Animal Models of Genetic Disorders of Myelin

Hogan, Edward L.; Greenfield, Seymour

doi:10.1007/978-1-4757-1830-0_14

Animal Models of Genetic Disorders of Myelin

Edward L. Hogan² &
Seymour Greenfield²

Chapter

281 Accesses
75 Citations

Abstract

Improved understanding of the assembly and maintenance of myelin is afforded by the study of animals in which interference with these processes occurs as a result of genetic variation, toxic exposure, immunological attack, viral infection, nutritional or endocrine deficiency, and physical or circulatory insult. Mutant animals are particularly suitable for the biochemical approach, since they make it possible to obtain genetic homogeneity, sufficient quantities for experimental use, and strict control of their nutrition and environment. During the past decade, considerable work has been carried out on several mouse mutants with genetic defects in and limited to the formation of myelin. The first recognized were the jiutpy, quaking, and myelin synthesis deficiency mutants, and study of them contributed importantly to the identification of myelin constituents and clarification of major features of myelin metabolism and myelinogenesis. In recent years, several additional mouse mutants have been described and shown to have myelin disorders with special characteristics that further reveal the biology of myelin. The most notable of these are shiverer, which has an almost complete lack of myelin basic protein; twitcher, which resembles human globoid cell leukodystm ophy in having a severe deficiency of galactosylceramide ß-galactosidase; trembler, which has a primary disorder of peripheral nerve and Schwann cell; and muscular dystrophy, which has amyelinated segments of nerve roots. All these mutants share advantages for experimental study in that the pathology is limited to the myelin, their genetic nature presages likely abnormality of protein structure, serial developmental analysis is possible, and dilfereuees in both phenotype and genotype of myelin disorders have been shown.

Keywords

Sciatic Nerve
Schwann Cell
Myelin Basic Protein
Myelin Protein
Organotypic Culture

These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.

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Medical University of South Carolina, Charleston, 29425, South Carolina, USA
Edward L. Hogan & Seymour Greenfield

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Edward L. Hogan
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University of North Carolina at Chapel Hill, Chapel Hill, North Carolina, USA
Pierre Morell

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Hogan, E.L., Greenfield, S. (1984). Animal Models of Genetic Disorders of Myelin. In: Morell, P. (eds) Myelin. Springer, Boston, MA. https://doi.org/10.1007/978-1-4757-1830-0_14

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DOI: https://doi.org/10.1007/978-1-4757-1830-0_14
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