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Complex I mutations result in decreased flux through the ETC, leading to local hyperoxia, which can further inhibit complex I activity. Hypoxia orNDUFA6/nuo-3(G60D)mutation restore complex I forward activity in a manner dependent on residues surrounding the CoQ binding cavity. For all panels, statistical significance was calculated using one-way ANOVA followed by Tukey’s multiple comparison test. Error bars represent standard deviation. n.s., not significant,*p value < 0.05,**p value < 0.01,***p value < 0.001. See alsoFigure S7.

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