Article
Cu/Zn superoxide dismutase mutants associated with amyotrophic lateral sclerosis show enhanced formation of aggregates in vitro.
Proceedings of the National Academy of Sciences of the United States of America - 10 Jun 2003
Stathopulos P B, Rumfeldt J A O, Scholz G A, Irani R A, Frey H E, Hallewell R A, Lepock J R, Meiering E M
Abstract excerpt
Mutations in Cu/Zn superoxide dismutase (SOD) are associated with the fatal neurodegenerative disorder amyotrophic lateral sclerosis (ALS). There is considerable evidence that mutant SOD has a gain of toxic function; however, the mechanism of this toxicity is not known. We report here that purified SOD forms aggregates in vitro under destabilizing solution conditions by a process involving a transition from small...
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