Article
Immature copper-zinc superoxide dismutase and familial amyotrophic lateral sclerosis.
Experimental biology and medicine (Maywood, N.J.) - 1 Oct 2009
Seetharaman Sai V, Prudencio Mercedes, Karch Celeste, Holloway Stephen P, Borchelt David R, Hart P John
Abstract excerpt
Mutations in human copper-zinc superoxide dismutase (SOD1) cause an inherited form of amyotrophic lateral sclerosis (ALS, Lou Gehrig's disease, motor neuron disease). Insoluble forms of mutant SOD1 accumulate in neural tissues of human ALS patients and in spinal cords of transgenic mice expressing these polypeptides, suggesting that SOD1-linked ALS is a protein misfolding disorder. Understanding the molecular...
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