Mouse Models in the Study of Genetic Neurological Disorders

The number of mouse models that are available for the study of human genetic neurological disorders is large and growing rapidly. Therefore, it was difficult to select the models that were reviewed in this volume. Clearly, there are important models that are not discussed, and perhaps a volume twice this size would have been more appropriate. Moreover, the pace at which new models are being developed and analyzed is rapid. As this volume goes to press, I am sure that additional mouse genes responsible for naturally occurring neurological disorders are being discovered and that many new transgenic and mutant mouse strains are being developed. Therefore, this volume should not be viewed as a comprehensive compendium, but rather as an update of work in progress. It is exhilarating to witness the fast pace at which these models are being established as important tools in the study of basic neuroscience and neurological disorders. It will be even more exciting to see their utilization in the development and testing of therapeutic interventions for these diseases. I would like to thank each of the authors who have contributed to this volume for their time and their expertise. I would also like to thank Drs. Timothy Coetzee and Joshua Corbin for their advice in the selection of the topics covered. I am deeply indebted to Dr. Kunihiko Suzuki, who first approached me with the idea for this volume, for his guidance throughout its preparation.

Contenu

1. An Overview of Mouse Models in Neuroscience Research; B. Popko. 2. X-Linked Dysmyelination: Mouse Models of Pelizaeus-Merzbacher Disease; K.-A. Nave. 3. Charcot-Marie-Tooth Disease: Pathology, Genetics and Animal Models; U. Suter. 4. Mouse Mutations in the Study of Cerebellar Development; D. Karagogeos, O. Pavlou . 5. The Role of Neurotrophic Factors in Development and Neurodegenerative Disorder; W.D. Snider, I. Silos-Santiago. 6. Transgenic Mice with Neurofilament Abnormalities; C. Lampron, et al. 7. Mouse Models of Amyotrophic Lateral Sclerosis; T.L. Williamson, D.W. Cleveland. 8. Transgenic Mouse Models of CAG Trinucleotide Repeat Neurologic Diseases; H.T. Orr, H.Y. Zoghbi. 9. Alzheimer's Disease and Genetically Engineered Animal Models; D.R. Borchelt, et al. 10. Model of Genetic Susceptibility to Late-Onset Alzheimer's Disease: Mice Transgenic for Human Apolipoprotein E Alleles; D.E. Schmechel, et al. 11. Lysosomal Disorders; K. Suzuki, et al. 12. Neurological Implications of the Genetic Mouse Modesl Human Phenylketonuria and Hyperphenylalinemia; J.D. McDonald. 13. Mouse Models of Down Syndrome; M.T. Davisson, A.C. Costa. 14. Modeling Epileptic Disorders in Mice; T.N. Seyfried, et al.