Hostname: page-component-784d4fb959-57n77 Total loading time: 0 Render date: 2025-07-16T00:18:16.375Z Has data issue: false hasContentIssue false
  • English
  • Français

Leucocyte Glutamate Dehydrogenase in Various Hereditary Ataxias

Published online by Cambridge University Press:  18 September 2015

A. Barbeau ,
M. Charbonneau  and
T. Cloutier
A. Barbeau*
Affiliation:
Department of Neurobiology, Clinical Research Institute of Montreal
M. Charbonneau
Affiliation:
Department of Neurobiology, Clinical Research Institute of Montreal
T. Cloutier
Affiliation:
Department of Neurobiology, Clinical Research Institute of Montreal
*
Clinical Research Institute of Montreal, 110 Pine Avenue Ave. West, Montreal, Quebec, Canada H2W 1R7
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.

Leucocyte Glutamate Dehydrogenase (GDH) activity was measured in 44 patients with various forms of ataxia and 44 age and sex-matched normal controls. The only significant change found was a moderate decrease in activity in Freidreich's ataxia and a few patients with OPCA. This decreased activity is not primary to the disease but probably reflects a regulatory defect affecting mitochondrial membranes in these patients.

Information

Type
Quebec Cooperative Study of Friedreich's Ataxia
Information
Canadian Journal of Neurological Sciences , Volume 7 , Issue 4 , November 1980 , pp. 421 - 424
Copyright
Copyright © Canadian Neurological Sciences Federation 1980

References

REFERENCES

Bouchard, J.P., Barbeau, A., Bouchard, R. and Bouchard, R.W. (1978). Autosomal recessive spastic ataxia of Charlevois-Saguenay. Can. J. Neurol. Sci., 5: 61–69.CrossRefGoogle Scholar
Butterworth, R.F., Hamel, E., Landreville, 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., Landreville, 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
Davidson, N. (1978). Neurotransmitter amino acids. London, Academic Press.Google Scholar
Geoffroy, G., Barbeau, A., Breton, G., Lemieux, B., Aubé, M., LéGer, C. and Bouchard, J.P. (1976). Clinical description of Roentgenologic evaluation of patients with Friedreich’s ataxia. Can. J. Neurol. Sci., 3: 279–286.CrossRefGoogle ScholarPubMed
Graham, L.T. and Aprison, M.H. (1969). Distribution of some enzymes associated with the metabolism of glutamate, aspartate, γ-amino butyrate and glutamine in cat spinal cord. J. Neurochem., 16: 559–566.CrossRefGoogle Scholar
Huxtable, R., Azari, X, 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
Konigsmark, B.W. and Weiner, L.P. (1970). The olivopontocerebellar atrophies. A review. Medicine, 49: 227–241.Google ScholarPubMed
Lowry, O.H., Rosenbrough, N.J., Ferr, A.L. and Randell, R.J. (1951). Protein measurement with the Folin-phenol reagent. J. Biol. Chem., 193: 265–268.CrossRefGoogle ScholarPubMed
McBride, W.J., Aprison, M.H. and Kusano, K. (1976). Contents of several amino acids in the cerebellum, brain stem and cerebrum of the ‘staggerer’, ‘weaver’ and ‘nervous’ neurologically mutant mice. J. Neurochem., 26: 867–870.CrossRefGoogle ScholarPubMed
Nelson, R.L., Povey, M.S. and Hopkinson, D.A. (1977). Electrophoresis of human L-glutamate dehydrogenase: tissue distribution and preliminary population survey. Biochem. Genet., 15: 87–91.CrossRefGoogle ScholarPubMed
Perry, T.L., Currier, R.D., Hansen, S. and Maclean, J. (1977). Aspartate-taurine imbalance in dominantly inherited olivopontocerebellar atrophy. Neurology, 27: 257–261.CrossRefGoogle ScholarPubMed
Plaitakis, A., Nicklas, W.J. and Desnick, R.J. (1980). Glutamate dehydrogenase deficiency in three patients with spinocerebellar syndrome. Ann. Neurol., 7: 297–303.CrossRefGoogle ScholarPubMed
Robinson, N. (1968). Chemical changes in spinal cord in Friedreich’s Ataxia and motor neurone disease. J. Neurol. Neurosurg. Psychiat., 31: 330–333.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: 269–293.CrossRefGoogle ScholarPubMed
Schmidt, E. (1974). Glutamate dehydrogenase, in: Bergmeyer, H.V. “Methods in engymatic analysis”.; Academic Press, New York, 2nd edition, pp. 650–656.CrossRefGoogle Scholar
Wastiaux, J.P., Lamoureux, G., Bouchard, J.P., Durivage, A., Barbeau, C. and Barbeau, A. (1978). HLA and complement typing in olivo-ponto-cerebellar atrophy. Can. J. Neurol. Sci., 5: 75–81.CrossRefGoogle ScholarPubMed