Article
Localization of a toxic form of superoxide dismutase 1 protein to pathologically affected tissues in familial ALS.
Proceedings of the National Academy of Sciences of the United States of America - 3 Apr 2012
Brotherton Terrell E, Li Yingjie, Cooper Deborah, Gearing Marla, Julien Jean-Pierre, Rothstein Jeffrey D, Boylan Kevin, Glass Jonathan D
Abstract excerpt
Mutations in the gene encoding superoxide dismutase 1 (SOD1) account for about 20% of the cases of familial amyotrophic lateral sclerosis (fALS). It is not known how the mutant protein causes disease, or why only a subset of cell types (motor neurons) are targeted. The aggregation and misfolding of mutant SOD1 are implicated in disease pathogenesis in both animal models and humans. We used a monoclonal antibody,...
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