메뉴 건너뛰기




Volumn 271, Issue 5248, 1996, Pages 515-518

Altered reactivity of superoxide dismutase in familial amyotrophic lateral sclerosis

Author keywords

[No Author keywords available]

Indexed keywords

CHELATING AGENT; DIETHYLDITHIOCARBAMIC ACID; HYDROGEN PEROXIDE; PENICILLAMINE; SUPEROXIDE DISMUTASE;

EID: 0029671220     PISSN: 00368075     EISSN: None     Source Type: Journal    
DOI: 10.1126/science.271.5248.515     Document Type: Article
Times cited : (670)

References (36)
  • 2
    • 0027401203 scopus 로고
    • D. R. Rosen et al., Nature 362, 59 (1993)
    • (1993) Nature , vol.362 , pp. 59
    • Rosen, D.R.1
  • 3
    • 0027426169 scopus 로고
    • H. X Deng et al., Science 261, 1047 (1993)
    • (1993) Science , vol.261 , pp. 1047
    • Deng, H.X.1
  • 4
    • 13344265498 scopus 로고
    • M. E. Gumey et al., ibid. 264, 1772 (1994); M. E Ripps, G. W. Huntley, P. R. Hof, J. H Mornson, J. W. Gordon, Proc. Natl. Acad. Sci U.S.A 92, 689 (1995); C. A. Pardo et al., ibid., p. 954
    • (1994) Science , vol.264 , pp. 1772
    • Gumey, M.E.1
  • 6
    • 13344251465 scopus 로고    scopus 로고
    • M. E. Gumey et al., ibid. 264, 1772 (1994); M. E Ripps, G. W. Huntley, P. R. Hof, J. H Mornson, J. W. Gordon, Proc. Natl. Acad. Sci U.S.A 92, 689 (1995); C. A. Pardo et al., ibid., p. 954
    • Proc. Natl. Acad. Sci U.S.A , pp. 954
    • Pardo, C.A.1
  • 9
    • 0014691242 scopus 로고
    • J. M. McCord and I. Fridovich, J. Biol. Chem 244, 6049 (1969); I Fndovich, Annu. Rev. Biochem. 64, 97 (1995).
    • (1995) Annu. Rev. Biochem. , vol.64 , pp. 97
    • Fndovich, I.1
  • 11
    • 0016816804 scopus 로고    scopus 로고
    • E. K. Hodgson and I. Fndovich, Biochemistry 14, 5294 (1975); ibid , p 5299.
    • Biochemistry , pp. 5299
  • 14
    • 0027456505 scopus 로고
    • M. B. Yim, P B Chock, E R. Stadtman, Proc. Natl. Acad Sci. USA. 87, 5006 (1990), J. Biol. Chem. 268, 4099 (1993).
    • (1993) J. Biol. Chem. , vol.268 , pp. 4099
  • 17
    • 12044249993 scopus 로고
    • 4 [C. R Nishida, E. B. Gralla, J. S. Valentine, Proc. Natl Acad. Sci U.S.A. 91, 9906 (1994)]. The extent of metallation was confirmed by atomic absorption spectrometry The specific activities of the mutant enzymes were determined with the 6-hydroxydopamine assay (24) and were shown to be 96% for A4V and 75% for G93A, relative to the human WT enzyme
    • (1993) J. Am Chem. Soc. , vol.115 , pp. 5907
    • Lu, Y.1
  • 18
    • 0023113744 scopus 로고
    • 4 [C. R Nishida, E. B. Gralla, J. S. Valentine, Proc. Natl Acad. Sci U.S.A. 91, 9906 (1994)]. The extent of metallation was confirmed by atomic absorption spectrometry The specific activities of the mutant enzymes were determined with the 6-hydroxydopamine assay (24) and were shown to be 96% for A4V and 75% for G93A, relative to the human WT enzyme
    • (1987) Biotechnology , vol.5 , pp. 363
    • Halliwell, R.A.1
  • 19
    • 0028149084 scopus 로고
    • 4 [C. R Nishida, E. B. Gralla, J. S. Valentine, Proc. Natl Acad. Sci U.S.A. 91, 9906 (1994)]. The extent of metallation was confirmed by atomic absorption spectrometry The specific activities of the mutant enzymes were determined with the 6-hydroxydopamine assay (24) and were shown to be 96% for A4V and 75% for G93A, relative to the human WT enzyme
    • (1994) Proc. Natl Acad. Sci U.S.A. , vol.91 , pp. 9906
    • Nishida, C.R.1    Gralla, E.B.2    Valentine, J.S.3
  • 20
    • 0028098752 scopus 로고
    • Assignment of this signal to DMPO-OH was based on its typical A-B-B-A quadruplet pattern and its hyperline coupling constant of 14.9 G (9) We also performed similar reactions but with addition of ethanol (2 3 or 3 1 M) and observed a small signal attributable to the hydroxyethyl adduct of DMPO in addition to the large signal attributable to DMPO-OH [L. A. Reinke, D. R. Moore, C. M. Hague, P B. McCay, Free Radical Hes. 21, 213 (1994)]
