-
2
-
-
0027401203
-
-
D. R. Rosen et al., Nature 362, 59 (1993)
-
(1993)
Nature
, vol.362
, pp. 59
-
-
Rosen, D.R.1
-
3
-
-
0027426169
-
-
H. X Deng et al., Science 261, 1047 (1993)
-
(1993)
Science
, vol.261
, pp. 1047
-
-
Deng, H.X.1
-
4
-
-
13344265498
-
-
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
-
5
-
-
0028888945
-
-
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
-
(1995)
Proc. Natl. Acad. Sci U.S.A
, vol.92
, pp. 689
-
-
Ripps, M.E.1
Huntley, G.W.2
Hof, P.R.3
Mornson, J.H.4
Gordon, J.W.5
-
6
-
-
13344251465
-
-
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
-
-
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
-
-
E. K. Hodgson and I. Fndovich, Biochemistry 14, 5294 (1975); ibid , p 5299.
-
Biochemistry
, pp. 5299
-
-
-
13
-
-
0025285382
-
-
M. B. Yim, P B Chock, E R. Stadtman, Proc. Natl. Acad Sci. USA. 87, 5006 (1990), J. Biol. Chem. 268, 4099 (1993).
-
(1990)
Proc. Natl. Acad Sci. USA.
, vol.87
, pp. 5006
-
-
Yim, M.B.1
Chock, P.B.2
Stadtman, E.R.3
-
14
-
-
0027456505
-
-
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
-
-
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
-
-
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
-
-
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
-
-
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
-
-
note
-
2 concentration of 3 mM.
-
-
-
-
23
-
-
0027327481
-
-
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
-
-
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
-
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13344256749
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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
-
26
-
-
0002817436
-
-
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)].
-
(1990)
Soc. Neurosci. Abstr.
, vol.16
, pp. 40
-
-
Durand, M.M.1
Chugani, D.C.2
Mahmoudi, M.3
Phelps, M.E.4
-
27
-
-
0027318190
-
-
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
-
-
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
-
-
-
-
29
-
-
0027293275
-
-
J S. Beckman, M. Carson, C. D. Smith, W. H Koppenol, Nature 364, 584 (1993).
-
(1993)
Nature
, vol.364
, pp. 584
-
-
Beckman, J.S.1
Carson, M.2
Smith, C.D.3
Koppenol, W.H.4
-
30
-
-
13344254277
-
-
note
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- Enz-Cu(II)(·OH) + DMPO → Enz-Cu(II) + DMPO-OH
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-
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-
-
1842544244
-
-
Halsted, New York
-
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)
-
(1989)
Sulphur-Containing Drugs and Related Organic Compounds: Chemistry, Biochemistry, and Toxicology
, vol.3 B
-
-
Damani, L.A.1
-
32
-
-
0028893813
-
-
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
-
-
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.)
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