title: Effect of the human SOD1-G93A gene on the Nrf2/ARE signaling pathway in NSC-34 cells.
abstract: Dominant mutations in superoxide dismutase 1 (SOD1) are a frequent cause of the lethal neurodegenerative disease amyotrophic lateral sclerosis (ALS). The nuclear factor erythroid 2‑related factor 2 (Nrf2)/antioxidant response element (ARE) signaling pathway is the major cellular defense mechanism against oxidative stress, however, its role in ALS remains to be fully elucidated. Therefore, the present study aimed to investigate whether the human SOD1-G93A gene affected the Nrf2/ARE signaling pathway in an ALS cell model. The soma became round and the number of neurites decreased in the NSC-34 cells transfected with the hSOD1-G93A gene, and the neurites were shorter and oxidative stress was increased compared with the normal NSC-34 cells. Furthermore, the mRNA and protein expression of Nrf2, heme oxygenase-1 and
  quinone oxidoreductase 1 was significantly decreased in the NSC-34 cells transfected with the human SOD1-G93A gene. The present study indicated that human SOD1-G93A damaged the Nrf2/ARE signaling pathway in the ALS cell model and reduced the ability of cells to protect against oxidative injury.
authors.affiliation: Department of Clinical Neurology, School of Neurology, Weifang Medical University, Weifang, Shandong 261053, P.R. China.
authors.forename: Fumin
authors.initials: F
authors.lastname: Wang
authors.affiliation: Central Laboratory, Linyi People's Hospital, Linyi, Shandong 276003, P.R. China.
authors.forename: Yucheng
authors.initials: Y
authors.lastname: Lu
authors.affiliation: Department of Neurology, Linyi People's Hospital, Linyi, Shandong 276003, P.R. China.
authors.forename: Faying
authors.initials: F
authors.lastname: Qi
authors.affiliation: Central Laboratory, Linyi People's Hospital, Linyi, Shandong 276003, P.R. China.
authors.forename: Quanping
authors.initials: Q
authors.lastname: Su
authors.affiliation: Central Laboratory, Linyi People's Hospital, Linyi, Shandong 276003, P.R. China.
authors.forename: Long
authors.initials: L
authors.lastname: Wang
authors.affiliation: Central Laboratory, Linyi People's Hospital, Linyi, Shandong 276003, P.R. China.
authors.forename: Cuiping
authors.initials: C
authors.lastname: You
authors.affiliation: Department of Neurology, Linyi People's Hospital, Linyi, Shandong 276003, P.R. China.
authors.forename: Fengyuan
authors.initials: F
authors.lastname: Che
authors.affiliation: Department of Neurology, Linyi People's Hospital, Linyi, Shandong 276003, P.R. China.
authors.forename: Jixu
authors.initials: J
authors.lastname: Yu
mesh_terms: D000690:Amyotrophic Lateral Sclerosis / Q000235:genetics / Q000378:metabolism; D061745:Antioxidant Response Elements; D002460:Cell Line; D004843:Epistasis, Genetic; D005786:Gene Expression Regulation; D006801:Humans; D015227:Lipid Peroxidation; D050217:Metabolic Detoxication, Phase II; D051267:NF-E2-Related Factor 2 / Q000235:genetics / Q000378:metabolism*; D018384:Oxidative Stress; D015398:Signal Transduction; D013482:Superoxide Dismutase / Q000235:genetics* / Q000378:metabolism*
chemical_list: D051267:NF-E2-Related Factor 2; C109415:SOD1 G93A protein; D013482:Superoxide Dismutase