Article
Reduced reactivation rate in mutant CuZnSOD and progression rate of amyotrophic lateral sclerosis.
European journal of neurology - 1 Jun 2004
Völkel H, Selzle M, Walk T, Jung G, Link J, Ludolph A C, Reuter A
Abstract excerpt
Mutations in the SOD1 gene are associated with familial amyotrophic lateral sclerosis (fALS). The mechanisms by which these mutations lead to anterior horn cell loss are unknown, however, increased binding of Hsps on the demetallated mutant SOD1 has been described which would make the HSPs unavailable for other purposes, and reduce the SOD1 concentration in mitochondria, thereby creating a proapoptotic situation...
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