Article
The cysteine-reactive small molecule ebselen facilitates effective SOD1 maturation.
Nature communications - 27 Apr 2018
Capper Michael J, Wright Gareth S A, Barbieri Letizia, Luchinat Enrico, Mercatelli Eleonora, McAlary Luke, Yerbury Justin J, O'Neill Paul M, Antonyuk Svetlana V, Banci Lucia, Hasnain S Samar
Abstract excerpt
Superoxide dismutase-1 (SOD1) mutants, including those with unaltered enzymatic activity, are known to cause amyotrophic lateral sclerosis (ALS). Several destabilizing factors contribute to pathogenicity including a reduced ability to complete the normal maturation process which comprises folding, metal cofactor acquisition, intra-subunit disulphide bond formation and dimerization. Immature SOD1 forms toxic...
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