    • (1994) Free Radical Hes. , vol.21 , pp. 213
    • Reinke, L.A.1    Moore, D.R.2    Hague, C.M.3    McCay, P.B.4
  • 21
    • 13344291801 scopus 로고    scopus 로고
    • note
    • 2 concentration of 3 mM.
  • 23
    • 0027327481 scopus 로고
    • P. M. Brooks, R. O. Day, G. G. Graham, K M Williams, Eds Birkhäuser, Basel
    • D. A Joyce, in Variability in Response to Anti-Rheumatic Drugs, P. M. Brooks, R. O. Day, G. G. Graham, K M Williams, Eds (Birkhäuser, Basel, 1993), pp. 203-207
    • (1993) Variability in Response to Anti-Rheumatic Drugs , pp. 203-207
    • Joyce, D.A.1
  • 24
    • 13344257533 scopus 로고    scopus 로고
    • note
    • Experiments were performed with conditions identical to those in Fig. 3, except that d,I-penicillamine was used in place of DDC. For both WT and A4V, the DMPO-OH signal was reduced by 60 to 95% in the presence of 13 μM penicillamine.
  • 25
    • 13344256749 scopus 로고    scopus 로고
    • unpublished data
    • 2+ chelators during the SOD assay, and the potential interference of these reagents with the assay itself (24) make conclusions concerning the concentrations required to inhibit the SOD activity of the enzymes and the mechanism of this activation uncertain (J. J Goto, M. Wiedau-Pazos, E. B. Gralla, J. S. Valentine, unpublished data).
    • Goto, J.J.1    Wiedau-Pazos, M.2    Gralla, E.B.3    Valentine, J.S.4
  • 27
    • 0027318190 scopus 로고
    • CSM14 1 is a conditionally immortalized rat nigral neural cell line [M M. Durand, D. C. Chugani, M Mahmoudi, M. E Phelps, Soc. Neurosci. Abstr. 16, 40 (1990), L. T Zhong et al., Proc. Natl. Acad. Sci U.S.A 90, 4533 (1993)].
    • (1993) Proc. Natl. Acad. Sci U.S.A , vol.90 , pp. 4533
    • Zhong, L.T.1
  • 28
    • 13344264423 scopus 로고    scopus 로고
    • note
    • Studies with penicillamine and cultured CSM14.1 cells were performed as described for DDC. d,I-Penicillamine (100 or 500 μM) was added to the cultures at the time of serum withdrawal. Penicillamine had no significant effect on survival of cells transfected with the vector alone (control) or with the WT CuZnSOD construct In contrast, addition of penicillamine to cells expressing A4V, G41D, or G85R mutant enzymes increased survival significantly. Cells expressing the G37R mutant showed a small penicillamine-induced improvement in survival, but this effect did not achieve statistical significance
  • 30
    • 13344254277 scopus 로고    scopus 로고
    • note
    • - Enz-Cu(II)(·OH) + DMPO → Enz-Cu(II) + DMPO-OH
  • 32
    • 0028893813 scopus 로고
    • L. A. Damani, Ed , Sulphur-Containing Drugs and Related Organic Compounds: Chemistry, Biochemistry, and Toxicology (Halsted, New York, 1989), vol 3B; D R Gandara et al., J. Clin. Oncol 13, 490 (1995)
    • (1995) J. Clin. Oncol , vol.13 , pp. 490
    • Gandara, D.R.1
  • 36
    • 13344258253 scopus 로고    scopus 로고
    • note
    • We thank C. J Epstein and T -T. Huang for the WT CuZnSOD genomic clone; D. R. Borchett, P. C. Wang, and S. S. Sisodia for the mutant CuZnSOD genomic clones; R H. Brown Jr, T B. Usdin, M. J. Brownstein, and D. McKenna-Yasek for mutant CuZnSOD complementary DMAs; K. F Faull for assistance with electrospray mass spectrometry; E. M. Landaw for assistance with statistical evaluation of the data; M. Durand and D. C. Chugani for CSM14.1 cells; J. Shi for technical assistance, and R. Y. N Ho for technical advice. Supported by NIH grants AG12282 (D E.B.), GM28222 (J S V.), and DK46828 (J.S.V.) and grants from the Amyotrophic Lateral Sclerosis Association (E.B.G.) and VivoRx Corp (S R.)


* 이 정보는 Elsevier사의 SCOPUS DB에서 KISTI가 분석하여 추출한 것입니다.