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Murine mutations affecting myelination: models to study myelin diseases in the human

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Inborn Errors of Metabolism in Humans

Abstract

The myelin sheath (Figure 13.1) is a modified and highly specialized plasma membrane which is wrapped around a portion of the axon like a spiral. The myelin membrane is an extension of the oligodendrocyte plasma membrane in the central nervous system (CNS) and of the Schwann-cell plasma membrane in the peripheral nervous system (PNS). During the process of myelin formation, the cytoplasm is extruded and the cellular leaflets are fused to form the major dense line (Figure 13.2). The external surfaces come together and form the myelin intraperiod line (also called minor dense line, Figure 13.2).

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Authors
  1. J.-M. Matthieu
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Editors and Affiliations

  1. University Department of Child Health, Royal Hospital for Sick Children, Yorkhill, Glasgow, G3 8SJ, UK

    Forrester Cockburn

  2. Division of Metabolism, University Paediatric Department, Kinderspital, 8032, Zurich, Switzerland

    Richard Gitzelmann

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© 1982 The Society for the Study of Inborn Errors of Metabolism

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Matthieu, JM. (1982). Murine mutations affecting myelination: models to study myelin diseases in the human. In: Cockburn, F., Gitzelmann, R. (eds) Inborn Errors of Metabolism in Humans. Springer, Dordrecht. https://doi.org/10.1007/978-94-009-7325-1_13

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  • DOI: https://doi.org/10.1007/978-94-009-7325-1_13

  • Publisher Name: Springer, Dordrecht

  • Print ISBN: 978-94-009-7327-5

  • Online ISBN: 978-94-009-7325-1

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