Paola S. Timiras, E. Edward Bittar and Mark P. Mattson (Eds.)978-0-7623-0405-9
Genetic Aberrancies and Neurodegenerative Disorders presents critical reviews and emerging findings concerning the roles of genetic mutations and polymorphisms in the pathogenesis of a range of neurological disorders including Alzheimer’s and Parkinson’s diseases, trinucleotide repeat disorders, stroke, epilepsy and others. This volume, written by leading experts, brings together fundamental information concerning the roles of inherited traits in the pathogenesis of different neurodegenerative disorders. In addition to providing a catalogue of the known genetic alterations that are linked to specific neurodegenerative disorders, the chapters detail the current state of understanding of the cellular and biochemical mechanisms whereby the genetic aberrancies lead to neuronal dysfunction and degeneration. The emerging picture of each disorder, painted by pathological, biochemical and molecular brushes, suggests that they share key mechanisms including increased levels of oxidative stress, perturbed ion homeostasis, mitochondrial dysfunction and apoptotic proteolytic cascades. The existence of specific molecular defects provides the opportunity to design experiments that can establish the precise pathogenic mechanism of a specific mutation or genetic risk factor. The value of this approach is exemplified by recent studies of how mutations in Cu/Zn-superoxide dismutase cause amyotrophic lateral sclerosis and how presenilin mutations result in early-onset Alzheimer’s disease. A theme developed among the different chapters is that events that occur during aging predispose neurons to genetic aberrancies that promote degenerative cascades, and that specific genetic defects exert their influence on particular populations of neurons in a disorder-specific manner. The chapters in this volume will stimulate readers to generate new hypotheses concerning the pathogenic mechanisms of genetic aberrancies that can be experimentally tested. |
Table of contents :
Content:
Edited by
Page iii Copyright page
Page iv List of Contributors
Pages vii-ix Preface
Pages xi-xiv
Mark P. Mattson Chapter 1 Genetic Contributions to the Pathogenesis of Alzheimer’s Disease Original Research Article
Pages 1-31
Mark P. Mattson Chapter 2 The Biology of Trinucleotide Repeat Disorders Original Research Article
Pages 33-79
P. Hemachandra Reddy, Danilo A. Tagle Chapter 3 The Genetic Basis and Molecular Pathogenesis of Huntington’s Disease Original Research Article
Pages 81-92
Neil W. Kowall, Stephan Kuemmerle, Robert J. Ferrante Chapter 4 Genetic Abnormalities in Amyotrophic Lateral Sclerosis Original Research Article
Pages 93-133
Edward J. Kasarskis, Daret K.St. Clair Chapter 5 Human Prion Diseases Original Research Article
Pages 135-187
Bernardino Ghetti, Pierluigi Gambetti Chapter 6 Progress in Understanding the Genetics of Epilepsy Original Research Article
Pages 189-241
Carl E. Stafstrom, Asuri N. Prasad, Chitra Prasad, John T. Slevin Chapter 7 Cerebrovascular Disease Original Research Article
Pages 243-286
Laroy Penix, Douglas Lanska Chapter 8 Genetic Susceptibility in Multiple Sclerosiultiple Sclerosis Original Research Article
Pages 287-311
Robert B. Bell Chapter 9 The Role of Mitochondrial Genome Mutations in Neurodegenerative Disease Original Research Article
Pages 313-354
Gordon W. Glazner Chapter 10 Hereditary Disorders of Copper Metabolism Original Research Article
Pages 355-389
Zeynep Tümer, Nina Horn Chapter 11 The Neuronal Ceroid-lipofuscinoses (Batten Disease) Original Research Article
Pages 391-420
R.D. Jolly, A. Kohlschütter, D.N. Palmer, S.U. Walkley Index
Pages 421-428 |
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