Stathopulos PB, Rumfeldt JA, Scholz GA, Irani RA, Frey HE, Hallewell RA, Lepock JR, Meiering EM.
Cu/Zn superoxide dismutase mutants associated with amyotrophic lateral sclerosis show enhanced formation of aggregates in vitro.
Proc Natl Acad Sci U S A. 2003 Jun 10;100(12):7021-6.
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The concept of mutated SOD1 aggregate formation was previously evoked, but this article gives experimental evidence supporting this hypothesis. It remains now to explain why not all motoneurons (i.e. oculomotor nucleus) are not impaired.
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