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Glutamate metabolism of leukocytes and skin fibroblasts in spinocerebellar degeneration with lowered glutamate dehydrogenase activity
Author(s) -
Tatsumi C.,
Yorifuji S.,
Kajiyama K.,
Ueno S.,
Takahashi M.,
Tarui S.
Publication year - 1989
Publication title -
acta neurologica scandinavica
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.967
H-Index - 95
eISSN - 1600-0404
pISSN - 0001-6314
DOI - 10.1111/j.1600-0404.1989.tb03816.x
Subject(s) - glutamate receptor , glutamate dehydrogenase , degeneration (medical) , metabolism , toxicity , glutathione , glutamic acid , biochemistry , medicine , chemistry , endocrinology , biology , amino acid , pathology , enzyme , receptor
— 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.