title: Decreased glutamate dehydrogenase protein in spinocerebellar degeneration.
abstract: A radioimmunoassay system for determining content of glutamate dehydrogenase (GDH) in human leukocytes was established and studied in 14 patients with spinocerebellar ataxia or atypical Parkinsonism. The protein content of leukocyte GDH was decreased in four patients and the reduction in the protein content was proportional to that in the enzyme activity. The ratio of GDH activity to protein content was invariable in healthy controls, diseased controls and patients with reduced GDH activity. These results suggested that at least a portion of the partial GDH deficiency was due to the decreased level of the enzyme protein.
authors.affiliation: Second Department of Internal Medicine, Osaka University, Medical School, Japan.
authors.forename: K
authors.initials: K
authors.lastname: Kajiyama
authors.forename: S
authors.initials: S
authors.lastname: Ueno
authors.forename: T
authors.initials: T
authors.lastname: Tatsumi
authors.forename: S
authors.initials: S
authors.lastname: Yorifuji
authors.forename: M
authors.initials: M
authors.lastname: Takahashi
authors.forename: S
authors.initials: S
authors.lastname: Tarui
mesh_terms: D000328:Adult; D005969:Glutamate Dehydrogenase / Q000097:blood / Q000172:deficiency*; D006801:Humans; D007962:Leukocytes / Q000201:enzymology; D008297:Male; D008875:Middle Aged; D009849:Olivopontocerebellar Atrophies / Q000201:enzymology; D010302:Parkinson Disease, Secondary / Q000201:enzymology; D011863:Radioimmunoassay; D013132:Spinocerebellar Degenerations / Q000201:enzymology* / Q000235:genetics
chemical_list: D005969:Glutamate Dehydrogenase