Nishida CR, Gralla EB, Valentine JS  Proc Natl Acad Sci U S A  1994 Oct 11;91(21):9906-10


Department of Chemistry and Biochemistry, University of California, Los Angeles 90024.

Sequences encoding three copper-zinc superoxide dismutase (CuZnSOD) mutantproteins analogous to those coded for in familial amyotrophic lateral sclerosis(fALS) were constructed in the Saccharomyces cerevisiae CuZnSOD gene andexpressed in yeast lacking CuZnSOD (sod1-). Gly85–>Arg CuZnSOD failed to rescue the oxygen-sensitive phenotype of sod1- yeast, but Gly93–>AlaCuZnSOD and Lys100–>Gly CuZnSOD were apparently fully functional in vivo.The Gly85–>Arg mutant protein was purified and its metal-binding propertiesand SOD activity were found to be significantly altered relative to wildtype. The Gly93–>Ala CuZnSOD was likewise purified but, in contrast, demonstrated metal-binding comparable to wild type and activity 80% that of wild type. These results suggest that SOD activity of human fALS mutantCuZnSODs may vary considerably in vivo, with at least some of them retaining a considerable amount of activity. Alternative theories to increased free-radicaldamage should be considered in attempting to explain fALS.

Please Note

We are sorry but we can offer no international Paypal orders. These must be made by Fax. Any inadvertent international Paypal orders placed will be refunded less $20 transaction fee.

P.O. Box 1357
Sparta, NC 28675
Phone: 336-793-6524
Fax: 336-372-1541
Email: sotousa@skybest.com

Shipping Charges

$1.00 - $50.00 = $18.00
$50.01 - $100.00 = $22.00
$100.01 - $150.00 = $27.00
$150.01 - $200.00 = $32.00
Over $200 or more and orders including books ask for shipping charges