title: Blood superoxide dismutase, catalase and glutathione peroxidase activities in familial and sporadic amyotrophic lateral sclerosis.
abstract: Recent studies have implicated free radicals in the pathogenesis of amyotrophic lateral sclerosis (ALS), a fatal, paralytic disorder of motor neurons. Herein we report on measurements of erythrocyte activity of the three main free radical scavenging enzymes: copper/zinc superoxide dismutase (Cu/Zn-SOD), catalase, and glutathione peroxidase. We studied 31 patients with sporadic ALS, 18 with familial ALS, and 24 controls, Mean Cu/Zn-SOD activity was reduced in eight familial ALS patients with mutations of Cu/Zn-SOD but was normal in patients with both familial ALS without identified Cu/Zn-SOD mutations and sporadic ALS. Glutathione peroxidase activity was significantly reduced only in sporadic ALS patients treated with insulin-like growth factor I (100 micrograms/kg). Catalase activity was normal in sporadic and familial ALS. Neither glutathione peroxidase nor catalase activities correlated significantly with duration of symptoms or age at onset. Vitamin E, vitamin C, and beta-carotene did not affect any of the three enzyme activities. These observations indicate that disturbances of catalase and glutathione peroxidase function are not likely to be central factors in the pathogenesis of ALS.
authors.affiliation: Department of Neurology, Columbia University, New York, NY 10032, USA.
authors.forename: S
authors.initials: S
authors.lastname: Przedborski
authors.forename: D M
authors.initials: DM
authors.lastname: Donaldson
authors.forename: P L
authors.initials: PL
authors.lastname: Murphy
authors.forename: O
authors.initials: O
authors.lastname: Hirsch
authors.forename: D
authors.initials: D
authors.lastname: Lange
authors.forename: A B
authors.initials: AB
authors.lastname: Naini
authors.forename: D
authors.initials: D
authors.lastname: McKenna-Yasek
authors.forename: R H
authors.initials: RH
authors.lastname: Brown
mesh_terms: D017668:Age of Onset; D000690:Amyotrophic Lateral Sclerosis / Q000097:blood / Q000201:enzymology* / Q000235:genetics; D002374:Catalase / Q000097:blood* / Q000235:genetics*; D004912:Erythrocytes / Q000201:enzymology*; D005260:Female; D005979:Glutathione Peroxidase / Q000097:blood*; D006801:Humans; D007334:Insulin-Like Growth Factor I / Q000627:therapeutic use*; D008297:Male; D008875:Middle Aged; D009154:Mutation; D010919:Placebos; D012016:Reference Values; D012044:Regression Analysis; D018709:Statistics, Nonparametric; D013482:Superoxide Dismutase / Q000097:blood*
chemical_list: D010919:Placebos; D007334:Insulin-Like Growth Factor I; D002374:Catalase; D005979:Glutathione Peroxidase; D013482:Superoxide Dismutase