Article
Impaired post-translational folding of familial ALS-linked Cu, Zn superoxide dismutase mutants.
The EMBO journal - 7 Feb 2007
Bruns Cami K, Kopito Ron R
Abstract excerpt
Over 110 structurally diverse missense mutations in the superoxide dismutase (SOD1) gene have been linked to the pathogenesis of familial amyotrophic lateral sclerosis (FALS), yet the mechanism by which these lead to cytotoxicity still remains unknown. We have synthesized wild-type and mutant SOD1 in synchronized cell-free reticulocyte extracts replete with the full complement of molecular chaperones and folding...
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