Hostname: page-component-84b7d79bbc-fnpn6 Total loading time: 0 Render date: 2024-07-27T17:45:45.215Z Has data issue: false hasContentIssue false
  • English
  • Français

Amino Acid Changes in the Mouse Mutant Dystonia Musculorum Similar to Those in Friedreich’s Ataxia

Published online by Cambridge University Press:  18 September 2015

A. Messer
A. Messer*
Affiliation:
Birth Defects Institute, Center for Laboratories and Research, New York State Department of Health, Albany, New York
Rights & Permissions [Opens in a new window]

Summary:

Core share and HTML view are not available for this content. However, as you have access to this content, a full PDF is available via the ‘Save PDF’ action button.

An autosomal recessive mutant strain of mouse with a progressive neurological disorder is described. Histopathology is dramatic in the sensory afferents and in the red nucleus. In the cerebellar vermis the concentrations of glutamate, aspartate, glycine and GABA are significantly reduced, and in the cerebellar hemispheres the taurine/glutamate ratio is elevated. These mice may provide a useful experimental model of Friedreich’s ataxia.

Type
Research Article
Information
Canadian Journal of Neurological Sciences , Volume 9 , Issue 2 , May 1982 , pp. 185 - 188
Copyright
Copyright © Canadian Neurological Sciences Federation 1982

References

REFERENCES

Butterworth, R.F., Hamel, E., Landerville, F. and Barbeau, A. (1978). Cerebellar ataxia produced by 3-acetyl pyridine in rat. Can. J. Neurol. Sci., 5, 131–133.CrossRefGoogle ScholarPubMed
Butterworth, R.F., Hamel, E., Landerville, F. and Barbeau, A. (1979). Amino acid changes in thiamine deficient encephalopathy: some implications for the pathogenesis of Friedreich’s ataxia. Can. J. Neurol. Sci., 6, 217–222.CrossRefGoogle ScholarPubMed
Duchen, L.W., Strich, S.T. and Falconer, D.S. (1964). Clinical and pathological studies of an hereditary neuropathy in mice (dystonia musculorum). Brain, 87, 367–378.CrossRefGoogle ScholarPubMed
Hanker, J.S. and Peach, R.Histochemical and ultrastructural studies of primary sensory neurones in mice with dystonia musculorum. Neuropath. Appl. Neurobiol., 2, 79–97.CrossRefGoogle Scholar
Hughes, T.J., Brownell, B. and Hewer, R.L. (1968). The peripheral sensory pathway in Friedreich’s ataxia. An examination by light and electron microscopy of the posterior nerve roots, sensory nerves in cases of Friedreich’s ataxia. Brain, 91, 803–818.CrossRefGoogle ScholarPubMed
Huxtable, R.J., Azari, J., Reisine, T., Johnson, P., Yamamura, H.I. and Barbeau, A. (1979). Regional distribution of amino acids in Friedreich’s ataxia brains. Can. J. Neurol. Sci., 6, 255–258.CrossRefGoogle ScholarPubMed
Janota, I. (1972). Ultrastructural studies of an hereditary sensory neuropathy in mice. (dystonia musculorum). Brain, 95, 529–536.CrossRefGoogle ScholarPubMed
Lowry, O.H., Rosebrough, N.J., Farr, A.L. and Randall, R.J. (1951). Protein measurement with the folin phenol reagent. J. Biol. Chem., 193, 265–275.CrossRefGoogle ScholarPubMed
Messer, A., and Gordon, D. (1979). Changes in whole tissue biosynthesis of 7-aminobutyric acid (GABA) in basal ganglia of the dystonia (dtAlb) mouse. Life Sci., 25, 2217–2221.CrossRefGoogle Scholar
Messer, A. and Strominger, N.L. (1980). An allele of the mouse mutant dystonia musculorum exhibits lesions in red nucleus and striatum. Neuroscience, 5, 543–549.CrossRefGoogle ScholarPubMed
Nadi, N.S., Kanter, D., Mcbride, W.J. and Aprison, M.H. (1977). Effects of 3-acetylpyridine on several putative neurotransmitter amino acids in the cerebellum and medulla of the rat. J. Neurochem., 28, 661–662.CrossRefGoogle ScholarPubMed
Oppenheimer, D.R. (1979). Brain lesions in Friedreich’s ataxia. Can. J. Neurol. Sci., 6, 173–176.CrossRefGoogle ScholarPubMed
Peyronnard, J.M., Lapointe, L., Bouchard, J.P., Lamontagne, A., Lemieux, B., and Barbeau, A. (1976). Nerve conduction studies and electromyography in Friedreich’s ataxia. Can. J. Neurol. Sci., 3, 313–317.CrossRefGoogle ScholarPubMed
Riker, D.K., Messer, A., and Roth, R.H. (1981). Increased noradrenergic metabolism in the cerebellum of the mouse mutant dystonia musculorum. J. Neurochem., 37, 649–654.CrossRefGoogle ScholarPubMed
Roffler-Tarlov, S., and Sidman, R.L. (1978). Concentrations of glutamic acid in cerebellar cortex and deep nuclei of normal mice and weaver, staggerer and nervous mutants. Brain Res., 142, 269–283.CrossRefGoogle ScholarPubMed
Sidman, R.L., Green, M.S., and Appel, S.H. (1965). Catalog of the Neurological Mutants of the Mouse, Harvard University Press, Cambridge, Mass. pp. 19–21.CrossRefGoogle Scholar
Thornburg, L.P., and Hanker, J.S. (1975). Lysosomal hydrolases in the trigeminal ganglion of mice afflicted with an hereditary sensory neuropathy (dystonia musculorum). Acta Neuropat., (Berl) 32, 91–101.CrossRefGoogle ScholarPubMed