Article
Modulation of mutant superoxide dismutase 1 aggregation by co-expression of wild-type enzyme.
Journal of neurochemistry - 1 Feb 2009
Prudencio Mercedes, Durazo Armando, Whitelegge Julian P, Borchelt David R
Abstract excerpt
Mutations in superoxide dismutase 1 (SOD1, EC 1.15.1.1) cause familial amyotrophic lateral sclerosis; with aggregated forms of mutant protein accumulating in spinal cord tissues of transgenic mouse models and human patients. Mice over-expressing wild-type human SOD1 (WT hSOD1) do not develop amyotrophic lateral sclerosis-like disease, but co-expression of WT enzyme at high levels with mutant SOD1 accelerates the...
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