Motor neurons in Cu/Zn superoxide dismutase-deficient mice develop normally but exhibit enhanced cell death after axonal injury

Nature Genetics

Volumn 13, Issue 1, 1996, Pages 43-47

  Reaume, Andrew G ^a   Elliott, Jeffrey L ^b   Hoffman, Eric K ^a   Kowall, Neil W ^c,d   Ferrante, Robert J ^c,d   Siwek, Donald F ^c,d   Wilcox, Heide M ^a   Flood, Dorothy G ^a   Beal, M Flint ^e   Brown Jr , Robert H ^e   Scott, Richard W ^a   Snider, William D ^b  

^a CEPHALON INC   (United States)

^b WASHINGTON UNIVERSITY SCHOOL OF MEDICINE   (United States)

^c VETERANS AFFAIRS MEDICAL CENTER   (United States)

^d BOSTON UNIVERSITY SCHOOL OF MEDICINE   (United States)

^e MASSACHUSETTS GENERAL HOSPITAL   (United States)

Author keywords

[No Author keywords available]

Indexed keywords

COPPER ZINC SUPEROXIDE DISMUTASE;

AMYOTROPHIC LATERAL SCLEROSIS; ARTICLE; ENZYME ACTIVITY; ENZYME DEFICIENCY; FAMILIAL DISEASE; GENE MAPPING; MOTONEURON; MOUSE; NERVE CELL NECROSIS; NONHUMAN; OXIDATIVE STRESS; PATHOGENESIS; PRIORITY JOURNAL;

ANIMALS; AXONS; FACIAL NERVE; GLUTATHIONE; LIPID PEROXIDATION; MICE; MICE, MUTANT STRAINS; MOTOR NEURONS; RECOMBINATION, GENETIC; REFERENCE VALUES; SPINAL CORD; SUPEROXIDE DISMUTASE;

ANIMALIA;

EID: 15844393658     PISSN: 10614036     EISSN: None     Source Type: Journal    
DOI: 10.1038/ng0596-43     Document Type: Article

References (23)

1. Fridovich, I. Superoxide dismutases: an adaptation to a paramagnetic gas. J. Biol. Chem. 264, 7761-7764 (1989).
2. McCord, J.M Human disease, free radicals and the oxidant/antioxiant balance. Clin. Biochem. 26, 351-357 (1993)
3. Beyer, W., Imlay, J & Fridovich, I. Superoxide dismutases. Prog Nucl. Acid Res. Mol. Biol. 40, 221-253 (1991)
4. Bilinski, T , Krawiec, Z , Liczmanski, A & Utwinska, J. Is hydroxyl radical generated by the Fenton reaction in vivo?. Biochem Biophys. Res. Commun. 130, 533 (1985).
5. Phillips, J.P., Campbell, S.D., Michaud, D., Charbonneau, M. & Hilliker, A.J. Null mutation of copper/zinc superoxide dismutase in Drosophila confers hypersensitivity to paraquat and reduced longevity. Proc. Natl. Acad Sci USA 86, 2761-2765 (1989).
6. Li, Y et al. Dilated cardiomyopathy and neonatal lethality in mutant mice lacking manganese superoxide dismutase Nature Genet. 11, 376-381 (1995)
7. Rosen, D.R. et al Mutations m Cu/Zn superoxide dismutase gene are associated with familial amyotrophic lateral sclerosis Nature 362, 59-62 (1993).
8. Deng, H.-X et al. Amyotrophic lateral sclerosis and structural defects in Cu,Zn superoxide dismutase. Science 261, 1047-1051 (1993).
9. Bowling, A.C., Schulz, J B., Brown, R H. & Beal, M F. Superoxide dismutase activity, oxidative damage, and mitochondrial energy metabolism in familial and sporadic amyotrophic lateral sclerosis. J Neurochem. 61, 2322-2325 (1993)
10. Borchelt, D.R. et al. Superoxide dismutase 1 with mutations linked to familial amyotrophic lateral sclerosis possesses significant activity. Proc. Natl. Acad. Sci. USA 91, 8292-8296 (1994).
11. Gurney, M.E. et al Motor neuron degeneration in mice that express a human Cu,Zn superoxide dismutase mutation. Science 264, 1772-1775 (1994).
12. Wong, P.C. et al. An adverse property of a familal ALS-linked SOD1 mutation causes motor neuron disease characterized by vacuolar degeneration of mitochondria. Neuron 14, 1105-1116 (1995).
13. Carlioz, A. & Touati, D. Isolation of superoxide dismutase mutants in Escherichia coli: is superoxide dismutase necessary for aerobic life? EMBO J. 5, 623-630 (1986).
14. Marklund, S.L. Extracellular superoxide dismutase and other superoxide dismutase isoenzymes in tissues from nine mammalian species. Biochem. J. 222, 649-655 (1984)
15. Wiedau-Pazos, M. et al. Altered reactivity of superoxide dismutase in familial amyotrophic lateral sclerosis. Science 271, 515-518 (1996).
16. Beckman, J.S., Chen, J , Crow, J.P. & Ye, Y Z. Reactions of nitric oxide, superoxide and peroxynitrite with superoxide dismutase in neurodegeneration. Prog. Brain Res. 103, 371-380 (1994)
17. Reaume, A.G. et al. Cardiac malformation in neonatal mice lacking connexin43 Science 267, 1831-1833 (1995)
18. Wood, S.A., Allen, N.D., Rossant, J., Auerbach, A & Nagy. A Non-injection methods for the production of embryonic stem cell-embryo chimaeras. Nature 365, 87-89 (1993).
19. Paoletti, R, Aldinucci, D., Mocali, A. & Capamni, A. A sensitive spectrophotometric method for the determination of superoxide dismutase activity in tissue extracts. Anal. Biochem. 154, 536-541 (1986).
20. Levine, R.L., Williams, J A , Stadtman, E.R. & Shacter, E. Carbonyl assays for determination of oxidativeiy modified proteins Meth. Enz. 233, 346-357 (1994).
21. Griffith, D.W. Determination of glutathione and glutathione disulfide using glutathione reductase and 2-vinylpyridine. Anal Biochem. 106, 207-212 (1980)
22. Yan, Q , Elliott, J.L. & Snider, W.D Brain-derived neurotrophic factor (BDNF) rescues spinal motoneurons from axotomy-induced cell death. Nature 360, 753-755 (1992).
23. Mansour, S L., Thomas, K R. & Capecchi, M.R. Disruption of the protooncogene int-2 in mouse embryo-derived stem cells: a general strategy for targeting mutations to non-selectable genes. Nature 336, 348-362 (1988).