Article
Dysregulation of the proteasome increases the toxicity of ALS-linked mutant SOD1.
Genes to cells : devoted to molecular & cellular mechanisms - 1 Mar 2014
Kitamura Akira, Inada Noriko, Kubota Hiroshi, Matsumoto Gen, Kinjo Masataka, Morimoto Richard I, Nagata Kazuhiro
Abstract excerpt
A hallmark of protein conformational disease, exemplified by neurodegenerative disorders, is the expression of misfolded and aggregated proteins. The relationship between protein aggregation and cellular toxicity is complex, and various models of experimental pathophysiology have often yielded conflicting or controversial results. In this study, we examined the biophysical properties of amyotrophic lateral...
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