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The Journal of Neuroscience, January 15, 2000, 20(2):729-735

Immune Deficiency in Mouse Models for Inherited Peripheral Neuropathies Leads to Improved Myelin Maintenance

Christoph D. Schmid^{1, 2}, Martina Stienekemeier¹, Stephan Oehen³, Frank Bootz⁴, Jürgen Zielasek¹, Ralf Gold¹, Klaus V. Toyka¹, Melitta Schachner⁵, and Rudolf Martini^{1, 2}

¹ Department of Neurology, University of Würzburg, D-97080 Würzburg, Germany, ² Department of Neurobiology, Swiss Federal Institute of Technology, CH-8093 Zürich, Switzerland, Institutes of ³ Experimental Immunology and ⁴ Laboratory Animal Science, University of Zürich, CH-8092 Zürich, Switzerland, and ⁵ Zentrum für Molekulare Neurobiologie, University of Hamburg, D-20246 Hamburg, Germany

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 -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.

Key words: Charcot-Marie-Tooth disease; myelin degeneration; Schwann cell; P₀; myelin protein zero; immune system; immune deficiency; T-lymphocytes; macrophages; electron microscopy; electrophysiology

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Copyright © 2000 Society for Neuroscience  0270-6474/00/202729-07$05.00/0

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