ph.D., is a Professor of Neurology, Emeritus, at UCLA and an internationally recognized leader in efforts to understand and treat Alzheimer's disease. Dr. Teplow's group has used a multi-disciplinary approach to determine how neurotoxic peptides, such as the amyloid -protein (Alzheimer's disease) and -synuclein (Parkinsons disease), form neurotoxic structures that kill neurons and to develop the means to block these processes. Dr. Teplow received undergraduate training at UC Berkeley; a Ph.D. from the University of Washington; and was a postdoctoral scholar at Caltech. Before coming to UCLA, Dr. Teplow was a faculty member in the Department of Neurology, Harvard Medical School. Dr. Teplow has published >250 peer-reviewed articles, books and book chapters, and commentaries, in addition to serving on numerous national and international scientific advisory boards. Dr. Teplow was a founding editor of the Journal of Molecular Neuroscience and Current Chemical Biology, He is Co-Editor-in-Chief of the Elsevier serial Progress in Molecular Biology and Translational Science and is Associate Editor-in-Chief of the American Journal of Neurodegenerative Disease.