Oxidative stress and metal content in blood and cerebrospinal fluid of amyotrophic lateral sclerosis patients with and without a Cu, Zn-superoxide dismutase mutation.
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.
Ihara
Yuetsu
Y
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
Nobukuni
Keigo
K
Takata
Hiroshi
H
Hayabara
Toshiyuki
T
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*
D008670:Metals; D012694:Serine; C035766:leucinal; D000080242:8-Hydroxy-2'-Deoxyguanosine; D013482:Superoxide Dismutase; D003849:Deoxyguanosine; D007930:Leucine