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Glial Abnormalities in Genetically Determined Disorders of Myelin

  • Chapter
Understanding Glial Cells

Abstract

A deficiency of myelin can result either from failure to produce the normal amount of myelin during development (hypomyelination) or from myelin breakdown after it is formed (demyelination). Diseases involving myelin include acquired disorders having an allergic or infectious basis, toxic and nutritional disorders, and genetically determined disorders. A growing number of mutations in protein genes that lead to CNS hypomyelination in mice and other species have been identified and are models of particular value to investigate the structural roles and interactions of proteins in myelin. However, in recent years, most studies in mutant animals are focused on the development and relations of glial cells, since hypomyelination is associated with diverse and complex glial abnormalities affecting differentiation, cell cycle and numbers of glial cells. Oligodendroglial cells show the most severe abnormalities, including oligodendrocyte death. A wide range of reactive alterations in the astrocyte line and in microglial cells have also been described.

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Authors
  1. José Miguel Vela
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  2. Ishar Dalmau
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  3. Berta González
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  4. Bernardo Castellano
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Editors and Affiliations

  1. Department of Cell Biology and Physiology, Universitat Autònoma Barcelona, Spain

    Bernardo Castellano Ph.D.  & Berta González Ph.D.  & 

  2. Cajal Institute, C.S.I.C., Madrid, Spain

    Manuel Nieto-Sampedro Ph.D.

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Vela, J.M., Dalmau, I., González, B., Castellano, B. (1998). Glial Abnormalities in Genetically Determined Disorders of Myelin. In: Castellano, B., González, B., Nieto-Sampedro, M. (eds) Understanding Glial Cells. Springer, Boston, MA. https://doi.org/10.1007/978-1-4615-5737-1_18

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