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Braz J Otorhinolaryngol

Valproic acid aggravates epilepsy due to MELAS in a patient with an A3243G mutation of mitochondrial DNA

Il ruolo di tali integratori quello di stimolare l'organismo a produrre energia partendo dagli acidi grassi che, mediante il processo di -ossidazione mitocondriale, forniscono adeguate quantit di ATP

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As mitochondrial membranes are impermeable to coenzyme A (CoA) esters and long-chain fatty acids, binding of l-carnitine to acetyl groups via carnitine acyltransferase is essential for the shuttle of the acetylated fatty acids into the mitochondria and for their subsequent -oxidation in the matrix (Figure 1) [4]