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Neurobiology: Mutant Enzyme Provides New Insights Into the Cause of ALS

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Science  26 Jan 1996:
Vol. 271, Issue 5248, pp. 446-447
DOI: 10.1126/science.271.5248.446


Although neurobiologists have known for 3 years that mutations in the SOD1 gene cause some cases of familial amyotrophic lateral sclerosis (FALS), finding out exactly how those mutations cause the deadly neurodegeneration of the disease has proved tougher than expected. But two reports, one appearing on page page 515 of this issue and the other in the February Annals of Neurology, now suggest that one way the mutations act is by increasing the ability of the enzyme encoded by SOD1 to carry out peroxidation, a potentially toxic reaction that may lead to membrane degeneration and neuronal cell death. If so, then it may be possible to design better ALS therapies aimed at blocking the peroxidation reaction.