Mouse Models in the Study of Genetic Neurological Disorders

Mouse Models in the Study of Genetic Neurological Disorders

Bibliographic Details
Corporate Author: SpringerLink (Online service)
Other Authors: Popko, Brian. (Editor)
Format: eBook
Language:English
Published: New York, NY : Springer US : Imprint: Springer, 1999.
Edition:1st ed. 1999.
Series:Advances in Neurochemistry ; 9
Subjects:
Neurosciences.
Human genetics.
Human Genetics.
Online Access:https://doi.org/10.1007/978-1-4615-4887-4
Table of Contents:
  • 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.

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