Nervous System Disease Models

Matzner, U., Hartmann, D., Lullmann-Rauch, R., Coenen, R., Rothert, F., Mansson, J. E., Fredman, P., D Hooge, R., De Deyn, P. P. and Gieselmann, V. (2002). Bone marrow stem cell-based gene transfer in a mouse model for metachromatic leukodystrophy Effects on visceral and nervous system disease manifestations. Gene Ther. 9, 53-63. 

Zink MC, Clements JE (2002) A novel simian immunodeficiency virus model tliat provides insiglit into mechanisms of human immunodeficiency virus centi al nervous system disease. J Neurovirol 8(Suppl 2) 42-48. 

Pentylenetetrazol (188) is a drug with profound stimulatory activity on the central nervous system. As such, the agent was at one time used in shock therapy for treatment of mental disease. Although it has since been supplanted by safer methods, the agents still occupy an important role in various experimental animal models in pharmacology. Addition of hydrazine to the imino ether (186) obtained from caprolactam affords 187. Treat-... 

Xanthou G, Wilhams TJ, Pease JE (2003) Molecular characterization of the chemokine receptor CXCR3 evidence for the involvement of distinct extracellular domains in a multi-step model of ligand binding and receptor activation. Eur J Immunol 33 2927-2936 Xia MQ, Hyman BT (1999) Chemokines/chemokine receptors in the central nervous system and Alzheimer s disease. J Neurovirol 5 32-41... 

The human leukodystrophies are inherited disorders of central nervous system white matter. These disorders are characterized by a diffuse deficiency of myelin caused by a variety of genetic lesions and often manifest before 10 years of age (Table 38-1). Some are caused by mutations in the PLP gene and resemble the PLP animal mutants described in Chapter 4 [ 1,23]. As with the animal models, depending on the nature of the mutation, they vary from a severe form in connatal Pelizaeus-Merzbacher disease (PMD) through an intermediate phenotype in classical PMD to a mild phenotype in spastic paraplegia. It is noteworthy that some mutations of the PLP gene also cause a peripheral neuropathy [24], very probably related to the expression of low levels of PLP in peripheral nerve (see Ch. 4). 

Bard, F., Cannon, C., Barbour, R. et al. Peripherally administered antibodies against amyloid beta-peptide enter the central nervous system and reduce pathology in a mouse model of Alzheimer disease. Nature Med. 6 916-919,2000. 

Multiple sclerosis (MS) is the most frequent inflammatory demyeli-nating disease of the central nervous system that affects worldwide about 2.5 million people with no cure. Myelin oligodendrocyte glycoprotein-induced experimental autoimmune encephalomyelitis (MOG-induced EAE) in DA rats is an appropriate model for therapeutic testing, sharing many features with human multiple sclerosis. 

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