Article
Relationship between mutant Cu/Zn superoxide dismutase 1 maturation and inclusion formation in cell models.
Journal of neurochemistry - 1 Jan 2017
Ayers Jacob I, McMahon Benjamin, Gill Sabrina, Lelie Herman L, Fromholt Susan, Brown Hilda, Valentine Joan Selverstone, Whitelegge Julian P, Borchelt David R
Abstract excerpt
A common property of Cu/Zn superoxide dismutase 1 (SOD1), harboring mutations associated with amyotrophic lateral sclerosis, is a high propensity to misfold and form abnormal aggregates. The aggregation of mutant SOD1 has been demonstrated in vitro, with purified proteins, in mouse models, in human tissues, and in cultured cell models. In vitro translation studies have determined that SOD1 with amyotrophic...
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