Mouse Models in the Study of Genetic Neurological Disorders - Popko, Brian; SpringerLink (Online service) (New York, NY : Springer US : Imprint: Springer, 1999)
1 An Overview of Mouse Models in Neuroscience Research -- 1. Introduction -- 2. Naturally Occurring Mouse Models -- 3. Transgenic Models -- 4. Gene Targeting in Embryonic Stem Cells -- 5. Future Directions -- 6. Summary -- References -- 2 X-Linked Dysmyelination: Mouse Models of Pelizaeus-Merzbacher Disease -- 1. Pelizaeus-Merzbacher Disease and X-Linked Spastic -- Paraplegia -- 2. Myelin Proteolipid Protein (PLP/DM20) -- Acknowledgments -- References -- 3 Charcot-Marie-Tooth Disease: Pathology, Genetics, and Animal Models -- 1. Introduction -- 2. Charcot-Marie-Tooth Disease -- 3. Animal Models for Charcot-Marie-Tooth Disease -- 4. Summary and Outlook -- Acknowledgments -- References -- 4 Mouse Mutations in the Study of Cerebellar Development -- 1. Cerebellar Development -- 2. Cerebellar Mouse Mutants -- 3. Conclusions -- Acknowledgments -- References -- 5 The Role of Neurotrophic Factors in Development and Neurodegenerative Disorders -- 1. Introduction -- 2. Historical Review -- 3. The NGF and GDNF Families of Neurotrophic Factors -- 4. Regulation of Specific Populations of Peripheral Neurons by Neurotrophin and GDNF Family Members -- 5. Hereditary Conditions Associated with Growth Factor Receptor Mutations -- 6. Multiple Trophic Factors Regulate the Development of Some Peripheral Neurons -- 7. Regulation of Survival of CNS Neurons -- 8. Conclusions -- References -- 6 Transgenic Mice with Neurofilament Abnormalities -- 1. Introduction -- 2. Neurofilament Structure and Function -- 3. Transgenic Mouse Models with Neurofilament Accumulations -- 4. Defective Axonal Transport in NF-H Transgenic Mice -- 5. Factors that Can Potentially Induce the Accumulation of Neurofilaments -- 6. A Link between SODI and Neurofilaments? -- 7. Prospects -- Acknowledgments -- References -- 7 Mouse Models of Amyotrophic Lateral Sclerosis -- 1. SOD1 Activity and ALS -- 2. Linking Motor Neuron Growth and Death: Neurofilaments, Axonal Disorganization, and Motor Neuron Disease -- 3. Axonal Transport -- 4. Neurotrophic Factors, Bcl-2, and Apoptosis -- 5. Glutamate Excitotoxicity -- 6. Summary/Conclusions -- References -- 8 Transgenic Mouse Models of Cag Trinucleotide Repeat Neurologic Diseases -- 1. Introduction -- 2. Pathology and Neurological Alteration in Transgenic Mice with Expanded CAG Tracts -- 3. CAG Repeat Instability in Transgenic Mice -- 4. Conclusions and Closing Comments -- References -- 9 Alzheimer's Disease and Genetically Engineered Animal Models -- 1. Introduction -- 2. Alzheimer's Disease -- 3. Principal Risk Factors -- 4. Genetically Engineered Animal Models -- 5. Conclusions -- Acknowledgments -- References -- 10 Model of Genetic Susceptibility to Late-Onset Alzheimer's Disease: Mice Transgenic for Human Apolipoprotein E Alleles -- 1. Genetic Classification of AD -- 2. APOE Susceptibility Gene for AD -- 3. APOE in the Periphery -- 4. APOE in the Central Nervous System -- 5. APOE in the Peripheral Nervous System -- 6. APOE and Pathogenesis of AD-Extracellular Role -- 7. APOE and Pathogenesis of AD-Intracellular Role -- 8. Human Pattern of Neuronal and Glial Localization of APOE -- 9. Apolipoprotein E and Oxidative Injury -- 10. Genetic Models of APOE as a Susceptibility Gene in AD -- 11. APOE Gene Inactivation Model -- 12. Consideration of Genetic Background in Analysis of APOE "Knockouts" and Transgenes -- 13. APOE Transgenic Model with Human Regulatory Sequences -- 14. APOE Transgenic Model with Nonhuman Regulatory Sequences -- 15. APOE Targeted Replacement Animals -- 16. Current Models for the Effect of APOE Alleles on Susceptibility to AD -- 17. Summary/Conclusions -- Acknowledgments -- References -- 11 Lysosomal Disorders -- 1. Introduction -- 2. Naturally Occurring Mouse Models -- 3. Artificially Generated Mouse Models -- 4. Utility of Experimental Animal Models -- 5. On the Horizon -- Acknowledgments -- References -- 12 Neurological Implications of the Genetic Mouse Models for Human Phenylketonuria and Hyperphenylalaninemia -- 1. Introduction -- 2. Earlier Efforts to Produce Mouse PKU Models -- 3. The Genetic PKU Mouse Program -- 4. Future Studies Enabled by the Genetic Mouse Model for -- Human PKU -- References -- 13 Mouse Models of Down Syndrome -- 1. Introduction -- 2. Location of Human Chromosome 21 Genes in the Mouse Genome -- 3. History of Mouse Trisomy Models for DS -- 4. Mental Retardation and Neurological Deficits -- 5. Summary -- Acknowledgments -- References -- 14 Modeling Epileptic Disorders in Mice -- 1. Introduction -- 2. Monogenic Epilepsy Models in Mice -- 3. Multifactorial Epilepsy Models in Mice -- 4. Genetic Models of Spontaneous Lethal Epilepsies and Status Epilepticus -- 5. Mapping Epilepsy Genes in the Human and the Mouse -- Acknowledgments -- References.
Keywords: Neurosciences; Human genetics; Neurosciences; Human Genetics