Abstract
Hypertrophic neuropathy is a standard pathological reaction of peripheral nerve that occurs in different diseases. On transverse sections, it is characterized by onion-bulb disposition of proliferated Schwann cells around nerve fibers. These hypertrophic onion-bulb changes were first recognized in the hereditary neuropathies. Until recently, they were considered specific for Dejerine-Sottas disease, but from clinical and experimental studies they are known to be the non-specific consequence of Schwann cell proliferation resulting from repeated segmental demyelination and remyelination.1
This is a preview of subscription content, log in via an institution to check access.
Access this chapter
Tax calculation will be finalised at checkout
Purchases are for personal use only
Preview
Unable to display preview. Download preview PDF.
References
P.K. Thomas, Hereditary Neuropathies, Trends Neurosci. 4:X (1981).
D. Falconer, Two new mutants, Trembler and reeler, with neurological action in the house mouse (Mus Musculus L), J. Genet. 50:192 (1951).
M. Ayers, R. Anderson, Onion bulb neuropathy in the Trembler mouse: a model of hypertrophic interstitial neuropathy (Dejerine-Sottas) in man, Acta Neuropath. (Berl.) 25:54 (1973).
P. A. Low, J. G. McLeod, Hereditary demyelinating neuropathy in the Trembler mouse, J. Neurol. Sci. 26:565 (1975).
A. J. Aguayo, M. Attiwell, J. Trecarten, S. Perkins, G. M. Bray, Abnormal myelination in transplanted Trembler mouse Schwann cells, Nature, Lon. 265:73 (1977).
C. S. Perkins, A. J. Aguayo and G. M. Bray, Schwann cell multiplication in Trembler mice, Neuropath. and Applied Neurobiol. 7:115 (1981).
C. S. Perkins, A. J. Aguayo and G. M. Bray, Behavior of Schwann cells from Trembler mouse unmyelinated fibers transplanted into myelinated nerves, Exp. Neurobiol. 71:515 (1981).
A. J. Yates and J. R. Wherrett, Changes in the sciatic nerve of the rabbit and its tissue constituents during development, J. Neurochem. 23:993 (1974).
F. Klein and P. Mandel, Gangliosides of the peripheral nervous system of the rat, Life Sci. 16:751 (1975).
J. W. Fong, R. W. Ledeen, S. K. Kundu and S. W. Brostoff, Gangliosides of peripheral nerve myelin, J. Neurochem. 26:157 (1976).
A. J. Yates and D. K. Thompson, Ganglioside composition of peripheral nerve undergoing Wallerian degeneration, J. Neurochem. 30:1649 (1978).
K. H. Chou, C. E. Nolan and F. B. Jungalwala, Composition and metabolism of gangliosides in rat peripheral nervous system during development, J. Neurochem. 39:1547 (1982).
J. H. Hofteig, J. R. Mendell and A. J. Yates, Chemical and morphological studies on garfish peripheral nerves, J. Comp. Neur. 198:265 (1981).
J. I. Morgan and W. Seifert, Growth factors and gangliosides: a possible new perspective in neuronal growth control, J. Supramol. Struct. 10:111 (1979).
D. P. Purpura and H. J. Baker, Neurite induction in mature cortical neurons in feline GM1-ganglioside storage disease, Nature, 266:553 (1977).
M. Willinger and M. Schachner, GM1 gangliosie as a marker for neuronal differentiation in mouse cerebellum, Dev. Biol. 74:101 (1980).
J. J. Hauw, S. Fenelon, J. M. Boutry and R. Escourolle, Effet des gangliosides sur la croissance de ganglion spinal de cobaye en culture in vitro. Resultats preliminaires concernant une preparation de gangliosides de cortex cerebral de boeuf, C. R. Acad. Sci. 292:569 (1981).
F. J. Roisen, H. Bertfeld, R. Nagele and G. Yorke, Ganglioside stimulation of axonal sprouting in vitro, Science, 214:577 (1981).
M. M. Rapport, A. Gorio, eds., Gangliosides in Neurological and Neuromuscular function, Development and Repair, Raven Press, New York (1981).
P. Mandel, P. Dreyfus, H. Yusufi, AN. N. K. Sarlieve, L. Robert, J. Neskovic, N. Harth, S. and G. Rebel, Neuronal and glial cell cultures, a tool for investigation of ganglioside function, Adv. Exp. Med. Biol. 125:515 (1980).
M. L. Harpin, J. Portoukalian and N. Baumann, Modifications of ganglioside composition in peripheral nerve of myelin-deficient Trembler mutant mouse, Neurochem. Res. 7:1367 (1982).
F. Lachapelle, B. Zalc, N. Baumann and J. L. Guenet, “Production and use of genetically uniform dysmyelinating mutants of the mouse,” in: Neurological Mutations Affecting Myelination, N. Baumann, ed., Elsevier/North-Holland, Amsterdam, (1980).
S. Pollet, S. Ermidou, F. Le Saux, M. Monge and N. Baumann, Microanalysis of brain lipids: multiple two-dimensional thin-layer chromatography, J. Lipid Res. 19:916 (1978).
J. Folch, M. Lees and G. H. Sloane-Stanley, Method for the isolation and purification of total lipids from animal tissues, J. Biol. Chem. 226:497 (1957).
M. A. Williams and R. H. McCluer, The use of Sep-Pak C18 cartridges during the isolation of gangliosides, J. Neurochem. 35:266 (1980).
H. Dreyfus, P. F. Urban, P. Bosch, S. Edel-Harth, G. Rebel and P. Mandel, Effect of light on gangliosides from calf retina and photoreceptors, J. Neurochem. 22:1073 (1974).
L. Svennerholm, “Ganglioside metabolism,” in: Comprehensive Biochemistry, M. Florkin and E. H. Stotz eds., Elsevier/North-Holland, Amsterdam, (1970).
Proceedings of the VIth International Symposium on Glycoconjugates, T. Yamakawa, T. Osawa, S. Handa, Japan Society, Soc. Press, Tokoyo, (1981).
J. L. Magnani, B. Nilsson, M. Brockhaus, D. Zopf, Z. Steplewski, H. Koprowski and V. Ginsburg, A monoclonal antibody-defined antigen associated with gastrointestinal cancer is a ganglioside containing sialylated lacto-N-fucopentose II. J. Biol. Chem. 257:14365 (1982).
R. Hawkes, E. Niday and J. Gordon, A dot-immunobinding assay for monoclonal and other antibodies, Anal. Biochem. 119:142 (1982).
V. P. Skipski and M. Barkley, in: Methods in Enzymology, J. M. Lowenstein ed., 14:545 (1969).
Author information
Authors and Affiliations
Editor information
Editors and Affiliations
Rights and permissions
Copyright information
© 1984 Plenum Press, New York
About this chapter
Cite this chapter
Harpin, ML., Portoukalian, J., Zalc, B., Baumann, N. (1984). Ganglioside Alterations in the Genetically-Determined Hypertrophic Neuropathy of the Murine Neurological Mutant Trembler. In: Ledeen, R.W., Yu, R.K., Rapport, M.M., Suzuki, K. (eds) Ganglioside Structure, Function, and Biomedical Potential. Advances in Experimental Medicine and Biology, vol 174. Springer, Boston, MA. https://doi.org/10.1007/978-1-4684-1200-0_25
Download citation
DOI: https://doi.org/10.1007/978-1-4684-1200-0_25
Publisher Name: Springer, Boston, MA
Print ISBN: 978-1-4684-1202-4
Online ISBN: 978-1-4684-1200-0
eBook Packages: Springer Book Archive