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To further strengthen the contribution of OS in ALS, an increase in the level of several proteins oxidatively modified and consequently inactivated has been found in SOD1 G93A transgenic mice and correlated with SOD1 mutation: such proteins are SOD1 itself, translationally controlled tumor protein (TCTP, which normally processes calcium binding activity and acts as a cytoprotective factor), and ubiquitin carboxyl-terminal hydrolase isoenzyme L1 (UCH-L1, which plays an important role in the ubiquitin-proteasome system, UPS)