Prior studies indicated that PANK2 can localize in the intermembrane space (IMS) of the mitochondria [16] (Figure 2B), where it is most likely to be exposed to the activating action of Long Chain Acyl-carnitines [17] (Figure 1a), especially when Fatty Acid Oxidation (FAO) is active and production of Acyl-carnitines by the action of carnitine palmitoyl transferase 1A (CPT1A) is high. This evidence concerns the gene PANK2 and fatty acid oxidation.