Formation of granular cytoplasmic aggregates in COS7 cells expressing mutant Cu/Zn superoxide dismutase associated with familial amyotrophic lateral sclerosis

Neuroscience Letters

Volumn 257, Issue 1, 1998, Pages 29-32

  Koide, Takashi ^a   Igarashi, Shuichi ^a   Kikugawa, Koki ^a   Nakano, Ryoichi ^a   Inuzuka, Takashi ^a   Yamada, Mitsunori ^a   Takahashi, Hitoshi ^a   Tsuji, Shoji ^a  

^a Niigata University   (Japan)

Author keywords

Cu Zn superoxide dismutase; Familial amyotrophic lateral sclerosis; Granular cytoplasmic aggregates

Indexed keywords

COPPER ZINC SUPEROXIDE DISMUTASE; MUTANT PROTEIN;

AMYOTROPHIC LATERAL SCLEROSIS; ANIMAL CELL; ARTICLE; CONTROLLED STUDY; CYTOSOL; ENDOPLASMIC RETICULUM; ENZYME LOCALIZATION; MONKEY; NONHUMAN; PRIORITY JOURNAL; PROTEIN EXPRESSION;

AMINO ACID SUBSTITUTION; AMYOTROPHIC LATERAL SCLEROSIS; ANIMALS; BLOTTING, WESTERN; COS CELLS; CYTOPLASM; CYTOPLASMIC GRANULES; GENE EXPRESSION; MITOCHONDRIA; MUTATION; SUPEROXIDE DISMUTASE; TIME FACTORS; TRANSFECTION;

EID: 0032553346     PISSN: 03043940     EISSN: None     Source Type: Journal    
DOI: 10.1016/S0304-3940(98)00800-3     Document Type: Article

References (19)

1. Bruijn L.I., Becher M.W., Lee M.K., Anderson K.L., Jenkins N.A., Copeland N.G., Sisodia S.S., Rothstein J.D., Borchelt D.R., Price D.L., Cleveland D.W. ALS-linked SOD1 mutant G85R mediates damage to astrocytes and promotes rapidly progressive disease with SOD1-containing inclusions. Neuron. 18:1995;327-338.
2. Carrell R.W., Lomas D.A. Conformational disease, Lancet. 350:1997;134-138.
3. Deng H.-X., Hentati A., Tainer J.A., Iqbal Z., Cayabyab A., Hung W.-Y., Getzoff E.D., Hu P., Herzfeldt B., Roos R.P., Warner C., Deng G., Soriano E., Parge H.E., Ahmed A., Roses A.D., Hallewell R.A., Pericak-Vance M.A., Siddique T. Amyotrophic lateral sclerosis and structure defect in Cu. Zn superoxide dismutase, Science. 261:1993;1047-1051.
4. Durham H.D., Roy J., Dong L., Figlewicz D.A. Aggregation of human Cu/Zn superoxide dismutase proteins in a culture model of ALS. J. Neuropathol. Exp. Neurol. 56:1997;523-530.
5. Gurney M.E., Pu H., Chiu A.Y., Dal Canto M.C., Polchow C.Y., Alexander D.D., Caliendo J., Hentati A., Kwon Y.W., Deng H.X., Chen W., Zhai P., Sufit R.L., Siddique T. Motor neuron degeneration in mice that express a human Cu. Zn superoxide dismutase mutation, Science. 264:1994;1772-1775.
6. Jones C.T., Shaw P.J., Chari G., Brock D.J.H. Identification of a novel SOD1 mutation in an apparently sporadic amyotrophic lateral sclerosis patient. Mol. Cell. Probes. 8:1994;329-330.
7. Jones C.T., Swingler R.J., Brook D.J.H. Identification of a novel SOD1 mutation in an apparently sporadic amyotrophic lateral sclerosis patient and the detection of Ile113Thr in three others. Hum. Mol. Genet. 3:1994;649-650.
8. Kato S., Hayashi H., Nakashima K., Nanba E., Kato M., Hirano A., Nakano I., Asayama K., Ohama E. Pathological characterization of astrocytic hyaline inclusions in familial amyotrophic lateral sclerosi. Am. J. Pathol. 151:1997;611-620.
9. Kikugawa K., Nakano R., Inuzuka T., Kokubo Y., Narita Y., Kuzuhara S., Yoshida S., Tsuji S. A missense mutation in the SOD1 gene in patients with amyotrophic lateral sclerosis from the Kii Peninsula and its vicinity, Japan. Neurogenetics. 1:1997;113-115.
10. Mikaelian I., Sergeant A.A. General and fast method to generate multiple site directed mutations. Nucleic Acid Res. 20:1992;376.
11. Mizushima S., Nagata S. pEF-BOS, a powerful mammalian expression vector. Nucleic Acid Res. 18:1990;5332.
12. Nakano R., Inuzuka T., Kikugawa K., Takahashi H., Sakimura K., Fujii J., Taniguchi N., Tsuji S. Instability of mutant Cu/Zn superoxide dismutase (Ala4Thr) associated with familial amyotrophic lateral sclerosis. Neurosci. Lett. 211:1996;129-131.
13. Nakano R., Sato S., Inuzuka T., Sakimura K., Mishina M., Takahashi H., Ikuta F., Honma Y., Fujii J., Taniguchi N., Tsuji S. A novel mutation in Cu/Zn superoxide dismutase gene in Japanese familial amyotrophic lateral sclerosis. Biochem. Biophys. Res. Commun. 200:1994;695-703.
14. Rosen D.R., Siddique T., Patterson D., Figlewicz D.A., Sapp P., Hentati A., Donaldson D., Goto J., O'Regan J.P., Deng H.X., Rahmani Z., Krizus A., McKenna-Yasek D., Cayabyab A., Gaston S.M., Berger R., Tanzi R.E., Halperin J.J., Herzfeldt B., Van den Bergh R., Hung W.-Y., Bird T., Deng G., Mulder D.W., Smyth C., Laing N.G., Soriano E., Pericak-Vance M.A., Haines J., Rouleau G.A., Gusella J.S., Horvitz H.R., Brown R.H. Jr. Mutations in Cu/Zn superoxide dismutase gene are associated with familial amyotrophic lateral sclerosis. Nature. 362:1993;59-62.
15. Shaw C.E., Enayat Z.E., Chioza B.A., Al-Chalabi A., Raudunobic A., Powell J.F., Leigh P.N. Mutation in all five exons of SOD-1 may cause ALS. Ann. Neurol. 43:1998;390-394.
16. Shibata N., Hirano A., Kobayashi M., Komori T., Dal Canto M.C., Gurney M.E., Asayama K. Presence of superoxide dismutase-1 (SOD1) immunoreactivity in hyaline inclusion bodies of the spinal cord neurons of transgenic mice for mutant SOD1 gene. J. Neuropath. Exp. Neurol. 55:1996;632.
17. Takahashi H. Familial amyotrophic lateral sclerosis with or without mutation of the Cu/Zn superoxide dismutase gene. No to shinkei. 47:1995;535-541.
18. Takahashi K., Nakamura H., Okada E. Hereditary amyotrophic lateral sclerosis. Histochemical and electron microscopic study of hyaline inclusions in motor neurons. Arch. Neurol. 27:1972;292-299.
19. Watanabe Y., Kono Y., Nanba E., Ohama E., Nakashima K. Instability of expressed Cu/Zn superoxide dismutase with 2bp deletion found in familial amyotrophic lateral sclerosis. FEBS Lett. 400:1997;108-112.