title: Oxidative stress and metal content in blood and cerebrospinal fluid of amyotrophic lateral sclerosis patients with and without a Cu, Zn-superoxide dismutase mutation.
abstract: Hydroxyl radical, ascorbate free radical, superoxide dismutase (SOD) activities, Cu,Zn-SOD protein, Mn-SOD protein, 8-hydroxy-2' -deoxyguanosine (8-OHdG) and metals were compared in red blood cells (RBC), plasma and/or cerebrospinal fluid (CSF) between patients with sporadic amyotrophic lateral sclerosis (SALS), familial ALS (FALS) showing the Leu126Ser mutation in the Cu, Zn-SOD gene and controls. In patients with FALS or SALS, concentrations of hydroxyl radical in blood and ascorbate free radical and 8-OHdG in CSF were higher than control group values, while SOD activities in RBC and CSF were lower. In contrast, Cu, Zn-SOD protein concentrations in RBC were low only in FALS patients. Concentrations of Cu in CSF of SALS patients were higher than in controls. Thus, the pathogenesis of increased oxidative stress differs between SALS patients and FALS patients with a mutant Leu126Ser SOD1 gene.
authors.affiliation: Clinical Research Institute and Department of Neurology, National Hospital Organization Minami-Okayama Medical Center, 4066 Hayashima-cho, Tsukubo-gun, Okayama 701-0304, Japan. iharay@s-okayama.hosp.go
authors.forename: Yuetsu
authors.initials: Y
authors.lastname: Ihara
authors.forename: Keigo
authors.initials: K
authors.lastname: Nobukuni
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Takata
authors.forename: Toshiyuki
authors.initials: T
authors.lastname: Hayabara
mesh_terms: D000080242:8-Hydroxy-2'-Deoxyguanosine; D000368:Aged; D000690:Amyotrophic Lateral Sclerosis / Q000097:blood / Q000134:cerebrospinal fluid / Q000235:genetics / Q000503:physiopathology; D016022:Case-Control Studies; D003849:Deoxyguanosine / Q000031:analogs & derivatives* / Q000097:blood / Q000134:cerebrospinal fluid; D004912:Erythrocytes / Q000378:metabolism; D005260:Female; D006403:Hematologic Tests / Q000379:methods; D006801:Humans; D007930:Leucine / Q000031:analogs & derivatives* / Q000235:genetics; D008297:Male; D008670:Metals / Q000097:blood* / Q000134:cerebrospinal fluid*; D008875:Middle Aged; D009154:Mutation; D018384:Oxidative Stress / Q000502:physiology*; D012016:Reference Values; D012694:Serine / Q000235:genetics; D013482:Superoxide Dismutase / Q000097:blood / Q000134:cerebrospinal fluid / Q000235:genetics*
chemical_list: D008670:Metals; D012694:Serine; C035766:leucinal; D000080242:8-Hydroxy-2'-Deoxyguanosine; D013482:Superoxide Dismutase; D003849:Deoxyguanosine; D007930:Leucine