Article
A common property of amyotrophic lateral sclerosis-associated variants: destabilization of the copper/zinc superoxide dismutase electrostatic loop.
The Journal of biological chemistry - 6 Nov 2009
Molnar Kathleen S, Karabacak N Murat, Johnson Joshua L, Wang Qi, Tiwari Ashutosh, Hayward Lawrence J, Coales Stephen J, Hamuro Yoshitomo, Agar Jeffrey N
Abstract excerpt
At least 119 mutations in the gene encoding copper/zinc superoxide dismutase (SOD1) cause amyotrophic lateral sclerosis by an unidentified toxic gain of function. We compared the dynamic properties of 13 as-isolated, partially metallated, SOD1 variant enzymes using hydrogen-deuterium exchange. We identified a shared property of these familial amyotrophic lateral sclerosis-related SOD1 variants, namely structural...
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