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The H46R/H48Q SOD1 artificial copper site mutant displays caricature disease-related characteristics including a very strong propensity to exist in the disulphide-reduced state and weakened affinity for zinc 31 that leads to complete monomerization at micromolar concentrations (Supplementary Fig
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Therefore, the increased FA uptake that occurs in diabetes may drive uncoupling, contributing to reduced ATP synthesis efficiency
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