title: Radio-sensitivity of the cells from amyotrophic lateral sclerosis model mice transfected with human mutant SOD1.
abstract: In order to clarify the possible involvement of oxidative damage induced by ionizing radiation in the onset and/or progression of familial amyotrophic lateral sclerosis (ALS), we studied radio-sensitivity in primary cells derived from ALS model mice expressing human mutant SOD1. The primary mouse cells expressed both mouse and the mutant human SOD1. The cell survival of the transgenic mice (with mutant SOD1), determined by counting cell numbers at a scheduled time after X-irradiation, is very similar to that of cells from wild type animals. The induction and repair of DNA damage in the transgenic cells, measured by single cell gel electrophoresis and pulsed field gel electrophoresis, are also similar to those of wild type cells. These results indicate that the human mutant SOD1 gene does not seem to contribute to the alteration of radio-sensitivity, at least in the fibroblastic cells used here. Although it is necessary to consider the difference in cell types between fibroblastic and neuronal cells, the present results may suggest that ionizing radiation is not primarily responsible for the onset of familial ALS with the SOD1 mutation, and that the excess risks are probably not a concern for radiation diagnosis and therapy in familial ALS patients.
authors.affiliation: Department of Neurology, Kansai Medical University, Moriguchi, Japan.
authors.forename: Reika
authors.initials: R
authors.lastname: Wate
authors.forename: Sentaro
authors.initials: S
authors.lastname: Takahashi
authors.forename: Hidefumi
authors.initials: H
authors.lastname: Ito
authors.forename: Hirofumi
authors.initials: H
authors.lastname: Kusaka
authors.forename: Yoshihisa
authors.initials: Y
authors.lastname: Kubota
authors.forename: Katsutoshi
authors.initials: K
authors.lastname: Suetomi
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Sato
authors.forename: Ryuichi
authors.initials: R
authors.lastname: Okayasu
mesh_terms: D000690:Amyotrophic Lateral Sclerosis / Q000201:enzymology* / Q000235:genetics*; D000818:Animals; D002470:Cell Survival / Q000528:radiation effects; D002478:Cells, Cultured; D004247:DNA / Q000528:radiation effects; D004249:DNA Damage; D004307:Dose-Response Relationship, Radiation; D005347:Fibroblasts / Q000201:enzymology* / Q000473:pathology / Q000528:radiation effects*; D006801:Humans; D051379:Mice; D008822:Mice, Transgenic; D011829:Radiation Dosage; D011836:Radiation Tolerance; D013482:Superoxide Dismutase / Q000235:genetics* / Q000378:metabolism*; D014162:Transfection / Q000379:methods
chemical_list: D004247:DNA; D013482:Superoxide Dismutase