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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 : (667)

References (36)
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    • 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.
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    • 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
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    • note
    • - Enz-Cu(II)(·OH) + DMPO → Enz-Cu(II) + DMPO-OH
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    • 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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