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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*
Affiliation:
Birth Defects Institute, Center for Laboratories and Research, New York State Department of Health, Albany, New York
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Summary:

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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
Copyright
Copyright © Canadian Neurological Sciences Federation 1982

References

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