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The essential link between the mitochondrial carnitine transport and FAO is demonstrated by the life-threatening inherited carnitine/acylcarnitine translocase deficiency (OMIM 613698) that, in contrast to the PCD caused by OCTN2 defects, cannot be compensated by the administration of carnitine (Indiveri et al., 2011)
Insulin-resistance in glycogen storage disease type Ia: linking carbohydrates and mitochondria

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