Amino Acid Changes in the Mouse Mutant Dystonia Musculorum Similar to Those in Friedreich’s Ataxia
- 1 May 1982
- journal article
- research article
- Published by Cambridge University Press (CUP) in Canadian Journal of Neurological Sciences
- Vol. 9 (2) , 185-188
- https://doi.org/10.1017/s0317167100043936
Abstract
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.Keywords
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