Article
Metal deficiency increases aberrant hydrophobicity of mutant superoxide dismutases that cause amyotrophic lateral sclerosis.
The Journal of biological chemistry - 2 Oct 2009
Tiwari Ashutosh, Liba Amir, Sohn Se Hui, Seetharaman Sai V, Bilsel Osman, Matthews C Robert, Hart P John, Valentine Joan Selverstone, Hayward Lawrence J
Abstract excerpt
The mechanisms by which mutant variants of Cu/Zn-superoxide dismutase (SOD1) cause familial amyotrophic lateral sclerosis are not clearly understood. Evidence to date suggests that altered conformations of amyotrophic lateral sclerosis mutant SOD1s trigger perturbations of cellular homeostasis that ultimately cause motor neuron degeneration. In this study we correlated the metal contents and disulfide bond status...
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