Immune deficiency in mouse models for inherited peripheral neuropathies leads to improved myelin maintenance

Christoph D. Schmid, Martina Stienekemeier, Stephan Oehen, Frank Bootz, Jürgen Zielasek, Ralf Gold, Klaus V. Toyka, Melitta Schachner, Rudolf Martini

Research output: Contribution to journalArticle

110 Scopus citations

Abstract

The adhesive cell surface molecule P0 is the most abundant glycoprotein in peripheral nerve myelin and fulfills pivotal functions during myelin formation and maintenance. Mutations in the corresponding gene cause hereditary demyelinating neuropathies. In mice heterozygously deficient in P0 (P0 (+/-) mice), an established animal model for a subtype of hereditary neuropathies, T-lymphocytes are present in the demyelinating nerves. To monitor the possible involvement of the immune system in myelin pathology, we cross-bred P0 (+/-) mice with null mutants for the recombination activating gene 1 (RAG-1) or with mice deficient in the T-cell receptor α-subunit. We found that in P0 (+/-) mice myelin degeneration and impairment of nerve conduction properties is less severe when the immune system is deficient. Moreover, isolated T-lymphocytes from P0 (+/-) mice show enhanced reactivity to myelin components of the peripheral nerve, such as P0, P2, and myelin basic protein. We hypothesize that autoreactive immune cells can significantly foster the demyelinating phenotype of mice with a primarily genetically based peripheral neuropathy.

Original languageEnglish (US)
Pages (from-to)729-735
Number of pages7
JournalJournal of Neuroscience
Volume20
Issue number2
DOIs
StatePublished - Jan 15 2000

All Science Journal Classification (ASJC) codes

  • Neuroscience(all)

Keywords

  • Charcot-Marie-Tooth disease
  • Electron microscopy
  • Electrophysiology
  • Immune deficiency
  • Immune system
  • Macrophages
  • Myelin degeneration
  • Myelin protein zero
  • P
  • Schwann cell
  • T- lymphocytes

Fingerprint Dive into the research topics of 'Immune deficiency in mouse models for inherited peripheral neuropathies leads to improved myelin maintenance'. Together they form a unique fingerprint.

  • Cite this

    Schmid, C. D., Stienekemeier, M., Oehen, S., Bootz, F., Zielasek, J., Gold, R., Toyka, K. V., Schachner, M., & Martini, R. (2000). Immune deficiency in mouse models for inherited peripheral neuropathies leads to improved myelin maintenance. Journal of Neuroscience, 20(2), 729-735. https://doi.org/10.1523/jneurosci.20-02-00729.2000