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Volume 17, Number 12, Issue of June 15, 1997 pp. 4545-4551
Copyright ©1997 Society for NeuroscienceStructural Abnormalities and Deficient Maintenance of Peripheral Nerve Myelin in Mice Lacking the Gap Junction Protein Connexin 32
Received Jan. 27, 1997; revised March 19, 1997; accepted March 31, 1997.
Patrizia Anzini1, Dirk H.-H. Neuberg2, Melitta Schachner1, 3, Eric Nelles4, Klaus Willecke4, Jürgen Zielasek5, Klaus V. Toyka5, Ueli Suter2, and Rudolf Martini1, 5 Departments of 1 Neurobiology and 2 Cell Biology, Swiss Federal Institute of Technology, CH-8093 Zurich, Switzerland, 3 Institute of Biosynthesis of Neural Structures, Center for Molecular Neurobiology Hamburg, University of Hamburg, D-20246 Hamburg, Germany, 4 Institute of Genetics, University of Bonn, D-53117 Bonn, Germany, and 5 Department of Neurology, University of Würzburg, D-97080 Würzburg, Germany
Mutations affecting the connexin 32 (Cx32) gene are associated with the X-linked form of the hereditary peripheral neuropathy Charcot-Marie-Tooth disease (CMTX). We show that Cx32-deficient mice develop a late-onset progressive peripheral neuropathy with abnormalities comparable to those associated with CMTX, thus providing proof of the critical role of Cx32 in the maintenance of peripheral nerve myelin and an animal model for CMTX. Frequently observed features include abnormally thin myelin sheaths, cellular onion bulb formation reflecting myelin degeneration-induced Schwann cell proliferation, and enlarged periaxonal collars while nerve conductance properties are altered only slightly. These observations are consistent with earlier hypotheses suggesting a function of Cx32 as a channel-forming protein that facilitates the communication between the abaxonal and adaxonal aspects of Schwann cell cytoplasm.
Key words: connexin 32; myelin; Schwann cell; demyelination; Charcot-Marie-Tooth disease; transgenic mouse
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