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Eur J Neurosci. 1995 Mar 1;7(3):511-5.

Crucial role for the myelin-associated glycoprotein in the maintenance of axon-myelin integrity.

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Department of Neurobiology, Swiss Federal Institute of Technology, Hönggerberg, Zurich.


It has recently been shown that mice deficient in the gene for myelin-associated glycoprotein develop normal myelin sheaths in the peripheral nervous system. Here we report that in mutant mice older than 8 months the maintenance of axon-myelin units is disturbed, resulting in both axon and myelin degeneration. Morphological features include those typically seen in human peripheral neuropathies, where demyelination-induced Schwann cell proliferation and remyelination lead to the formation of so-called onion bulbs. Expression of tenascin-C, a molecule indicative of peripheral nerve degeneration, was up-regulated by axon-deprived Schwann cells and regenerating axons were occasionally seen. Myelin-associated glycoprotein thus appears to play a crucial role in the long-term maintenance of the integrity of both myelin and axons.

[Indexed for MEDLINE]

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