title: Decreased cytochrome c oxidase activity but unchanged superoxide dismutase and glutathione peroxidase activities in the spinal cords of patients with amyotrophic lateral sclerosis.
abstract: The cause of selective degeneration of motor neurons in the ventral horn of the spinal cord associated with amyotrophic lateral sclerosis (ALS) has still not been elucidated. Recently, so-called oxidative stress has been suggested to be a significant factor in the pathogenesis of this disease. We measured the antioxidant actions of superoxide dismutase (SOD), glutathione peroxidase (GSH-Px), and cytochrome c oxidase (CO) of the human spinal cord in patients with ALS in comparison with those in control patients. Total SOD activity in spinal cord transections from patients with sporadic ALS was not significantly different from the controls in ventral, lateral, or dorsal regions, although enzymic activity was relatively higher in the ventral compared with the dorsal region. GSH-Px activity in the spinal cord of ALS patients was not very different from that in the control tissue. In contrast, CO activity was significantly reduced in all three regions of the spinal cord in patients with ALS, although the reduction was more marked in the ventral region. These results suggest that reactive oxygen species may attack the mitochondrial respiratory chain, leading eventually to the degeneration of vulnerable motor neurons in the spinal cord, even though no obvious changes in the activity of antioxidant enzymes are detectable.
authors.affiliation: Department of Physiology, School of Medicine, Fujita Health University, Aichi, Japan.
authors.forename: K
authors.initials: K
authors.lastname: Fujita
authors.forename: M
authors.initials: M
authors.lastname: Yamauchi
authors.forename: K
authors.initials: K
authors.lastname: Shibayama
authors.forename: M
authors.initials: M
authors.lastname: Ando
authors.forename: M
authors.initials: M
authors.lastname: Honda
authors.forename: Y
authors.initials: Y
authors.lastname: Nagata
mesh_terms: D000328:Adult; D000368:Aged; D000690:Amyotrophic Lateral Sclerosis / Q000201:enzymology*; D003576:Electron Transport Complex IV / Q000378:metabolism*; D005260:Female; D005979:Glutathione Peroxidase / Q000378:metabolism*; D006801:Humans; D008297:Male; D008875:Middle Aged; D009419:Nerve Tissue Proteins / Q000096:biosynthesis; D009474:Neurons / Q000201:enzymology / Q000648:ultrastructure; D018384:Oxidative Stress / Q000502:physiology; D013116:Spinal Cord / Q000166:cytology / Q000201:enzymology*; D013482:Superoxide Dismutase / Q000378:metabolism*
chemical_list: D009419:Nerve Tissue Proteins; D005979:Glutathione Peroxidase; D013482:Superoxide Dismutase; D003576:Electron Transport Complex IV