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Acylcarnitines are transported across the inner mitochondrial membrane by the Carnitine/Acylcarnitine Carrier (CAC) in exchange for free carnitine

However, heart mitochondria isolated from these animals show severe dysfunction, i.e., inhibition of mitochondrial Complex I and respiration with NAD + -linked substrates, inhibition of aconitase, and increased sensitivity to Ca 2+ [113]