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Indeed, the components of this system are involved in the bi-directional transport of acyl moieties from cytosol to mitochondria and vice versa, thus playing a fundamental role in tuning the switch between the glucose and fatty acid metabolism

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In another study, cell cultures of patients with PEO/Kearns-Sayre syndrome grown in a medium containing KB, hence replacing glucose as the carbon source, showed reduced heteroplasmy mtDNA mutation levels (117)