OMA1 mediates local and global stress responses against protein misfolding in CHCHD10 mitochondrial myopathy.

The respiratory exchange ratio, measured by a comprehensive laboratory animal monitoring system (CLAMS), tended to be higher for C10 G58R mice at night (indicating increased carbohydrate reliance) and lower during the day (indicating increased fatty acid reliance) compared with that of C10 WT littermates, although these differences did not reach statistical significance ( Supplemental Figure 2E ).

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