Summary
The trembler mouse is a spontaneous mutant showing dominant inheritance. Clinical symptoms become manifest from 10 to 14 days of age as an action tremor affecting the head, neck and limbs, convulsions which decrease with increasing age, and weakness and rigidity of the limbs. Histological examination revealed a normal central nervous system, and a peripheral onion bulb neuropathy similar to hypertrophic interstitial neuropathy in man. Peripheral nerves of 14 day old and adult affected and control animals were examined with the electron microscope. The young mice showed retardation of myelin development, generalized myelin degeneration and early onion bulb development. Adult animals presented a picture almost identical to hypertrophic interstitial neuropathy in man with hypomyelination, segmental demyelination and well-developed inion bulbs, and an increase in endoneurial and perineurial connective tissue.
This animal is presented as a valuable experimental model of hypertrophic interstitial neuropathy in man.
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This investigation was supported by a Research Grant from the National Health and Medical Research Council of Australia.
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Ayers, M.M., Anderson, R.M. Onion bulb neuropathy in the trembler mouse: A model of hypertrophic interstitial neuropathy (Dejerine-Sottas) in man. Acta Neuropathol 25, 54–70 (1973). https://doi.org/10.1007/BF00686858
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DOI: https://doi.org/10.1007/BF00686858