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This process is especially important during physical activity, helping to increase endurance and support fat utilization as a fuel source

It is postulated that clinical symptoms, including CNS complications such as convulsion, coma and lethargy, may be triggered by the accumulation of metabolites and their acyl-CoA derivatives that disrupt intermediary metabolism [6669]

The disease is characterized by general metabolic derangement, cardiomyopathy, hyperammonemia, hypoglycemia, muscle weakness, and myopathy, in line with the crucial role of carnitine in FAO (Nezu et al., 1999)

On the day of the experiment, DMEM culture media was removed from the AC16 96-well plate, and the cells were incubated at 37C in 0% CO 2 in unbuffered Seahorse XFe base media supplemented with 5 mM glucose, 1 mM Na pyruvate, and 2 mM L-glutamine