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Excess beyond daily needs is excreted, largely unchanged in urine

These studies showed that through the targeted elimination of mutated mtDNA, a beneficial shift in heteroplasmy and improvement in the biochemical deficit can be achieved [62,63,64,65,66,67]

Moore, KP

These crucial intermediates (fatty acyl-CoA thioesters) undergo metabolism through mitochondrial -oxidation pathways, which involve a series of enzymes located either in the inner mitochondrial membrane or mitochondrial matrix (Fig