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Data availability All data relevant to the findings are included in the manuscript

For the electron coupling assays, respiratory substrates (10 mM pyruvate + 2 mM malate, 10 mM succinate + 2 M rotenone, 80 M palmitoyl-carnitine or octanoyl-carnitine + 0.5 mM malate or 10 mM glutamate + 10 mM malate) were diluted in MAS buffer with BSA (70 mM sucrose, 220 mM mannitol, 10 mM KH 2 PO 4 , 5 mM MgCl 2 , 2 mM HEPES and 1 mM EGTA, pH 7.2, in 0.2% FA-free BSA) and added to mitochondria

however, they remained higher than control mice

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