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Amino Acid Changes in Thiamine-Deficient Encephalopathy: Some Implications for the Pathogenesis of Friedreich's Ataxia

Published online by Cambridge University Press:  18 September 2015

R.F. Butterworth ,
E. Hamel ,
F. Landreville  and
A. Barbeau
R.F. Butterworth
Affiliation:
The Clinical Research Institute of Montreal
E. Hamel
Affiliation:
The Clinical Research Institute of Montreal
F. Landreville
Affiliation:
The Clinical Research Institute of Montreal
A. Barbeau
Affiliation:
The Clinical Research Institute of Montreal
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Summary

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Thiamine-deficient encephalopathy in the rat is characterized by ataxie gait, loss of righting reflex and curvature of the spine. N euro chemie ai changes include a diminished activity of cerebral pyruvate decarboxylase leading to abnormal pyruvate oxidation. The present study shows that this defective pyruvate oxidation produces a significant depletion of three important amino acid neurotransmitters, namely gamma aminobuiyrie acid (GABA), glutamic acid, andaspartic acid. Such changes could lead to severe neuronal dysfunction and the observed neurological symptoms of thiamine deficiency. Some implications for the pathogenesis of Friedreich's ataxia are discussed.

Type
Research Article
Information
Canadian Journal of Neurological Sciences , Volume 6 , Issue 2 , May 1979 , pp. 217 - 222
Copyright
Copyright © Canadian Neurological Sciences Federation 1979

References

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