title: Motor neurons in Cu/Zn superoxide dismutase-deficient mice develop normally but exhibit enhanced cell death after axonal injury.
abstract: The discovery that some cases of familial amyotrophic lateral sclerosis (FALS) are associated with mutations in the gene encoding Cu/Zn superoxide dismutase (SOD1) has focused much attention on the function of SOD1 as related to motor neuron survival. Here we describe the creation and characterization of mice completely deficient for this enzyme. These animals develop normally and show no overt motor deficits by 6 months in age. Histological examination of the spinal cord reveals no signs of pathology in animals 4 months in age. However Cu/Zn SOD-deficient mice exhibit marked vulnerability to motor neuron loss after axonal injury. These results indicate that Cu/Zn SOD is not necessary for normal motor neuron development and function but is required under physiologically stressful conditions following injury.
authors.affiliation: Department of Molecular Biology, Cephalon Inc., West Chester Pennsylvania 19380, USA.
authors.forename: A G
authors.initials: AG
authors.lastname: Reaume
authors.forename: J L
authors.initials: JL
authors.lastname: Elliott
authors.forename: E K
authors.initials: EK
authors.lastname: Hoffman
authors.forename: N W
authors.initials: NW
authors.lastname: Kowall
authors.forename: R J
authors.initials: RJ
authors.lastname: Ferrante
authors.forename: D F
authors.initials: DF
authors.lastname: Siwek
authors.forename: H M
authors.initials: HM
authors.lastname: Wilcox
authors.forename: D G
authors.initials: DG
authors.lastname: Flood
authors.forename: M F
authors.initials: MF
authors.lastname: Beal
authors.forename: R H
authors.initials: RH
authors.lastname: Brown
authors.forename: R W
authors.initials: RW
authors.lastname: Scott
authors.forename: W D
authors.initials: WD
authors.lastname: Snider
mesh_terms: D000818:Animals; D001369:Axons / Q000473:pathology / Q000502:physiology*; D005154:Facial Nerve / Q000166:cytology / Q000473:pathology / Q000502:physiology; D005978:Glutathione / Q000378:metabolism; D015227:Lipid Peroxidation; D051379:Mice; D008817:Mice, Mutant Strains; D009046:Motor Neurons / Q000473:pathology / Q000502:physiology*; D011995:Recombination, Genetic; D012016:Reference Values; D013116:Spinal Cord / Q000166:cytology / Q000473:pathology*; D013482:Superoxide Dismutase / Q000172:deficiency* / Q000235:genetics* / Q000378:metabolism
chemical_list: D013482:Superoxide Dismutase; D005978:Glutathione