Article
Aggregation and motor neuron toxicity of an ALS-linked SOD1 mutant independent from wild-type SOD1.
Science (New York, N.Y.) - 18 Sept 1998
Bruijn L I, Houseweart M K, Kato S, Anderson K L, Anderson S D, Ohama E, Reaume A G, Scott R W, Cleveland D W
Abstract excerpt
Analysis of transgenic mice expressing familial amyotrophic lateral sclerosis (ALS)-linked mutations in the enzyme superoxide dismutase (SOD1) have shown that motor neuron death arises from a mutant-mediated toxic property or properties. In testing the disease mechanism, both elimination and elevation of wild-type SOD1 were found to have no effect on mutant-mediated disease, which demonstrates that the use of SOD...
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