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Keywords:

  • spinocerebellar degeneration;
  • L-glutamate;
  • glutamate dehydrogenase;
  • fibroblast;
  • glutathione

ABSTRACT— From 21 patients with spinocerebellar degeneration 5 had markedly decreased glutamate dehydrogenase (GDH) activities and high values of serum plasma glutamate level after oral glutamate loading tests. Skin fibroblasts from patients with GDH deficiency showed intracellularly higher glutamate and lower glutathione contents than those from controls and showed significantly decreased viability in L-glutamate-containing medium. These data suggest that glutamate toxicity may at least play a part in this degeneration process.